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Sample records for autoimmune bullous dermatoses

  1. Dermatoses bolhosas auto-imunes Autoimmune bullous dermatoses

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    Paulo R. Cunha

    2009-04-01

    Full Text Available Dermatoses bolhosas autoimunes são doenças cuja manifestação cutânea primária e fundamental consiste em vesículas e bolhas. Classificam-se conforme a localização da bolha, em intraepidérmica e subepidérmica. Os pacientes produzem autoanticorpos contra estruturas específicas da pele detectáveis por técnicas de imunofluorescência, immunobloting e Elisa. Os recentes avanços da biologia molecular e celular têm permitido conhecer esses autoantígenos, contra os quais os pacientes se sensibilizam e que estão localizados na epiderme ou na junção dermoepidérmica. São doenças de baixa incidência, porém de elevada morbidade e por vezes letais. O objetivo deste trabalho é revisar e descrever os progressos nos conhecimentos de quatro doenças vésico-bolhosas autoimunes: pênfigo foliáceo endêmico (fogo selvagem, pênfigo vulgar, penfigóide bolhoso e dermatite herpetiforme.Autoimmune bullous dermatoses are diseases in which blisters and vesicles are the primary and fundamental types of skin lesion. Their classification is based on the location of the blister: intraepidermal and subepidermal. Patients produce autoantibodies against self-specific structures of the skin detectable by immunofluorescence techniques, immunoblotting and ELISA. Recent advances in molecular and cellular biology have brought to knowledge these self-antigens, against which patients are sensitized, and which are found in epidermis or in the dermo-epidermal junction. These are low incidence, but high morbidity diseases that may be fatal. The aim of this article is to review and describe the progress of four autoimmune vesiculobullous disorders: endemic pemphigus foliaceous (wild fire, pemphigus vulgaris, bullous pemphigoid and dermatitis herpetiformis.

  2. Loss of Work Productivity and Quality of Life in Patients With Autoimmune Bullous Dermatoses.

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    Heelan, K; Hitzig, S L; Knowles, S; Drucker, A M; Mittmann, N; Walsh, S; Shear, N H

    2015-01-01

    Little is known about quality of life and work productivity in autoimmune bullous dermatoses (AIBDs). To determine the impact of AIBDs on quality of life and work productivity. An observational cross-sectional study took place between February and May 2013 at an AIBD tertiary referral centre. Ninety-four patients were included. All participants completed the Dermatology Life Quality Index and the Work Productivity and Activity Impairment-Specific Health Problem questionnaires. Responders to treatment had less impairment (Pwork missed. Those with a higher Dermatology Life Quality Index score had greater work impairment and overall activity impairment (P=.041, P=.024). Nonresponders had increased impairment while working (Pwork impairment (PWork Productivity and Activity Impairment Questionnaire domains. AIBD has the potential to be a large burden on ability to work and quality of life. Larger studies are needed to clarify how these domains change over time and whether or not they improve with treatment. © The Author(s) 2015.

  3. The Etiopathogenesis of Autoimmune Bullous Diseases

    OpenAIRE

    2011-01-01

    Autoimmune bullous diseases are rare disorders affecting skin and mucous membranes which are mediated by pathogenic autoantibodies against target antigens whose function is adhesion within the epidermis or adhesion of epidermis to dermis. The pathogenesis of these disorders has been extensively investigated with advanced techniques in recent years. This review focuses on the etiopathogenesis of main autoimmune bullous disorders including pemphigus, bullous pemphigoid, anti-p200 pemphigoid, ci...

  4. The Etiopathogenesis of Autoimmune Bullous Diseases

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    Şebnem Aktan

    2011-06-01

    Full Text Available Autoimmune bullous diseases are rare disorders affecting skin and mucous membranes which are mediated by pathogenic autoantibodies against target antigens whose function is adhesion within the epidermis or adhesion of epidermis to dermis. The pathogenesis of these disorders has been extensively investigated with advanced techniques in recent years. This review focuses on the etiopathogenesis of main autoimmune bullous disorders including pemphigus, bullous pemphigoid, anti-p200 pemphigoid, cicatricial pemphigoid, pemphigoid gestationis, dermatitis herpetiformis, linear IgA bullous dermatosis and epidermolysis bullosa acquisita.

  5. Clinical Relevance of Autoantibodies in Patients with Autoimmune Bullous Dermatosis

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    Lilla Mihályi

    2012-01-01

    Full Text Available The authors present their experience related to the diagnosis, treatment, and followup of 431 patients with bullous pemphigoid, 14 patients with juvenile bullous pemphigoid, and 273 patients with pemphigus. The detection of autoantibodies plays an outstanding role in the diagnosis and differential diagnosis. Paraneoplastic pemphigoid is suggested to be a distinct entity from the group of bullous pemphigoid in view of the linear C3 deposits along the basement membrane of the perilesional skin and the “ladder” configuration of autoantibodies demonstrated by western blot analysis. It is proposed that IgA pemphigoid should be differentiated from the linear IgA dermatoses. Immunosuppressive therapy is recommended in which the maintenance dose of corticosteroid is administered every second day, thereby reducing the side effects of the corticosteroids. Following the detection of IgA antibodies (IgA pemphigoid, linear IgA bullous dermatosis, and IgA pemphigus, diamino diphenyl sulfone (dapsone therapy is preferred alone or in combination. The clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis is stressed.

  6. Epidemiology of Autoimmune Bullous Diseases

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    Erkan Alpsoy

    2011-06-01

    Full Text Available We have very limited knowledge about aotuimmune bullous disesases which are important causes of morbidity and mortality. They are generally rare disases in population. The yearly over all incidences of pemphigus and bullous pemhigoid are between 0.5 to 16.1/million and 2.5 to 42.8/million, respectively. Pemphigus vulgaris is the major type of pemphigus and it is most prevalent between ages of 40 and 50. Bullous pemphigoid is tpypically most prevalent in ages of over 70. In this review the results obtained from the studies which are especially about epidemiology of pemphigus and bullous pemphigoid were analised according to geographic regions.

  7. Bullous Skin Diseases: Classical Types of Autoimmune Diseases

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    Jan Damoiseaux

    2013-01-01

    Full Text Available The prototypic bullous skin diseases, pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, are characterized by the blister formation in the skin and/or oral mucosa in combination with circulating and deposited autoantibodies reactive with (hemidesmosomes. Koch’s postulates, adapted for autoimmune diseases, were applied on these skin diseases. It appears that all adapted Koch’s postulates are fulfilled, and, therefore, these bullous skin diseases are to be considered classical autoimmune diseases within the wide and expanding spectrum of autoimmune diseases.

  8. Tzanck Cytology in Diagnosis of Autoimmune Bullous Diseases

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    Murat Durdu

    2011-06-01

    Full Text Available Tzanck smear test is a simple, rapid, repeatable, and inexpensive diagnostic method based on the investigation of characteristics of individual cells. For diagnosis of cutaneous diseases, cytology was first used by Arnault Tzanck in 1947. After this date, Tzanck cytology has been used in the diagnosis of various erosive-vesiculobullous, nodular, and tumoral skin lesions. In daily dermatology practice, the most common use areas of cytology are diagnosis of herpetic infections, cutaneous leishmaniasis, leprosy, and autoimmune bullous diseases. The purpose of cytology in autoimmune bullous diseases is to rapidly distinguish pemphigus from subepidermal bullous disease. In this review article, taking and staining methods of cytologic specimen for the diagnosis of autoimmune bullous diseases, and the cytologic findings have been reviewed.

  9. Treatment of Intraepidermal Autoimmune Bullous Diseases

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    Tamer İrfan Kaya

    2011-06-01

    Full Text Available Pemfigus is an autoimmune bullous skin disease, characterized by intraepidermal blisters. It is a severe and potentially life-threatening chronic disease with blisters and erosions on the mucosae and the skin. Treatment options do not differ for two most common types of pemphigus, pemphigus vulgaris and pemphigus foliaceus, except that the latter is usually less resistant to treatment and corticosteroids can often be started at lower doses. Systemic corticosteroids are still the most widely used drugs in the treatment of pemphigus and continue to be the mainstay of therapy for this disease. Adjuvant drugs are commonly used in combination with the aims of increasing efficacy and of having a steroid-sparing action, thereby allowing reduced corticosteroid side-effects. Mortality and complete remission rates have improved since the introduction of adjuvant drugs to pemphigus. Adjuvant drugs include immunoadsorbtion, corticosteroid pulse therapy, intravenous immunoglobulin (IVIG, immunosuppressive agents such as azathioprine, cyclophosphamide, mycophenolate mofetil and and anti-CD20 monoclonal antibody (rituximab. The lack of consensus in the published literature about the treatment of this disorder is responsible for different treatment strategies. Treatments need to be chosen after careful consideration of the potential benefits and side effects according to the patients’ medical condition. Here, both conventional therapies and novel treatment regimens for pemphigus are discussed.

  10. Treatment of Subepidermal Autoimmune Bullous Diseases

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    Ekin Şavk

    2011-06-01

    Full Text Available The common therapeutic goal for all subepidermal bullous diseases is to prevent inflammation and production of pathogenic autoantibodies and/or to facilitate the elimination of these antibodies. Diseases included in this group are the pemphigoids, linear IgA dermatosis, epidermolysis bullosa acquisita, dermatitis herpetiformis and bullous systemic erythematosus. Corticosteroids are the most commonly used systemic medication. Other alternatives include dapsone, various antibiotics and nicotinamide, azathioprine, cyclophosphamide, methotrexate, cycloporine, mycophenolate mofetil, IV immunoglobulins and plasmapheresis. Rapid reepithelialization and prevention of secondary infections are provided by local hygenic measures including antiseptic baths and wound care.

  11. IgE-mediated mechanisms in bullous pemphigoid and other autoimmune bullous diseases.

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    van Beek, Nina; Schulze, Franziska S; Zillikens, Detlef; Schmidt, Enno

    2016-01-01

    Autoimmune bullous diseases (AIBDs) are characterized by autoantibodies against structural proteins of the dermal-epidermal junction (in pemphigoid diseases) and the epidermal/ epithelial desmosomes (in pemphigus diseases). By far, the most common AIBD is bullous pemphigoid, which is immunopathologically characterized by autoantibodies against BP180 (type XVII collagen) and BP230. IgG and, to a lesser extent, IgA autoantibodies are the major autoantibody isotypes in these disorders. IgE autoantibodies are increasingly reported in particular in bullous pemphigoid. The development of specific and sensitive anti-BP180 IgE ELISA systems, the report of two experimental murine models employing IgE autoantibodies against BP180, and the successful treatment of bullous pemphigoid with the anti-IgE antibody omalizumab have raised interest in the role of IgE autoantibodies and the modulation of their production in AIBDs. Here, the relevance of IgE autoantibodies in the diagnosis, pathophysiology, and treatment decisions of AIBDs, with a focus on bullous pemphigoid, is reviewed.

  12. A necessidade da imunofluorescência direta no diagnóstico da dermatose bolhosa por IgA The need for direct immunofluorescence in the diagnosis of IgA bullous dermatosis

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    Daniel Chang

    2012-02-01

    Full Text Available A dermatose bolhosa por imunoglobulina da classe A linear (DbIgA do adulto é uma doença autoimune rara caracterizada por formação de bolhas subepidérmicas e depósito linear de imunoglobulina da classe A (IgA na zona da membrana basal (ZMB. Por possuir aspectos clínicos e histológicos semelhantes a outras dermatoses bolhosas, principalmente a dermatite herpetiforme e o penfigoide bolhoso, faz-se necessária a realização de imunofluorescência direta para confirmação diagnóstica. Apresenta-se então, neste artigo, relato de caso ilustrando essa necessidade.Linear immunoglobulin A bullous dermatosis (DbIgA of adults is a rare autoimmune disease characterized by subepidermal blistering and linear deposits of immunoglobulin A (IgA in the basement membrane zone (BMZ. Owing to the fact it presents clinical and histological aspects similar to other bullous dermatosis, mainly dermatitis herpetiformis and bullous pemphigoid, direct immunofluorescence is required to confirm diagnosis. In this article, we describe a case that illustrates this need.

  13. The Clinical Diagnosis of Autoimmune Bullous Diseases Sürekli Eğitim

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    Rıfkiye Küçükoğlu

    2011-06-01

    Full Text Available The autoimmune bullous diseases were diagnosed on the clinical and histopathological basis, before the introduction of the immunological assays. However, not the clinical features, but the classification of the diseases has recently changed during the immunological development. According to this new classification, pemphigus group diseases include, pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus, pemphigus erythematosus, endemic pemphigus, IgA pemphigus, pemphigus herpetiformis, paraneoplastic pemphigus, and drug-induced pemphigus. The subepidermal bullous diseases are classified as pemphigoid group diseases, epidermolysis bullosa acquisita, dermatitis herpetiformis, and linear IgA bullous dermatosis. The pemphigoid-group diseases consist of bullous pemphigoid, childhood bullous pemphigoid, localised bullous pemphigoid, drug-induced pemphigoid, anti p200 pemphigoid, pemphigoid gestationes, pemphigoid nodularis, and cicatricial pemphigoid. In this review, the clinical features of the autoimmune bullous diseases are discussed according to the above mentioned classification. (Turkderm 2010; 45 Suppl 1: 16-25

  14. Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia.

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    Arakaki, Osao; Yamamoto, Yu-ichi; Awazawa, Ryoko; Nonaka, Kimiko; Taira, Kiyohito; Asato, Yutaka; Hagiwara, Keisuke; Oyama, Bungo; Ishii, Norito; Hashimoto, Takashi; Uezato, Hiroshi

    2008-07-01

    Linear immunoglobulin (Ig)A bullous dermatosis is a rare autoimmune subepidermal bullous dermatosis caused by circulating IgA autoantibodies directed against the antigens at the basement membrane zone. Most linear IgA bullous dermatosis cases are idiopathic, but some are associated with the use of certain drugs, infections, lymphoproliferative disorders, internal malignancies, autoimmune disorders, collagen diseases or, very rarely, other skin diseases, including autoimmune bullous diseases. Acquired hemophilia is also rare; it is a coagulation disease caused by anti-factor VIII IgG antibodies. Acquired hemophilia has been reported to be associated with malignant tumors, pregnancy or postpartum, drug reactions, collagen diseases such as rheumatoid arthritis, autoimmune disorders, and skin diseases such as psoriasis and pemphigus. We report a case of hemophilia acquired during the course of linear IgA bullous dermatosis and review reported cases of autoimmune bullous dermatoses associated with acquired hemophilia.

  15. Clinical Relevance of Autoantibodies in Patients with Autoimmune Bullous Dermatosis

    OpenAIRE

    Lilla Mihályi; Mária Kiss; Attila Dobozy; Lajos Kemény; Sándor Husz

    2012-01-01

    The authors present their experience related to the diagnosis, treatment, and followup of 431 patients with bullous pemphigoid, 14 patients with juvenile bullous pemphigoid, and 273 patients with pemphigus. The detection of autoantibodies plays an outstanding role in the diagnosis and differential diagnosis. Paraneoplastic pemphigoid is suggested to be a distinct entity from the group of bullous pemphigoid in view of the linear C3 deposits along the basement membrane of the perilesional skin ...

  16. The Etiopathogenesis of Autoimmune Bullous Diseases Sürekli Eğitim

    Directory of Open Access Journals (Sweden)

    Şebnem Aktan

    2011-06-01

    Full Text Available which are mediated by pathogenic autoantibodies against target antigens whose function is adhesion within the epidermis or adhesion of epidermis to dermis. The pathogenesis of these disorders has been extensively investigated with advanced techniques in recent years. This review focuses on the etiopathogenesis of main autoimmune bullous disorders including pemphigus, bullous pemphigoid, anti-p200 pemphigoid, cicatricial pemphigoid, pemphigoid gestationis, dermatitis herpetiformis, linear IgA bullous dermatosis and epidermolysis bullosa acquisita. (Turkderm 2011; 45 Suppl 1: 8-15

  17. The Etiopathogenesis of Autoimmune Bullous Diseases Sürekli Eğitim

    OpenAIRE

    2011-01-01

    which are mediated by pathogenic autoantibodies against target antigens whose function is adhesion within the epidermis or adhesion of epidermis to dermis. The pathogenesis of these disorders has been extensively investigated with advanced techniques in recent years. This review focuses on the etiopathogenesis of main autoimmune bullous disorders including pemphigus, bullous pemphigoid, anti-p200 pemphigoid, cicatricial pemphigoid, pemphigoid gestationis, dermatitis herpetiformis, linear IgA ...

  18. Intravenous Immunoglobulins: Mode of Action and Indications in Autoimmune and Inflammatory Dermatoses

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    Lyubomir A. Dourmishev

    2016-01-01

    Full Text Available Intravenous immunoglobulins (IVIGs, a mixture of variable amounts of proteins (albumin, IgG, IgM, IgA, and IgE antibodies, as well as salt, sugar, solvents, and detergents, are successfully used to treat a variety of dermatological disorders. For decades, IVIGs have been administered for treatment of infectious diseases and immune deficiencies, since they contain natural antibodies that represent a first-line defense against pathogens. Today their indication has expanded, including the off-label therapy for a variety of autoimmune and inflammatory diseases. In dermatology, IVIGs are administered for treatment of different disorders at different therapeutic regimens, mostly with higher doses then those administered for treatment of infectious diseases. The aim of this prospective review is to highlight the indications, effectiveness, side effects, and perspectives of the systemic treatment with IVIGs for patients with severe, life-threatening, and resistant to conventional therapies autoimmune or inflammatory dermatoses.

  19. Development of a disease registry for autoimmune bullous diseases: initial analysis of the pemphigus vulgaris subset.

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    Shah, Amit Aakash; Seiffert-Sinha, Kristina; Sirois, David; Werth, Victoria P; Rengarajan, Badri; Zrnchik, William; Attwood, Kristopher; Sinha, Animesh A

    2015-01-01

    Pemphigus vulgaris (PV) is a rare, potentially life threatening, autoimmune blistering skin disease. The International Pemphigus and Pemphigoid Foundation (IPPF) has recently developed a disease registry with the aim to enhance our understanding of autoimmune bullous diseases with the long-term goal of acquiring information to improve patient care. Patients were recruited to the IPPF disease registry through direct mail, e-mail, advertisements, and articles in the IPPF-quarterly, -website, -Facebook webpage, and IPPF Peer Health Coaches to complete a 38-question survey. We present here the initial analysis of detailed clinical information collected on 393 PV patients. We report previously unrecognized gender differences in terms of lesion location, autoimmune comorbidity, and delay in diagnosis. The IPPF disease registry serves as a useful resource and guide for future clinical investigation.

  20. A cross-sectional study of clinical, histopathological and direct immmunofluorescence diagnosis in autoimmune bullous diseases

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    Anchal Jindal

    2014-01-01

    Full Text Available Background: Immunobullous diseases are morphologically heterogeneous and the differentiation between various subtypes is essential for proper treatment and prognosis. Aim of our study was to analyze and correlate clinical, histopathological and immunofluorescence findings in autoimmune bullous diseases. Materials and Methods: A cross-sectional study was done over a period of two years (2010-2012 after approval of the ethics committee. Sixty patients, who met the inclusion criteria of immunobullous disease, were included in the study. Skin biopsy for histopathology and direct immunofluorescence (DIF examination was taken. DIF using salt-split technique was done in few of the cases. The final diagnosis was based on clinical, histopathology and DIF findings. Pearson′s coefficient of correlation (r was calculated. Statistical Analysis was done using Epi info version. 7.0. Results: Fifty-three cases with clinical diagnosis of autoimmune bullous diseases were evaluated. In 88.6% of cases, histopathology diagnosis was consistent with clinical diagnosis and in 75.5% of cases, DIF findings were consistent with clinical diagnosis. A positive relation was seen between clinical and DIF findings with r = 0.65 and between histopathology and DIF findings with r = 0.75. DIF positivity was seen in 100% cases of bullous pemphigoid (BP and pemphigus foliaceous and 94.7% cases of pemphigus vulgaris, which was statistically significant with p < 0.05. In DIF salt-split test, deposition was seen on roof of blister in BP whereas on floor in epidermolysis bullosa acquisita. Conclusion: Our study provides evidence-based guidance for the diagnosis and classification of various immunobullous disorders. DIF test should be done in conjunction with histopathology for definitive diagnosis and to minimize both: False-positive and false-negative results.

  1. Scabies masquerading as bullous pemphigoid: scabies surrepticius

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    Cohen PR

    2017-08-01

    Full Text Available Philip R Cohen Department of Dermatology, University of California San Diego, La Jolla, CA, USA Abstract: Scabies, a parasitic infestation caused by the mite Sarcoptes scabiei, is diagnosed by observing either the mite, its ova, or its excrement. The mite tracts, known as burrows and a characteristic presentation of the pruritic condition, are typically found on the web spaces between the fingers. Other cutaneous lesions include excoriated papules, pustules, and vesicles. However, atypical clinical variants of scabies, such as bullous, crusted, hidden, incognito, nodular, and scalp forms of the parasitic infestation, mimic the morphologic features of other non-parasitic dermatoses. A 76-year-old man presented with pruritic blisters and urticarial plaques that demonstrated not only pathology changes, but direct immunofluorescence also showed findings of bullous pemphigoid. His condition improved, but did not resolve, with topical corticosteroid cream for the management of the primary autoimmune blistering disorder. When other family members subsequently developed scabies, the correct diagnosis for his condition, bullous scabies, was established by demonstrating mites, ova, and scybala on a mineral oil preparation from a skin scraping of a newly appearing burrow. Bullous scabies can masquerade not only clinically, but also both pathologically and immunologically as bullous pemphigoid. Scabies serrupticius is introduced as a unifying term to designate all of the non-classic presentations of S. scabiei mite infestation. Keywords: bullous, crusted, egg, hidden, incognito, masquerade, mimic, mite, nodular, Norwegian, pemphigoid, Sarcoptes scabiei, scabies, scalp, scybala, surrepticius

  2. Exploring mechanisms of IgE-mediated autoimmunity through the lens of bullous pemphigoid.

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    Messingham, Kelly N; Randall, Grant; Fairley, Janet

    2016-04-01

    Bullous pemphigoid (BP) is the most common autoimmune blistering disease characterized by pathogenic autoantibodies targeting collagen XVII (col XVII), a hemidesmosomal adhesion molecule. Early studies utilizing IgG were critical for establishing col XVII-specific antibodies as primary mediators of blister formation; however, these studies lacked key features of the disease, including urticarial erythema and eosinophilic infiltration, which are often associated with IgE. Although it was recognized that BP patients often had elevated circulating IgE, investigations into the pathogenicity of these antibodies was delayed until discovery of col XVII-specific IgE in BP sera. Since then, a variety of in-vivo and in-vitro studies have provided clear evidence that IgE autoantibodies are a key component of BP. Furthermore, studies utilizing IgE receptor blockade in BP patients were the first to confirm a pathogenic role of IgE autoantibodies in human autoimmunity. In this review we will utilize BP as a prototypical autoimmune disease to better understand how IgE autoantibodies participate in human autoimmunity.

  3. Tissue Factor in Dermatitis Herpetiformis and Bullous Pemphigoid: Link between Immune and Coagulation System in Subepidermal Autoimmune Bullous Diseases

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    Agnieszka Zebrowska

    2015-01-01

    Full Text Available Dermatitis herpetiformis (DH and bullous pemphigoid (BP are skin diseases associated with eosinophilic and neutrophilic infiltrations. Although chemokines are critical for the selective accumulation and activation of various leukocyte subsets in the inflammatory process, there are few findings concerning inflammatory cells and production of coagulation factors in blistering diseases. Skin biopsies were taken from 14 patients with DH, 27 with BP, and 20 control subjects. The localization and expression of tissue factor (TF in skin lesions and perilesional skin were studied by immunohistochemistry and confirmed by Western Blot. Moreover the plasma concentrations of TF were measured by immunoassays. D dimers, fibrinogen, and selected coagulation parameters were measured by routine methods. Expression of TF in the epidermis and in inflammatory influxed cells in dermis was detected in skin biopsies from BP patients. Examined TF expression was detected in perilesional skin of all BP patients too. The expression of TF was not observed in biopsies from healthy people and DH patients. The findings of the study show an increased expression of tissue factor in the lesional and perilesional skin of patients with bullous pemphigoid. The difference in chemokine pattern expression and variations in the cellular infiltration in BP and DH cause variable expression of TF.

  4. Treatment of Intraepidermal Autoimmune Bullous Diseases Sürekli Eğitim

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    Tamer İrfan Kaya

    2011-06-01

    Full Text Available Pemfigus is an autoimmune bullous skin disease, characterized by intraepidermal blisters. It is a severe and potentially life-threatening chronic disease with blisters and erosions on the mucosae and the skin. Treatment options do not differ for two most common types of pemphigus, pemphigus vulgaris and pemphigus foliaceus, except that the latter is usually less resistant to treatment and corticosteroids can often be started at lower doses. Systemic corticosteroids are still the most widely used drugs in the treatment of pemphigus and continue to be the mainstay of therapy for this disease. Adjuvant drugs are commonly used in combination with the aims of increasing efficacy and of having a steroid-sparing action, thereby allowing reduced corticosteroid side-effects. Mortality and complete remission rates have improved since the introduction of adjuvant drugs to pemphigus. Adjuvant drugs include immunoadsorbtion, corticosteroid pulse therapy, intravenous immunoglobulin (IVIG, immunosuppressive agents such as azathioprine, cyclophosphamide, mycophenolate mofetil and and anti-CD20 monoclonal antibody (rituximab. The lack of consensus in the published literature about the treatment of this disorder is responsible for different treatment strategies. Treatments need to be chosen after careful consideration of the potential benefits and side effects according to the patients’ medical condition. Here, both conventional therapies and novel treatment regimens for pemphigus are discussed. (Turkderm 2011; 45 Suppl 1: 44-53

  5. Autoimmune dermatologic toxicities from immune checkpoint blockade with anti-PD-1 antibody therapy: a report on bullous skin eruptions.

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    Jour, George; Glitza, Isabella C; Ellis, Rachel M; Torres-Cabala, Carlos A; Tetzlaff, Michael T; Li, Janet Y; Nagarajan, Priyadharsini; Huen, Auris; Aung, Phyu P; Ivan, Doina; Drucker, Carol R; Prieto, Victor G; Rapini, Ronald P; Patel, Anisha; Curry, Jonathan L

    2016-08-01

    Monoclonal antibodies against the immune checkpoint programmed cell death receptor 1 (PD-1) improve the hosts' antitumor immune response and have showed tremendous promise in the treatment of advanced solid tumors and hematologic malignancies. Reports of serious autoimmune dermatologic toxicities from immune checkpoint blockade therapy, however, are emerging. We report our experience with five patients who presented with pruritic vesicles and blisters on the skin while treated with anti-PD-1 antibody immunotherapy with either nivolumab or pembrolizumab. Four of the patients' skin biopsies revealed subepidermal bullae with immunohistochemical study for type IV collagen labeling the floor of the blister cavity and direct immunofluorescence studies (in three of the four patients tested) decorated linear IgG and C3 immune deposits on the blister roof, diagnostic of bullous pemphigoid. One patient developed bullous erythema multiforme. All patients had partial or complete resolution of skin lesions following treatment with systemic corticosteroid and cessation of checkpoint blockade. Recognition and treatment of rare immune-related bullous dermatologic toxicities will become increasingly important as more patients are treated with effective and newer immune checkpoint blockade therapy. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  6. Epidemiology of Autoimmune Bullous Diseases Sürekli Eğitim

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    Erkan Alpsoy

    2011-06-01

    Full Text Available We have very limited knowledge about aotuimmune bullous disesases which are important causes of morbidity and mortality. They are generally rare disases in population. The yearly over all incidences of pemphigus and bullous pemhigoid are between 0.5 to 16.1/million and 2.5 to 42.8/million, respectively. Pemphigus vulgaris is the major type of pemphigus and it is most prevalent between ages of 40 and 50. Bullous pemphigoid is tpypically most prevalent in ages of over 70. In this review the results obtained from the studies which are especially about epidemiology of pemphigus and bullous pemphigoid were analised according to geographic regions. (Turkderm 2011; 45 Suppl 1: 3-7

  7. Treatment of Subepidermal Autoimmune Bullous Diseases Sürekli Eğitim

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    Ekin Şavk

    2011-06-01

    Full Text Available The common therapeutic goal for all subepidermal bullous diseases is to prevent inflammation and production of pathogenic autoantibodies and/or to facilitate the elimination of these antibodies. Diseases included in this group are the pemphigoids, linear IgA dermatosis, epidermolysis bullosa acquisita, dermatitis herpetiformis and bullous systemic erythematosus. Corticosteroids are the most commonly used systemic medication. Other alternatives include dapsone, various antibiotics and nicotinamide, azathioprine, cyclophosphamide, methotrexate, cycloporine, mycophenolate mofetil, IV immunoglobulins and plasmapheresis. Rapid reepithelialization and prevention of secondary infections are provided by local hygenic measures including antiseptic baths and wound care. (Turkderm 2011; 45 Suppl 1: 54-8

  8. Diagnosis and management of bullous disease.

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    Hurley, Maria Yadira; Mattox, Adam R

    2013-05-01

    As the population ages, the prevalence of bullous skin diseases will escalate. Efficient management depends on timely recognition by the physician and reduces the morbidity associated with the disease course. This article outlines the common bullous dermatoses affecting older adults and provides tips for a streamlined approach to workup and treatment.

  9. Penfigoide bolhoso no adulto mais jovem: relato de três casos Bullous pemphigoid in younger adults: three case reports

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    Roberta Richter Zanella

    2011-04-01

    Full Text Available O penfigoide bolhoso é uma dermatose bolhosa autoimune subepidérmica, mais comumente observada na população idosa (acima dos 70 anos. Autoanticorpos são formados contra antígenos específicos da zona de membrana basal: BP180 e BP230 (proteínas do hemidesmossomo. Apresentamos três casos de penfigoide bolhoso, em adultos com menos de 50 anos de idade, destacan do as peculiaridades clínicas na faixa etária mais jovem.Bullous pemphigoid is an autoimmune subepidermal bullous dermatosis more commonly observed in the elderly (over 70 years old. Autoantibodies are produced for specific antigens of the epidermal basement membrane zone: BP 180 and BP 230 (hemidesmosome proteins. We report three cases of bullous pemphigoid in adults younger than 50 years old, discussing the clinical characteristics of the disease in younger patients.

  10. Autoimmune bullous diseases with skin and eye involvement: Cicatricial pemphigoid, pemphigus vulgaris, and pemphigus paraneoplastica.

    Science.gov (United States)

    Broussard, Karen C; Leung, Theresa G; Moradi, Ahmadreza; Thorne, Jennifer E; Fine, Jo-David

    2016-01-01

    Autoimmune blistering diseases are a heterogeneous group of disorders that mostly affect the skin and mucous membranes. Occasionally, other organ systems may be involved, depending on the unique pathophysiology of each disease. Cicatricial pemphigoid, pemphigus vulgaris, and paraneoplastic pemphigus are distinct entities, but all have the potential to have cutaneous and ocular involvement. Awareness and early recognition of ocular involvement in these diseases is important given the increased risk for vision loss and blindness with delay in management. Several skin diseases may be associated with involvement of the external eye. The most common autoimmune diseases are cicatricial pemphigoid, pemphigus vulgaris, and paraneoplastic pemphigus.

  11. Adjuvant drugs in autoimmune bullous diseases, efficacy versus safety: Facts and controversies.

    Science.gov (United States)

    Schiavo, Ada Lo; Puca, Rosa Valentina; Ruocco, Vincenzo; Ruocco, Eleonora

    2010-01-01

    During the last decades, the conventional therapy for autoimmune blistering diseases has been high-dose, long-term systemic corticosteroid and immunosuppressive agents or adjuvant drugs. Long-term, high-dose steroid therapy can result in serious adverse effects. The rationale for using adjuvant drugs is that concerns reducing the need for corticosteroids, and hence, their side effects, or it may result in better control of the disease, or both. Immunosuppressive agents are not free of adverse effects, however. Prolonged immune suppression may account for high rates of morbidity, disability, and possible death. There is no consensus about the first-choice adjuvant drug for the management of blistering autoimmune diseases. This contribution evaluates six adjuvant drugs-cyclophosphamide, azathioprine, cyclosporine, mycophenolate mofetil, intravenous immunoglobulin, and rituximab-and discusses the choice of a "winning drug" that is effective and safe.

  12. Obstetriske dermatoser

    DEFF Research Database (Denmark)

    Hjortø, Sofie; Skov, Lone; Lykke, Jacob Alexander

    2014-01-01

    and ICP increase the risk of prematurity, fetal distress and stillbirth. Diagnosis is based on medical history, morphology, blood sample and biopsy. The dermatoses are treated with respectively ursodeoxycholic acid (in case of ICP) and steroids. Breast-feeding is recommended and induction of labour...

  13. Obstetric dermatoses

    DEFF Research Database (Denmark)

    Hjortø, Sofie; Skov, Lone; Lykke, Jacob Alexander

    2014-01-01

    and ICP increase the risk of prematurity, fetal distress and stillbirth. Diagnosis is based on medical history, morphology, blood sample and biopsy. The dermatoses are treated with respectively ursodeoxycholic acid (in case of ICP) and steroids. Breast-feeding is recommended and induction of labour...

  14. A cross-sectional study of direct immunofluorescence in the diagnosis of immunobullous dermatoses

    Directory of Open Access Journals (Sweden)

    Archana C Buch

    2014-01-01

    Full Text Available Background: Autoimmune blistering diseases are a group of bullous disorders characterized by pathogenic antibodies directed at the target antigens, which are components of the desmosomes or adhesion complex at the dermoepidermal junction. Direct immunofluorescence (DIF is invaluable in the diagnosis of these lesions. Aim: The aim of this study was to evaluate the sensitivity of DIF in immunobullous dermatoses and to study the pattern of DIF. The study also aims to correlate DIF with clinical and histologic findings and to analyze discrepancies. Materials and Methods: Total 100 biopsies received over a period of 2 years in the Department of Pathology were analyzed. DIF, histopathology and clinical data were reviewed. Results: Out of 100, 89 cases showed DIF patterns concordant with clinical/histologic diagnosis. The sensitivity of DIF was 94.44% (51/58 in the pemphigus and 84% (21/25 in the bullous pemphigoid (BP group, 100% each in dermatitis herpetiformis (2/2 and linear IgA disease (1/1. A total of 11 histologically proven cases of immunobullous lesions were DIF negative-four (three of pemphigus vulgaris and one of BP due to having no epidermis, three (cases of BP owing to sampling/technical errors and the remaining four (cases of pemphigus vulgaris due to being on treatment. Conclusion: Immunofluorescence helps confirm the diagnosis of bullous lesions in which there is clinical and the histopathologic overlap. Sampling errors contributed to false negative (FN results.

  15. A necessidade da imunofluorescência direta no diagnóstico da dermatose bolhosa por IgA The need for direct immunofluorescence in the diagnosis of IgA bullous dermatosis

    OpenAIRE

    Daniel Chang

    2012-01-01

    A dermatose bolhosa por imunoglobulina da classe A linear (DbIgA) do adulto é uma doença autoimune rara caracterizada por formação de bolhas subepidérmicas e depósito linear de imunoglobulina da classe A (IgA) na zona da membrana basal (ZMB). Por possuir aspectos clínicos e histológicos semelhantes a outras dermatoses bolhosas, principalmente a dermatite herpetiforme e o penfigoide bolhoso, faz-se necessária a realização de imunofluorescência direta para confirmação diagnóstica. Apresenta-se ...

  16. Linear IgA Bullous Dermatosis

    DEFF Research Database (Denmark)

    Lings, Kristina; Bygum, Anette

    2015-01-01

    Linear IgA bullous dermatosis (LAD) is an autoimmune, chronic bullous disease affecting primarily young children and adults. Studies on LAD are relatively sparse and from Scandinavia we could only find a few case reports. Therefore we decided to conduct a retrospective investigation of patients...

  17. Bullous pemphigoid

    Science.gov (United States)

    Bernard P, Borradori L. Pemphigoid group. In: Bolognia JL, Jorizzo JL, Schaffer JV, eds. Dermatology. 3rd ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 30. Scott M, Werth VP. Bullous pemphigoid. In: ...

  18. Serological diagnosis of autoimmune bullous skin diseases: Prospective comparison of the BIOCHIP mosaic-based indirect immunofluorescence technique with the conventional multi-step single test strategy

    Directory of Open Access Journals (Sweden)

    van Beek Nina

    2012-08-01

    Full Text Available Abstract Background Various antigen-specific immunoassays are available for the serological diagnosis of autoimmune bullous diseases. However, a spectrum of different tissue-based and monovalent antigen-specific assays is required to establish the diagnosis. BIOCHIP mosaics consisting of different antigen substrates allow polyvalent immunofluorescence (IF tests and provide antibody profiles in a single incubation. Methods Slides for indirect IF were prepared, containing BIOCHIPS with the following test substrates in each reaction field: monkey esophagus, primate salt-split skin, antigen dots of tetrameric BP180-NC16A as well as desmoglein 1-, desmoglein 3-, and BP230gC-expressing human HEK293 cells. This BIOCHIP mosaic was probed using a large panel of sera from patients with pemphigus vulgaris (PV, n = 65, pemphigus foliaceus (PF, n = 50, bullous pemphigoid (BP, n = 42, and non-inflammatory skin diseases (n = 97 as well as from healthy blood donors (n = 100. Furthermore, to evaluate the usability in routine diagnostics, 454 consecutive sera from patients with suspected immunobullous disorders were prospectively analyzed in parallel using a the IF BIOCHIP mosaic and b a panel of single antibody assays as commonly used by specialized centers. Results Using the BIOCHIP mosaic, sensitivities of the desmoglein 1-, desmoglein 3-, and NC16A-specific substrates were 90%, 98.5% and 100%, respectively. BP230 was recognized by 54% of the BP sera. Specificities ranged from 98.2% to 100% for all substrates. In the prospective study, a high agreement was found between the results obtained by the BIOCHIP mosaic and the single test panel for the diagnosis of BP, PV, PF, and sera without serum autoantibodies (Cohen’s κ between 0.88 and 0.97. Conclusions The BIOCHIP mosaic contains sensitive and specific substrates for the indirect IF diagnosis of BP, PF, and PV. Its diagnostic accuracy is comparable with the conventional multi

  19. Novel feline autoimmune blistering disease resembling bullous pemphigoid in humans: IgG autoantibodies target the NC16A ectodomain of type XVII collagen (BP180/BPAG2).

    Science.gov (United States)

    Olivry, T; Chan, L S; Xu, L; Chace, P; Dunston, S M; Fahey, M; Marinkovich, M P

    1999-07-01

    In humans and dogs, bullous pemphigoid (BP) is an autoimmune blistering disease associated with the production of basement membrane autoantibodies that target the 180-kd type XVII collagen (BP180, BPAG2) and/or the 230-kd plakin epidermal isoform BPAG1e (BP230). In two adult cats, an acquired dermatosis and stomatitis was diagnosed as BP subsequent to the fulfillment of the following criteria: 1) presence of cutaneous vesicles, erosions, and ulcers; 2) histologic demonstration of subepidermal vesiculation with inflammatory cells, including eosinophils; 3) in vivo deposition of IgG autoantibodies at the epidermal basement membrane zone; and 4) serum IgG autoantibodies targeting a 180-kd epidermal protein identified as type XVII collagen. In both cats, the antigenic epitopes targeted by IgG autoantibodies were shown to be situated in the NC16A ectodomain of type XVII collagen, a situation similar to that of humans and dogs with BP. Feline BP therefore can be considered a clinical, histopathologic, and immunologic homologue of BP in humans and dogs.

  20. PREGNANCY DERMATOSES

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    Tina Priscilla Katta

    2016-11-01

    Full Text Available BACKGROUND Pregnancy is a physiological status of a woman. Every organ is adapting in order to accept another human body. The main changes occur in the endocrine, immune, metabolic and vascular systems. The skin is no exception. Many skin changes during pregnancy are considered to be normal or physiological including striae gravidarum or melasma. These physiological skin changes are usually well tolerated by the pregnant woman. There is no balance between these systems, however, and abnormalities can appear. Immunologic status of the woman plays an important role in the manifestations exhibited in the skin. Alterations of the skin during pregnancy can be classified as physiologic skin changes, changes in pre-existing skin diseases and specific dermatoses of pregnancy. MATERIALS AND METHODS 200 pregnant women with skin manifestations attending Dermatology and Obstetric departments attached to Government General Hospital, Kurnool, were studied. 1. A detailed proforma was taken, which included: a. Detailed history including chief complaints related to skin. b. Onset in relation to duration of pregnancy. c. Complete general physical and systemic examination. d. Associated skin/medical disorders. 2. Investigations-CBP, CUE, RBS, LFT, HBsAg, VDRL, HIV 1 and 2 were done routinely. 3. KOH mount, saline mount and skin biopsy performed wherever required. Inclusion criteria- All pregnant women having skin lesions were included in the study irrespective of the duration of pregnancy and gravidity. Exclusion criteria- Pregnant women having any underlying medical diseases. All pregnant women attending antenatal OPD and those admitted into wards having symptoms related to skin and mucosa, at KIMS Hospital are studied. 1. Detailed history including chief complaints related to skin. 2. Onset in relation to duration of pregnancy. 3. Complete general physical and systemic examination. 4. Associated skin/medical disorders. 5. Investigations-CBP, CUE, RBS, LFT, HBs

  1. Immunofluorescence patterns in selected dermatoses, including blistering skin diseases utilizing multiple fluorochromes

    Directory of Open Access Journals (Sweden)

    Ana Maria Abreu-Velez

    2015-01-01

    Full Text Available Background: Autoimmune vesiculobullous disorders represent a heterogeneous group of dermatoses whose diagnosis is made based on clinical history, histologic features, and immunopathologic features. The most commonly used techniques for the diagnosis of these diseases are direct and indirect immunofluorescence (DIF and IIF, including salt-split processing. NaCl split skin is used to determine the level of blister formation, and the localization of autoantibodies relative to the split. Classically, immunofluorescence has been performed with one fluorochrome in the diagnosis of autoimmune bullous skin diseases. Aims: To compare DIF and IIF of the skin, using a single fluorochrome versus multiple fluorochromes. Materials and Methods: We studied 20 autoimmune skin disease cases using fluorescein isothiocyanate (FITC alone, in comparison to multiple fluorochromes (with or without DNA counterstaining. Results: The use of multiple fluorochromes helped to simultaneously visualize reactivity in multiple skin areas, in contrast to using FITC alone. Conclusions: Using multiple fluorochromes allows simultaneous labeling of two or more antigens within the same cell/or tissue section, assists in colocalization of unknown antigens with known molecules, and helps in ruling out "background" staining.

  2. Dermatose por IgA linear induzida pela gestação Linear IgA dermatosis induced by pregnancy

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    Telma Kanagusuko

    2008-02-01

    Full Text Available A dermatose por IgA linear é doença bolhosa auto-imune subepidérmica rara, caracterizada pelo depósito linear de IgA na zona da membrana basal da epiderme. Nos relatos de gestação em pacientes com essa dermatose, nota-se sempre melhora do quadro clínico. Contrariando essas observações,é apresentado caso de dermatose por IgA linear induzida pela gestação, que demonstrou boa resposta terapêutica à dapsona e prednisona , sem complicações materno-fetais.Linear IgA disease is a rare autoimmune subepidermal bullous disorder characterized by linear IgA deposits at the epidermal basement membrane zone. According to the literature, in patients who have linear IgA disease and become pregnant, the disease tends to improve. We report a case of linear IgA disease induced by pregnancy, successfully treated with dapsone and prednisone with no adverse effects observed in the patient and her newborns.

  3. Dermatose por IgA e IgG linear: relato de caso com boa resposta terapêutica à dapsona e ao micofenolato mofetil Linear IgA/IgG bullous dermatosis: successful treatment with dapsone and mycophenolate mofetil

    Directory of Open Access Journals (Sweden)

    Leny Passos

    2011-08-01

    Full Text Available Relata-se o caso de paciente feminina, de 21 anos, com dermatose por IgA e IgG linear. Inicialmente, a resposta clínica foi favorável à dapsona. Após a interrupção desta medicação, por crise de anemia sintomática, precipitada por malária, houve piora da doença, apesar da utilização da prednisona e pulsoterapia com metilprednisolona. A reintrodução da dapsona, associada ao micofenolato mofetil, possibilitou o controle da enfermidadeA 21-year-old female presenting linear IgA and IgG disease initially responded well to dapsone therapy. However, the treatment with dapsone was withdrawn due to severe anemia induced by malaria, which led to worsening of the clinical picture. Although prednisone and methylprednisolone were tried, the patient responded only to the association of dapsone and mycophenolate mofetil

  4. Neutrophilic dermatoses in children.

    Science.gov (United States)

    Berk, David R; Bayliss, Susan J

    2008-01-01

    The neutrophilic dermatoses are rare disorders, especially in children, and are characterized by neutrophilic infiltrates in the skin and less commonly in extracutaneous tissue. The neutrophilic dermatoses share similar clinical appearances and associated conditions, including inflammatory bowel disease, malignancies, and medications. Overlap forms of disease demonstrating features of multiple neutrophilic dermatoses may be seen. The manuscript attempts to provide an up-to-date review of (i) classical neutrophilic dermatoses, focusing on distinctive features in children and (ii) neutrophilic dermatoses which may largely be pediatric or genodermatosis-associated (Majeed, SAPHO [synovitis, severe acne, sterile palmoplantar pustulosis, hyperostosis, and osteitis] syndrome, PAPA (pyogenic sterile arthritis, pyoderma gangrenosum, and acne), PFAPA (periodic fever with aphthous stomatitis, pharyngitis, and cervical adenopathy), and other periodic fever syndromes, and congenital erosive and vesicular dermatosis healing with reticulated supple scarring).

  5. Linear IgA bullous dermatosis: report of an exuberant case.

    Science.gov (United States)

    Souza, Beatriz Cavalcanti de; Fregonesi, Nádire Cristina Freire Pontes; Tebcherani, Antônio José; Sanchez, Ana Paula Galli; Aoki, Valéria; Fernandes, Juliana Christien

    2013-01-01

    Linear immunoglobulin A dermatosis is a rare autoimmune bullous disease, but the most common autoimmune bullous dermatosis in children. We report a typical exuberant case of linear IgA dermatosis in a ten-month old child, who showed good response to treatment with corticosteroids and dapsone.

  6. Linear IgA bullous dermatosis: report of an exuberant case*

    OpenAIRE

    Souza,Beatriz Cavalcanti de; Fregonesi, Nádire Cristina Freire Pontes; Tebcherani,Antônio José; Sanchez,Ana Paula Galli; AOKI, Valéria; Fernandes, Juliana Christien

    2013-01-01

    Linear immunoglobulin A dermatosis is a rare autoimmune bullous disease, but the most common autoimmune bullous dermatosis in children. We report a typical exuberant case of linear IgA dermatosis in a ten-month old child, who showed good response to treatment with corticosteroids and dapsone.

  7. 自身免疫性大疱性皮肤病评分系统在天疱疮治疗中的应用%The application of autoimmune bullous skin disorders intensity score in the treatment of pemphigus

    Institute of Scientific and Technical Information of China (English)

    阳艺; 郭宜青; 周红梅

    2013-01-01

    Pemphigus is a kind of serious, chronic skin-mucosa bullous disorder. Glucocorticoid, as the first choice drug, could reduce the mortality of the disease largely, while long-term use could lead to fatal adverse reaction. Series of evaluation system have been put forward by foreign scholars, which contribute significantly to disease and drug efficacy assessment and monitoring. Autoimmune bullous skin disorders intensity score system in this paper is a relatively accepted system, and some suggestions for improvement would be put forward according to our clinical practice.%  天疱疮是一类严重的、慢性皮肤-黏膜大疱性疾病,糖皮质激素是其治疗的首选药物,但长期用药可能导致较严重的并发症甚至死亡。近年来,国外学者提出一系列天疱疮评估系统,有助于较准确评估病情、客观评价药物疗效、监测病情转归等。本文将重点介绍较受公认的自身免疫性大疱性皮肤病评分系统,并根据临床实践对其提出思考和改进意见。

  8. [Acquired bullous diseases of the oral mucosa].

    Science.gov (United States)

    Vaillant, L; Hüttenberger, B

    2005-11-01

    Bullous diseases of the oral cavity cause painful erosion. They must be distinguished from aphthae and vesicles which may have a similar presentation. Acute, chronic and congenital conditions are recognized. Acute lesions may involve a polymorphous oral erhythema which has an polymorphous erythematous presentation or toxidermia (Stevens-Johnson syndrome, Lyell syndrome, fixed pigmented erythema). Examination of the skin and history taking are the keys to diagnosis. Patients with chronic bullous diseases may have a congenital condition (bullous epidermolysis or lymphangioma) suggested by the age at onset and the clinical presentation. Acquired chronic bullous diseases include lichen planus and autoimmune bullous diseases. Careful examination is essential to identify mucosal or cutaneous involvement and to obtain a biopsy for histological examination. Search for antibodies deposited in the perilesional mucosa is necessary. Chronic erosive gingivitis is a frequent presentation. Most of the patients have cicatricial pemphigoid, lichen planus, and more rarely pemphigus. The pinch sign is highly discriminative to differentiate the cause of this syndrome. Symptomatic treatment of bullous lesions of the oral cavity include adapted diet and correct and early use of antalgesics.

  9. High-Frequency Color Doppler Sonography of Bullous Pemphigoid: Correlation With Histologic Findings.

    Science.gov (United States)

    Porriño-Bustamante, María Librada; Alfageme, Fernando; Suárez, Lola; de Domingo, María Antonia González; Hospital, Mercedes; Roustán, Gastón

    2016-08-01

    Bullous pemphigoid is the most frequent autoimmune-mediated blistering skin disease, belonging to the group of subepidermal bullae. We performed high-frequency color Doppler sonography in 3 cases of bullous pemphigoid, in bullous and adjacent non-bullous skin, which showed homogeneous sonographic findings. Subepidermal cystic structures with dermal hypoechogenicity were observed in bullous skin. In nonbullous skin, the dermis showed hypoechogenicity compared to normal skin. Color Doppler signals were increased in both areas. These findings correlate histologically with subepidermal bullae and dermal inflammatory infiltrates.

  10. The dermatoses of pregnancy

    Directory of Open Access Journals (Sweden)

    Sachdeva Silonie

    2008-01-01

    Full Text Available The skin changes in pregnancy can be either physiological (hormonal, changes in pre-existing skin diseases or development of new pregnancy specific dermatoses. Pregnancy-specific skin dermatoses include an ill-defined heterogeneous group of pruritic skin eruptions which are seen only in pregnancy. These include atopic eruption of pregnancy, polymorphic eruption of pregnancy, pemphigoid gestationis and intrahepatic cholestasis of pregnancy. Atopic eruption of pregnancy is the most common of these disorders. Most skin eruptions resolve postpartum and require only symptomatic treatment. Antepartum surveillance is recommended for patients with pemphigoid gestationis and intrahepatic cholestasis of pregnancy as they carry fetal risk. This article deals with the classification, clinical features and treatment of the specific dermatoses of pregnancy.

  11. Clinical analysis on 11 patients with psoriasis vulgaris coexisted with autoimmune bullous diseases%银屑病并发免疫性大疱性皮肤病11例临床分析

    Institute of Scientific and Technical Information of China (English)

    董娟; 吴桂菊; 郑捷; 潘萌

    2012-01-01

    Objective: To analyze the clinical features, relevant factors of the patients with psoriasis vulgaris coexisted with autoimmune bullous disease. Methods: 11 patients with psoriasis vulgaris coexisted with autoimmune bullous disease were collected, and retrospectively analyzed on clinical manifestations, triggering factors, pathological features and the treatment of them. The specific antibodies were also detected. Results: Among the patients, the ratio of male to female was 10:1. The average age was 70.55±11.08 years old. The average disease duration of psoriasis was 15.36±9.19 years. Four patients had a definite NB-U-VB therapy before. Seven patients were diagnosed as pemphigus, among which four showed positive IF, two negative but had high titers of anti-Dsgl antibody. Four patients were diagnosed as pemphigoid, among which three showed positive IF, two had high titers of anti-BP180 antibody. Low-dose glucocorticoids combined with immunosuppressive agents were used for the treatment. Conclusions; It is not common for the patients with psoriasis coexsisted with autoimmune bullous disease. We should make the diagnosis earlier and provide a proper treatment for these patients.%目的:对银屑病并发免疫性大疱性皮肤病患者的临床相关因素及其临床特征进行回顾性分析.方法:收集11例银屑病并发免疫性大疱性皮肤病患者资料,对其临床特征、大疱病的诱发因素、免疫病理以及治疗等方面进行回顾性分析;并对部分患者的特异性致病性抗体进行检测.结果:11例患者中1例为女性,10例为男性,平均年龄(70.55±11.08)岁.患者在银屑病发生后平均(15.36±9.19)年出现大疱性皮肤病;4例患者在出现大疱病前有明确的窄谱中波紫外线(NB-UVB)照射史.7例患者并发天疱疮,4例并发类天疱疮.7例天疱疮患者中4例免疫荧光检查阳性,而阴性的2例患者中外周血检出高滴度抗Dsg1抗体,另有1例结果不详;4

  12. Linear IgA bullous dermatosis in a patient with renal cell carcinoma

    NARCIS (Netherlands)

    Van der Waal, RIF; Van de Scheur, MR; Pas, HH; Jonkman, MF; Van Groeningen, CJ; Nieboer, C; Starink, TM

    2001-01-01

    Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal bullous disease with heterogeneous clinical manifestations, characterized by linear deposition of IgA along the epidermal basement membrane zone. We report a patient with a metastasized renal cell carcinoma who developed an extensive

  13. Bullous Systemic Lupus Erythematosus: Case report

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    Miziara, Ivan Dieb

    2014-01-01

    Full Text Available Introduction: Bullous systemic lupus erythematosus (BSLE is an autoantibody-mediated disease with subepidermal blisters. It is a rare form of presentation of SLE that occurs in less than 5% of cases of lupus. Case Report: A 27-year-old, female, FRS patient reported the appearance of painful bullous lesions in the left nasal wing and left buccal mucosa that displayed sudden and rapid growth. She sought advice from emergency dermatology staff 15 days after onset and was hospitalized with suspected bullous disease. Intravenous antibiotics and steroids were administered initially, but the patient showed no improvement during hospitalization. She displayed further extensive injuries to the trunk, axillae, and vulva as well as disruption of the bullous lesions, which remained as hyperemic scars. Incisional biopsy of a lesion in the left buccal mucosa was performed, and pathological results indicated mucositis with extensive erosion and the presence of a predominantly neutrophilic infiltrate with degeneration of basal cells and apoptotic keratinocytes. Under direct immunofluorescence, the skin showed anti-IgA, anti-IgM, and anti-IgG linear fluorescence on the continuous dermal side of the cleavage. Indirect immunofluorescence of the skin showed conjugated anti-IgA, was anti-IgM negative, and displayed pemphigus in conjunction with anti-IgG fluorescence in the nucleus of keratinocytes, consistent with a diagnosis of bullous lupus erythematosus. Discussion: BSLE is an acquired autoimmune bullous disease caused by autoantibodies against type VII collagen or other components of the junctional zone, epidermis, and dermis. It must be differentiated from the secondary bubbles and vacuolar degeneration of the basement membrane that may occur in acute and subacute cutaneous lupus erythematosus.

  14. Bullous Systemic Lupus Erythematosus: Case report

    Science.gov (United States)

    Miziara, Ivan Dieb; Mahmoud, Ali; Chagury, Azis Arruda; Alves, Ricardo Dourado

    2013-01-01

    Summary Introduction: Bullous systemic lupus erythematosus (BSLE) is an autoantibody-mediated disease with subepidermal blisters. It is a rare form of presentation of SLE that occurs in less than 5% of cases of lupus. Case Report: A 27-year-old, female, FRS patient reported the appearance of painful bullous lesions in the left nasal wing and left buccal mucosa that displayed sudden and rapid growth. She sought advice from emergency dermatology staff 15 days after onset and was hospitalized with suspected bullous disease. Intravenous antibiotics and steroids were administered initially, but the patient showed no improvement during hospitalization. She displayed further extensive injuries to the trunk, axillae, and vulva as well as disruption of the bullous lesions, which remained as hyperemic scars. Incisional biopsy of a lesion in the left buccal mucosa was performed, and pathological results indicated mucositis with extensive erosion and the presence of a predominantly neutrophilic infiltrate with degeneration of basal cells and apoptotic keratinocytes. Under direct immunofluorescence, the skin showed anti-IgA, anti-IgM, and anti-IgG linear fluorescence on the continuous dermal side of the cleavage. Indirect immunofluorescence of the skin showed conjugated anti-IgA, was anti-IgM negative, and displayed pemphigus in conjunction with anti-IgG fluorescence in the nucleus of keratinocytes, consistent with a diagnosis of bullous lupus erythematosus. Discussion: BSLE is an acquired autoimmune bullous disease caused by autoantibodies against type VII collagen or other components of the junctional zone, epidermis, and dermis. It must be differentiated from the secondary bubbles and vacuolar degeneration of the basement membrane that may occur in acute and subacute cutaneous lupus erythematosus. PMID:25992032

  15. [Linear IgA bullous dermatosis of childhood: case report].

    Science.gov (United States)

    Reyes-Baraona, Francisco; Andino, Romina; Carrasco, Juan Eduardo; Arriagada, Camila; Guerrero, Silvia

    2014-04-01

    Linear IgA bullous dermatosis is a rare acquired autoinmune vesiculobullous disease characterized by linear IgA deposit on the dermo-epidermal basement membrane observed with direct inmunofluorescence. The characteristic lesions are vesicles and tense serous bullae, which most often are grouped giving a "cluster of jewels" appearance. Differential diagnosis must be established with other autoimmune dermatosis, such as dermatitis herpetiformis and bullous pemphigoid. Dapsone is the first line therapy, with excellent response in a short period. This is a benign disease that tends to wax and wane in severity until it disappears spontaneously. We report the case of a 5-year-old girl presenting with bullous lesions, being diagnosed a linear IgA bullous dermatosis, with excellent response to dapsone in less than 2 weeks.

  16. Linear immunoglobulin A bullous dermatosis.

    Science.gov (United States)

    Fortuna, Giulio; Marinkovich, M Peter

    2012-01-01

    Linear immunoglobulin A (IgA) bullous dermatosis, also known as linear IgA disease, is an autoimmune mucocutaneous disorder characterized by subepithelial bullae, with IgA autoantibodies directed against several different antigens in the basement membrane zone. Its immunopathologic characteristic resides in the presence of a continuous linear IgA deposit along the basement membrane zone, which is clearly visible on direct immunofluorescence. This disorder shows different clinical features and distribution when adult-onset of linear IgA disease is compared with childhood-onset. Diagnosis is achieved via clinical, histopathologic, and immunopathologic examinations. Two common therapies are dapsone and sulfapyridine, which reduce the inflammatory response and achieve disease remission in a variable period of time.

  17. Neutrophilic dermatoses and inflammatory bowel diseases.

    Science.gov (United States)

    Marzano, A V; Menicanti, C; Crosti, C; Trevisan, V

    2013-04-01

    Pyoderma gangrenosum (PG) and Sweet's Syndrome (SS) are inflammatory skin diseases caused by the accumulation of neutrophils in the skin and, rarely, in internal organs, which led to coining the term of neutrophilic dermatoses (ND) to define these conditions. Recently, ND have been included among the autoinflammatory diseases, which are forms due to mutations of genes regulating the innate immune responses. Both PG and SS are frequently associated with inflammatory bowel diseases (IBD), a group of chronic intestinal disorders which comprises ulcerative colitis and Crohn's disease and whose pathogenesis involves both the innate and adaptive immunity in genetically prone individuals. Patients with IBD develop PG in 1-3% of cases, while SS is rarer. PG presents with deep erythematous-to-violaceous painful ulcers with undermined borders, but bullous, pustular, and vegetative variants can also occur. SS, also known as acute febrile neutrophilic dermatosis, is characterized by the abrupt onset of fever, peripheral neutrophilia, tender erythematous skin lesions and a diffuse neutrophilic dermal infiltrate. In this review that will be focused on PG and SS, we will describe also the aseptic abscesses syndrome, a new entity within the spectrum of ND which frequently occurs in association with IBD and is characterized by deep abscesses mainly involving the spleen and skin and by polymorphic cutaneous manifestations including PG- and SS-like lesions.

  18. Occupational dermatoses: An Asian perspective

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    Riti Bhatia

    2017-01-01

    Full Text Available Occupational dermatoses contribute to a significant portion of work-related diseases, especially in Asia, where a major portion of the workforce is in the unorganized sector. This review article is focussed on the frequency and pattern of occupational skin diseases reported across Asian countries and type of allergens implicated in different occupations. The literature was searched systematically using key words 'occupational dermatoses,' 'occupational skin disease' and name of each Asian country. Ninty five full-text articles were considered relevant and evaluated. Some of the dermatoses seen in industrial workers in Asian countries are similar to those in Western countries, including dermatoses due to chromate in construction and electroplating workers, epoxy resin, and chromate in painters, wood dust in workers in the furniture industry, azo dyes in textile workers and formaldehyde and chromates in those working in the leather and dyeing industries, dermatoses in domestic workers, chefs and health-care workers. Dermatoses in workers engaged in agriculture, beedi (tiny cigars manufacture, agarbatti (incense sticks production, fish processing, carpet weaving, sanitation and those working in coffee plantations and coal mines appear to be unique to Asian countries. Recognition of clinical patterns and geographic variations in occupational skin diseases will provide an impetus to further strengthen future research in these areas, as well as improving their management.

  19. Diffuse Bullous Eruptions in an Elderly Woman: Late-Onset Bullous Systemic Lupus Erythematosus

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    Prajwal Boddu

    2016-10-01

    Full Text Available Vesiculobullous eruptions in the elderly represent a diverse range of varying pathophysiologies and can present a significant clinical dilemma to the diagnostician. Diagnosis requires a careful review of clinical history, attention to detail on physical and histomorphological examination, and appropriate immunofluorescence testing. We describe the case of a 73-year-old female who presented to our hospital with a painful blistering skin rash developed over 2 days. Examination of the skin was remarkable for numerous flaccid hemorrhagic bullae on a normal-appearing nonerythematous skin involving both the upper and lower extremities. Histopathology of the biopsy lesion showed interface change at the epidermo-dermal region with subepidermal blister formation, mild dermal fibrosis, and sparse interstitial neutrophilic infiltrate. Immunohistological analysis was significant for positive IgG basement membrane zone antibodies with a dermal pattern of localization on direct immunofluorescence and positive IgG antinuclear antibodies on indirect immunofluorescence. Evidence of antibodies to type VII collagen suggested the diagnosis of epidermolysis bullosa acquisita versus bullous systemic lupus erythematosus (BSLE. A diagnosis of BSLE was made based on positive American College of Rheumatology criteria, acquired vesiculo-bullous eruptions with compatible histopathological and immunofluorescence findings. This case illustrates one of many difficulties a physician encounters while arriving at a diagnosis from a myriad of immunobullous dermatoses. Also, it is important for internists and dermatologists alike to be aware of and differentiate this uncommon and nonspecific cutaneous SLE manifestation from a myriad of disorders presenting with vesiculobullous skin eruptions in the elderly.

  20. AUTOIMMUNE BASEMENT MEMBRANE AND SUBEPIDERMAL BLISTERING DISEASES

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    Ana Maria Abreu Velez

    2013-11-01

    Full Text Available Autoimmune mucocutaneous blistering diseases (ABDs represent a group of conditions that manifest with blisters on the skin and/or mucous membranes. Bullous pemphigoid (BP is the most common autoimmune mucocutaneous blistering disease. In BP, the location of the blisters is subepidermal and the oral involvement is rare. Variants of BP have been described, including pemphigoid vegetans; however, this disease is not completely characterized. The majority of ABDs have blisters and/or vesicles, that are often pruritic, and manifest autoantibodies to diverse proteins. These proteins include 1 hemidesmosomal plaque proteins(ie, BP230, plectins, 2 transmembrane proteins such as BP180 and α6β4-integrin, which are connected via laminin 332 to type VII collagen and 3 currently uncharacterized 105 kDa and 200 kDa molecules. Other ABDs include drug-induced linear IgA disease, bullous systemic lupus erythematosus (BSLE, dermatitis herpetiformis (DH, cicatricial pemphigoid (CP; also termed mucous membrane pemphigoid, lichen planus pemphigoides (LPP, pemphigoid gestationis (PG, herpes gestationis(HG, chronic bullous dermatosis of childhood (CBDC and the localized forms of CP, such as Brunsting-Perry pemphigoid. The diagnosis of ABDs requires clinical data; skin biopsies (in 10% buffered formalin for hematoxylin and eosin (H&E examination and skin biopsies(in Michel’s transport medium for direct immunofluorescence (DIF. In many ABDs, the histopathologic findings demonstrate a subepidermal vesicle or bulla with a luminal inflammatory infiltrate of neutrophils, eosinophils and/or lymphocytes. In many ABDs, an extensive perivascular and interstitial inflammatory infiltrate is also noted subjacent to the blister in the upper dermis. Normal skin adjacent to an ABD plaque is often excellent for DIF results. Many ABD biopsies reveal autoantibody deposition at the lesional basement membrane zone (BMZ; IgG, IgM, IgA, other immunoglobulins, complement components and

  1. Aquatic sports dermatoses: part 1. In the water: freshwater dermatoses.

    Science.gov (United States)

    Tlougan, Brook E; Podjasek, Joshua O; Adams, Brian B

    2010-08-01

    The first of this three-part series on water-related dermatoses involving the athlete will include sports occurring with the majority of time spent in the water. These sports include swimming, diving, scuba, snorkeling and water polo. Numerous authors have described dermatologic conditions commonly seen in swimmers. This series provides an updated and comprehensive review of these water dermatoses. In order to organize the vast number of skin conditions related to water exposure, we divided the skin conditions into groupings of infectious and organism-related dermatoses, irritant and allergic dermatoses and miscellaneous dermatoses. The vast majority of skin conditions involving the water athlete result from chemicals and microbes inhabiting each environment. When considering the effects of swimming on one's skin, it is also useful to differentiate between exposure to freshwater (lakes, ponds and swimming pools) and exposure to saltwater. This review will serve as a guide for dermatologists, sports medicine physicians and other medical practitioners in recognition and treatment of these conditions.

  2. Hemorrhagic bullous dermatosis: a rare heparin-induced cutaneous manifestation.

    Science.gov (United States)

    Govind, Bhuvanesh; Gnass, Esteban; Merli, Geno; Eraso, Luis

    2016-01-01

    Heparin is one of the most widely prescribed medications. Cutaneous reactions distant to the injection site are rare and under-reported in the literature. We present an elderly man with history of CNS lymphoma who underwent treatment of a deep venous thrombosis with enoxaparin and subsequently developed well demarcated bullous lesions within days of heparin initiation. The exact pathophysiology is not well understood. Hemorrhagic bullous dermatosis is a rare cutaneous reaction that is temporally associated with the initiation of heparin products. The handful of cases thus far suggest that regression of these seemingly benign lesions may or may not be associated with dose reduction or discontinuation of heparin products and typically occur within a few weeks. Elderly age appears to be one potential risk factor for development of these rare asymptomatic lesions. Malignancy may have some contributing factor and differentiation between this rare cutaneous manifestation from heparin products and other dermatological findings in patients with malignancy is key. Because of the asymptomatic and self-limiting nature of hemorrhagic bullous dermatoses in the setting of heparin product use, we presume that the reported incidence does not reflect true clinical practice.

  3. Erythema gyratum repense like eruption in bullous pemphigoid: A case report

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    Hojat Eftekhari

    2016-07-01

    Full Text Available Bullous pemphigoid (BP is an autoimmune disease characterized by presence of bullous eruption on the trunk and extremities especially flexural aspects of the limbs. This disease usually occurs in the elderly. The initial presentation of BP is variable. An urticarial or erythematous rash may precede the appearance of the blister formation and can be associated with itch or pruritus. We presented 87 year old bedridden man diagnosed with BP who initially presented with erythema gyratum repens like eruption before blister formation. This case report discusses the presentation of figurate erythema in non-bullous phase of BP for clinicians.

  4. [Retarded bullous dermographism].

    Science.gov (United States)

    Franco, N; Scaletzky, A; Cabo, H; Lutzky, F; Calb, I

    1982-01-01

    A case of what we denominate Bullous Delayed Dermographism in a woman who had neither allergic nor urticarial antecedents is registered. We comment the features of the lesions which appeared after a period of 4 to 12 hs. This lesions appeared by friction or percussion. We describe the existence of an endocrine and psychological predisposition mechanism and the non specificity of the histopathological characters. The nonexistence of bibliographical antecedents of the illness is noted. Also its difference with bullous and pressure urticaria. This differentiation is based on clinical features, and the nonexistence of allergic antecedents and urticarial lesions. Also on the existence of friction mechanism different to the simple pressure and the nonexistence of immunoglobulins in the subepidermal blister.

  5. Acrally distributed dermatoses: Vascular dermatoses (purpura and vasculitis).

    Science.gov (United States)

    Kazandjieva, Jana; Antonov, Dimitar; Kamarashev, Jivko; Tsankov, Nikolai

    Purpuric lesions appear in acral distribution in a variety of conditions and often provide clues to the clinical diagnosis. Purpuric means "hemorrhagic"-that is, the lesions do not blanch from pressure. This review focuses on dermatoses that produce hemorrhagic lesions in acral distribution from the large groups of the vasculitic diseases and their mimics. Cutaneous small vessel vasculitis is confined to the skin, involves mainly postcapillary venules, and has the hallmark manifestation of palpable purpura. Henoch-Schönlein purpura is an immune complex-mediated systemic vasculitis of the small vessels with manifestations from the skin, joints, kidneys, and gastrointestinal system. Only cases where the immune complexes contain immunoglobulin A type are classified as Henoch-Schönlein purpura. Cryoglobulinemic vasculitis is induced by the deposition of cold-precipitated immune complexes in the small vessels. Urticarial vasculitis comprises a spectrum of conditions with the characteristic course of chronic urticaria, with wheals that persist longer than 24 hours, leave hyperpigmentation, and have leukocytoclastic vasculitis on histologic examination. Polyarteritis nodosa is a rare multisystem, segmental necrotizing vasculitis of mainly the medium-sized vessels. Pigmented purpuric dermatoses are chronic benign dermatoses characterized by petechiae, purpura, and increased skin pigmentation. The hallmark of pigmented purpuric dermatoses is their orange-brown, speckled, cayenne pepper-like discoloration. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. Cyclic Catamenial Dermatoses

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    Trinh Hermanns-Lê

    2013-01-01

    Full Text Available Circulating sex hormones follow major fluctuations during the ovarian cycle. The so-called premenstrual syndrome represents a global condition grouping the diversity of catamenial disorders. At the skin level, the sebaceous gland activity is obviously modulated by these endocrine fluctuations. In addition, a series of pathological manifestations take place simultaneously in some women. Among them, the most frequent skin condition is represented by catamenial acne. Concurrently, the autoimmune progesterone dermatitis refers to a diversity of skin alterations resulting from an immune reaction to progesterone. It is present under variable clinical aspects. A series of other recurrent skin conditions are not specifically induced but are merely exacerbated at the end of the ovarian cycle.

  7. Bullous disorders in Konya: A study of 93 cases

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    Munise Daye

    2013-12-01

    Full Text Available Background and Design: Bullous disorders are a group of disorders caused by specific antibodies against tissue adhesion molecules in skin and mucosa or genetic inheritance. There are limited detailed epidemiological studies about bullous disorders in Turkey. In this study we evaluated the demographic, clinical, immunopathologic, course and follow-up properties, cases in Konya. Most of cases were in pemphigus and bullous pemphigoid group, so we gave more information about them. Materials and Methods: Ninty three cases that are followed up during the period of 2006-2012 were included in our study. Results: Female cases were %53,8, male cases were %46,2. The mean age was 52,9. In all of cases; %37,6 cases were pemhigus vulgaris, % 34,4 bullous pemphigoid, %15 herediter epidermolysis bullosa, %4,3 were dermatitis herpetiformis, %4,3 were pemphigus foliaceus, %2,2 pemphigus vegetans, %1,1 Hailey- Hailey disease, %1,1 cicatricial pemphigoid. Mean age of Pemhigus vulgaris onset was 45,9 year and mostly it was observed in females. Pemhigus vulgaris has been started at oral mucosae and the time for diagnosis of pemhigus vulgaris, which began at oral mucosae, was longer than other geogrophic regions. Mean age bullous pemphigoid onset was 70 year and mostly it was observed in females. In % 12,5 of cases disease started at oral mucosae. None of the cases had additional autoimmune disorder. We have treated 25 cases with systemic corticosteriods, 41 cases with systemic corticosteriods and adjuvan agents, 11 cases with only one of adjuvan agents, 16 cases with only local streoids. Conclusions: We report the demographic, clinical, immunopathologic, course and follow-up properties of the bullous disorders in Konya.

  8. 10-Year Observation of Bullous Pemphigoid Patients

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    Bengu Nisa Akay

    2010-06-01

    Full Text Available Background and Design: Bullous pemphigoid (BP is an autoimmune skin disorder characterized by large, tense blisters of the skin. It occurs mainly in the elderly. The one year mortality associated with bullous pemphigoid ranges from 19% to 41%. We aim to analyse the etiologic factors, demographic features, clinical course and response to treatment in patients with BP. Material and Method: Between January 1999 and January 2008, 31 patients with BP were evaluated retrospectively. We assessed duration of the disease, presence of associated symptoms, clinical findings, drug intake, response to treatment, recurrence rates and also extensive laboratory testing and radiologic evaluations on each patient to clarify a possible underlying malignancy. Results: In a 10-year period, 31 patients with BP, female male ratio was 19/12, ranging in age from 59 to 96 (mean 78.8±8.67 years were evaluated. Duration of the disease was 16.58±18.48 months. Direct immunoflourescence of the perilesional skin showed linear deposition of IgG and C3 along the dermoepidermal junction in 24 patients (77.4%. There was no significant relationship between drug usage and bullous pemphigoid. No malignancy was detected. Medium dosage of prednisolone (50-60 mg/day was the first choice of treatment in 8 patients with a complete response rate in all but two patients died during therapy. Four patients died during hospitalization with unknown reasons before any treatment was started. In 16 cases the initial treatment was prednisolone and azathioprine. The mean follow-up period was 12 months and 11 (35% cases had relapsed. The longest remission was achieved with prednisolone and azathioprine combination. Conclusion: In conclusion, no association was found between BP and drug usage or malignancy. All patients responded well to treatment. Therapy with prednisone maintained with azathiopyrine significantly reduced the recurrences. However, disease-specific mortality is found to be high and

  9. Systemic Lupus Erythematosus and Bullous Pemphigoid with Dramatic Response to Dapsone

    Science.gov (United States)

    Maggio, Maria Cristina; Corsello, Giovanni; Prinzi, Eugenia; Cimaz, Rolando

    2017-01-01

    Patient: Female, 11 Final Diagnosis: Bullous pemphigoid in systemic lupus erythematosus Symptoms: Bullous lupus • photosensitive rash • synovitis Medication:— Clinical Procedure: Pharmacological treatment Specialty: Rheumatology Objective: Unusual clinical course Background: Bullous pemphigoid is an autoimmune blistering disease, with relapses, isolated or associated with other autoimmune diseases such as systemic lupus erythematosus (SLE). Joint manifestations rapidly respond to small or moderate doses of corticosteroids, whereas skin manifestations usually respond to antimalarial drugs. Case Report: We describe the clinical case of an 11-year-old girl with SLE. She showed bullous skin lesions with arthralgia, mild proteinuria, resolved after steroid treatment. At the tapering of her prednisone dose, the patient had new skin lesions requiring an increased dose of prednisone. She started dapsone at the dosage of 1 mg/kg/day, maintaining low dose prednisone; this treatment was successfully followed by the dramatic disappearance of skin lesions and limb pain. Conclusions: Bullous skin lesions can represent the first clinical presentation of pediatric SLE and could influence the treatment and the outcome of these patients. This case showed an atypical course as both skin manifestations and arthritis promptly and persistently resolved with dapsone without the use of high-dose glucocorticoids. Only a few cases of patients with SLE associated with bullous pemphigoid have been reported in the literature, and very few in the pediatric population. PMID:28352068

  10. Tetracycline and niacinamide control bullous pemphigoid but not pemphigus foliaceus when these conditions coexist.

    Science.gov (United States)

    Shiohara, Junko; Yoshida, Kanako; Hasegawa, Junichi; Uhara, Hisashi; Takata, Minoru; Saida, Toshiaki; Ohyama, Bungo; Oyama, Bungo; Hashimoto, Takashi

    2010-07-01

    Pemphigus and pemphigoid are different types of autoimmune bullous disease and can occur in the same patient. We report a female patient with this condition. At first, we diagnosed her with bullous pemphigoid, and we treated her with tetracycline, niacinamide and a topical steroid. Tense bullas disappeared shortly after that, but crusted erythemas mainly on her head and trunk persisted. We examined BP180 and desmoglein 1 enzyme-linked immunosorbent assays, and also histological features, which showed coexistence of bullous pemphigoid and pemphigus foliaceus concurrently. Therefore, we tried prednisolone, which could control both conditions. This case showed that tetracycline and niacinamide could control bullous pemphigoid, but could not control pemphigus foliaceus, and that prednisolone was effective for both conditions.

  11. Drug-induced linear IgA bullous dermatosis.

    Science.gov (United States)

    Navi, Daniel; Michael, Daniel J; Fazel, Nasim

    2006-09-08

    A 73-year-old man was admitted to the University of California Davis Medical Center for treatment of a pleural effusion and congestive heart failure. His hospital course was complicated by asymptomatic sustained ventricular tachycardia requiring placement of an implantable cardiac defibrillator. The patient was treated with vancomycin and cefazolin during the procedure. After 3 days he developed tense vesicles over the dorsal aspect of the hands. Perilesional skin biopsy showed subepidermal cleavage with a neutrophilic infiltrate. Direct immunofluorescence revealed granular IgA and C3 deposition along the dermal epidermal junction. A diagnosis of drug-induced linear IgA bullous dermatosis secondary to vancomycin was established. Linear IgA bullous dermatosis is a rare autoimmune blistering disorder with clinical features that can overlap with bullous pemphigoid and dermatitis herpetiformis. Drug-induced linear IgA bullous dermatosis is a less common variant that is correspondingly less well characterized. Although a variety of medications have been implicated, vancomycin is the most common associated drug.

  12. Childhood linear IgA bullous dermatosis in Tunisia.

    Science.gov (United States)

    Kenani, Nesrine; Mebazaa, Amel; Denguezli, Mohamed; Ghariani, Najet; Sriha, Badreddine; Belajouza, Colandane; Nouira, Rafia

    2009-01-01

    The objective was to determine the demographic characteristics, the clinical features, the immuno-histological findings and response to treatment of childhood linear IgA bullous dermatosis (LABD) in Tunisia. We collected all the cases of auto-immune bullous diseases of childhood, diagnosed from January 1987 to December 2006. Based on clinical, histological, and immunofluorescent features, we identified 25 cases of LABD. Sixteen male and nine female children with a mean age of 7.5 years were identified. Clinical manifestations were characterized by a vesiculo-bullous eruption in all cases associated with mucous membrane involvement in two cases. Dapsone was the main therapy in 19 cases, associated with systemic corticosteroids in eight cases. Exclusive antibiotic therapy was successful in five cases. Sixteen of those patients had resolution of disease after a mean period of 15 months and eight patients had severe clinical presentation and required a prolonged follow-up. Childhood LABD is the most frequent bullous dermatosis in Tunisia. The majority of our patients responded rapidly to dapsone treatment and were stabilized for long time. Our cases were characterized by a minimal mucosal involvement and favorable outcome. Treatment with antibiotherapy was interesting. Erythromycin and oxacillin may be considered as an alternative therapy.

  13. Bullous pemphigoid after radiation therapy

    Energy Technology Data Exchange (ETDEWEB)

    Duschet, P.; Schwarz, T.; Gschnait, F.

    1988-02-01

    Electron beam therapy applied to a lymph node metastasis from a squamous cell carcinoma was followed by the development of histologically and immunologically typical bullous pemphigoid, the lesions being initially strictly confined to the irradiation area. This observation suggests that the bullous pemphigoid antigen may be altered or unmasked by electron beam radiotherapy, leading subsequently to the production of autoantibodies. The disease in this case effectively responded to the administration of tetracycline and niacinamide, a therapeutic regimen described recently.

  14. Tibolone induced Bullous pemphigoid.

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    Vishal. R. Tandon, Annil Mahajan* & Sudhaa Sharma**

    2008-01-01

    Full Text Available We present first ever report of Bullous pemphigoid induced by Tibolone, a STEAR (Selective tissueestrogenic activity regulator that has progestogenic, some androgenic as well as estrogenic effects prescribedas an alternative to estrogen replacement therapy for treatment of climacteric symptoms in one ofthe 51 year old postmenopausal women with one and half year duration since menopause with previoushistory of use of estrogen progesterone pills during her active sexual life. The mechanism for this ADR isnot well understood. But possible explanation could be progesterone activity of the drug leading to autoimmunityas reported previously. The present patient was managed by dechallenge of drug, local, oral corticosteroidsand injectable, methotrexate, along with folic acid and antibiotic coverage fearing anemia andsecondary infections.

  15. Autoimmunity-related neutrophilic dermatosis: a newly described entity that is not exclusive of systemic lupus erythematosus.

    Science.gov (United States)

    Saeb-Lima, Marcela; Charli-Joseph, Yann; Rodríguez-Acosta, Elva Dalia; Domínguez-Cherit, Judith

    2013-08-01

    Neutrophilic dermatoses have long been known to be associated with autoinmune systemic diseases. Recently, a small number of cases of a disorder distinct from Sweet syndrome or bullous lupus erythematosus (LE) have been described as specifically related to systemic LE under diverse terms, including nonbullous neutrophilic dermatosis, nonbullous neutrophilic LE, and Sweet-like neutrophilic dermatosis. We describe 7 patients that developed urticarial lesions in the context of a known or concurrently diagnosed autoimmune connective tissue disease. Of a total of 7 patients, 6 were afflicted by systemic LE and 1 by rheumatoid arthritis and secondary Sjögren syndrome. Histological findings in all patients included an interstitial and perivascular neutrophilic infiltrate with leukocytoclasia, vacuolar alteration along the dermal-edidermal junction, and no vasculitis. Most patients had active systemic disease at the time of the cutaneous eruption. Skin lesions resolved rapidly after the administration of immunomodulating agents. In conclusion, we provide additional evidence of the existence of a recently defined nonbullous neutrophilic dermatosis in the context of autoimmune connective tissue diseases and propose the term autoimmunity-related neutrophilic dermatosis as an appropriate designation. Furthermore, we believe that this entity should prompt physicians to screen the presence of an active systemic disorder in afflicted patients.

  16. Marine and other aquatic dermatoses

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    Jandhyala Sridhar

    2017-01-01

    Full Text Available Occupational and recreational aquatic activity predisposes our population to a wide variety of dermatoses. Sunburn, urticaria, jellyfish stings, and contact dermatitis to rubber equipment are common allergies that are encountered in the aquatic environment. Among the infections, tinea versicolor, intertrigo, and verruca vulgaris are widespread. Swimmer's itch may occur due to skin penetration by schistosome cercariae, while free-floating nematocysts of marine coelenterates may precipitate seabather's eruption. “Suit squeeze” due to cutaneous barotrauma and lymphoedematous peau d'orange due to decompression are rare, described entities. This review serves as a ready reckoner for Indian dermatologists and medical practitioners to identify and manage these conditions.

  17. Seasonal variation in pediatric dermatoses

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    Banerjee Sabyasachi

    2010-01-01

    Full Text Available Introduction: The under-five population is a unique and vulnerable component of our society that always demands special attention. Aims: Our present work aimed to study the seasonal variation, age-wise variation and distribution of lesions of common dermatoses of this age group. Materials and Methods: We clinically studied all fresh cases attending the skin OPD of our hospital for one month each from summer, rainy season and winter. Total number of patients was 879. Results: The top six skin diseases in our study were impetigo, miliaria, scabies, furunculosis, seborrheic dermatitis and papular urticaria. On statistical analysis, scabies and seborrheic dermatitis were more prevalent during winter while impetigo, furunculosis and miliaria were more during summer and rainy season. Papular urticaria was more frequent in the rainy season. Seborrheic dermatitis predominantly affected the infants while impetigo, furunculosis, miliaria and popular urticaria were commoner in older age groups. Conclusion: Distribution of lesions of common dermatoses will help diagnose difficult cases and extensive evaluation of the body parts which, by virtue of being commonly affected, are must-examine sites in under-five children.

  18. The autoimmune blistering diseases in Australia: status and services.

    Science.gov (United States)

    Daniel, Benjamin S; Dermawan, Andrew; Murrell, Dédée F

    2011-10-01

    The exact epidemiology of autoimmune bullous disorders in Australia is unknown. This article describes the establishment of a registry and support group to serve patients with these disorders. The registry aids research in Australia by providing epidemiologic data and maintaining an up-to-date database with patient contact details. The support group caters to the needs of patients and/or caregivers. Teledermatology, diagnosis, and management of autoimmune bullous disease are briefly reviewed.

  19. Dermatoses of the hand-an observation

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    Kumar Pramod

    1999-01-01

    Full Text Available After going through 1000 case files of patients registered in the skin department of K.M.C. Hospital, it was found that 166 (16.6% of them had dermatoses restricted to their hands. Based on the clinical findings and clinical diagnosis recorded, the various dermatoses were tabulated and evaluated. Ninety-two males and 74 females had dermatoses of hands. Warts and eczemas formed the major chunk of cases. Females out numbered males among patients with warts and eczemas.

  20. "Half-half" blisters in bullous pemphigoid successfully treated with adjuvant high-dose intravenous immunoglobulin.

    Science.gov (United States)

    Pacheco, David; Lopes, Leonor; Soares-Almeida, Luis; Marques, Manuel Sacramento; Filipe, Paulo

    2012-09-01

    Bullous pemphigoid is a rare, autoimmune blistering disease. Its clinical presentation is tense blisters that may arise on normal-appearing or erythematous skin. Bullous pemphigoid refractory to systemic corticosteroids in combination with immunosuppressants such as azathioprine and mycophenolate mofetil may benefit from adjuvant high-dose intravenous immunoglobulin (IVIg). We describe a particular case with an unusual clinical presentation unresponsive to systemic corticosteroids plus azathioprine, in which the addition of high-dose IVIg was successful. The combined therapy of systemic corticosteroids and azathioprine plus high-dose IVIg can be an option in refractory cases due to its efficiency and tolerability.

  1. Dermatoses Específicas da Gravidez

    OpenAIRE

    2013-01-01

    During pregnancy immunological, metabolic, hormonal and vascular changes occur, and can cause specific skin diseases. The specific dermatoses of pregnancy have undergone numerous changes in nomenclature and classification, partly due to advances in the knowledge of the pathogenesis of these skin diseases. Currently the following diseases are considered specific dermatoses of pregnancy: pemphigoid gestations, polymorphic eruption of pregnancy, intrahepatic cholestasis of pregnancy and atopic e...

  2. Association of bullous pemphigoid with malignancy: A myth or reality?

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    Joycelin Fernandes

    2014-01-01

    Full Text Available Bullous pemphigoid (BP is an autoimmune sub-epidermal blistering disorder of the skin. The association of BP with internal malignancy has always been a matter of debate with no consensus reached despite many published case reports and clinical trials. However, we report a hitherto unreported association of BP with squamous cell carcinoma of the tongue, wherein the patient had a recalcitrant downhill course despite adequate treatment measures with control of skin lesions being achieved only following excision of the tumor, and relapse coinciding with detection of metastasis. Hence, given the clinical behavior, it is reasonable to speculate that the association of malignancy was more than co-incidental.

  3. [Linear IgA bullous dermatosis of children].

    Science.gov (United States)

    Pierchalla, A; Bruch-Gerharz, D; Homey, B; Reifenberger, J

    2011-04-01

    Linear IgA bullous dermatosis is an acquired autoimmune subepidermal blistering disease, characterized by linear IgA deposits at the basement membrane zone. Described in both children and adults, it occurs as tense pruritic vesicles and bullae in a "cluster of jewels" configuration with central crusting on an inflammatory elevated base. It is typically located on the face, anogenital region and trunk. Whilst the adult manifestations can be chronic, in children a spontaneous remission has often been reported. Our patient showed a spontaneous remission after 8 weeks of symptomatic topic treatment with methylprednisolone and oral cetirizine dihydrochloride.

  4. A study of pattern and profile of non infectious dermatoses in paediatric age group <12 years.

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    Dr. Jeta Buch

    2017-06-01

    Full Text Available The aim of the study is to assess the incidence of non infectious dermatoses in children under 12 years of age; the incidence and prevalence of various physiological, genetic, papulosquamous, nutritional, autoimmune and drug induced disorders; the systemic association in various dermatoses and early identification of genetic disorder which will help in estimating the genetic risk and planning of future pregnancies. Present clinical study comprises of 1000 children less than 12 years of age in the department of Dermatology, V.S General Hospital, Ahmedabad. The study was conducted from Oct 2012 to Oct 2014. The time of occurrence, extent of involvement and the anatomical location was recorded. Relevant obstetrical history, history of any illness during pregnancy, history of drug ingestion, sibling history and parental consanguinity was noted and various skin lesions recorded. Observations were tabulated and statistically analysed. Follow up was done every 15 days. The dermatoses were classified as papulosquamous (61.0%, nevi (4.8%, genetic (4.2%, hair (9.6%, autoimmune (5.8%, drug reactions (3.1%, nutritional (2.8%, physiological (11.4%, and others (2.5%.The commonest skin manifestations observed were papulosquamous, genetic and autoimmune disorders. Better parent awareness, proper hygiene, adequate nutrition and early identification of the condition help in preventing many of these disorders.

  5. Bullous pemphigoid in a leg affected with hemiparesia: a possible relation of neurological diseases with bullous pemphigoid?

    Science.gov (United States)

    Foureur, N; Descamps, V; Lebrun-Vignes, B; Picard-Dahan, C; Grossin, M; Belaich, S; Crickx, B

    2001-01-01

    We report a typical case of bullous pemphigoid (BP) associated with a neurological disorder and study a possible link between neurological disorders and BP. An 84-year-old hemiplegic woman presented with unilateral BP on the hemiparetic side. BP was confirmed by histological and immunofluorescence data. The medical records of the previous 46 consecutive patients with BP were retrospectively analyzed (average age: 79; median age: 85). Thirty of the 46 patients with BP had neurological disorders. These disorders included dementia, epilepsy, multiple sclerosis, cerebral stroke, Parkinson's disease, gonadotropic adenoma, trembling, dyskinesia, lumbar spinal stenosis. In a control group of the 46 consecutive oldest patients (older than 71; average age: 82,5; median age: 80) with another skin disease referred during the previous two-year-period to our one-day-unit only, 13 patients had a neurological disorder. This study demonstrates that there is a high prevalence of neurological disorders in patients with BP (p = 0.0004). A prospective case control study with neurological examination and psychometrical evaluation is warranted to confirm these data. We speculate that neuroautoimmunity associated with the aging process or neurological disorders may be involved in pemphigoid development via an autoimmune response against dystonin which shares homology with bullous pemphigoid antigen 1. Bullous pemphigoid could be considered to be a marker of neurological disorder.

  6. [Linear IgA bullous dermatosis: the importance of a correct differential diagnosis].

    Science.gov (United States)

    Colombo, M; Volpini, S; Orini, S; Fazi, M C; Vettore, E; Tedoldi, S; Cappellaro, E

    2008-06-01

    Linear IgA bullous dermatosis is an acquired subepidermal blistering disease which belongs to bullous autoimmune diseases, along with dermatitis herpetiformis and bullous pemphigoid. Inflammatory blisters are the main clinical characteristics and the areas of common involvement are: perioral region, abdomen, perineum, buttocks and the interior side of thighs. Essential for the diagnosis is to find by direct immunofluorescence the presence of a linear band of IgA antibodies at the level of the basement membrane. We present the case of a 5 year-old Moroccan girl which arrived at our First Aid Department for bullous dermatitis, localized mainly on the abdomen, legs and thighs. During a short stay in Morocco, a month before, the little girl was stung by an insect and developed bullous dermatitis by a residual lesion. The child was in a good state of health but blood exams showed an increase of total IgE antibodies. The girl was admitted and during her hospitalisation we made a skin biopsy which led to a diagnosis of linear IgA dermatosis. She began a steroid therapy and there was a progressive regression of the lesions. At present, she does not take medicines anymore, she feels well and is submitted to ambulatory medical follow-up.

  7. Bullous pemphigoid: associations and management guidelines: facts and controversies.

    Science.gov (United States)

    Ruocco, Eleonora; Wolf, Ronni; Caccavale, Stefano; Brancaccio, Gabriella; Ruocco, Vincenzo; Lo Schiavo, Ada

    2013-01-01

    Bullous pemphigoid (BP) is an organ-specific human autoimmune disease typical of the elderly, which may be associated with many different disorders (immune, neurologic, psychiatric, hematologic, pulmonary, and cardiovascular), as well as with different types of tumors. The crucial question is whether these associations are casual or causal. In particular, the relationship between BP and malignancy is still a matter of debate. Although this association often has been emphasized, it is not clear whether the coexistent malignancy and BP are pathogenically connected or if their association is merely linked to aging. In our survey, the BP/neoplasm association ratios have not increased with age. Instead, significantly higher ratios have been found in younger patients; therefore, a meticulous investigation for a malignant neoplasm should always be pursued in young or middle-aged patients with BP, in particular regarding the severe forms of the disorder or those refractory to conventional treatment. The management of BP strictly depends on the multifactorial pathogenesis of this disorder. In fact, inflammation in BP seems to be more crucial for blister formation than in other autoimmune bullous disorders, such as pemphigus. Even if first-line treatments are typically immunosuppressants, newer therapeutic agents targeting specific pathogenic steps that are linked with inflammation have been proposed. Treatments selectively suppressing autoantibody formation, inflammation cascade, or both are available. Grouping therapies according to their mechanism of action may be useful to test new drug associations or to evaluate efficacy and tolerability of drugs with the same target.

  8. Patophysiological mechanism of pigmented purpuric dermatoses

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    Vujanac Andreja

    2013-12-01

    Full Text Available Aim: Patophysiological modeling of pigmented purpuric dermatoses based on venous hypertension. Capillaritis are considered to be patophysiological equivalent, or etiological basis of pigmented purpuric dermatoses. The exact mechanism has not been established and suggests the following: increased venous pressure, odontogenic processes, hypersensitivity to carbamezepine, meprobamate, furosemide, vitamin B1, contact dermatitis (khaki-colour dermatitis, capillary fragility and perforating vein incompetence. In this paper we presented a mechanism based on increased venous pressure. Methods: Graphic presentation of Crank, Krogh and Bessel equation. Results: In Schamberg's disease relative and absolute hemoglobin concentrations are changed much more slowly than in Majocchi disease. Curves based on Bessel function provides better explanation for hemoglobin changes according to clinical presentation. Conclusion: This review study could be starting point for further investigation of pigmented purpuric dermatoses.

  9. A study on dermatoses of pregnancy

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    Neerja Puri

    2013-01-01

    Full Text Available Certain dermatoses are specifically seen in pregnancy or postpartum period. It is therefore important for the clinicians to recognize and treat these cutaneous disorders to minimize maternal and fetal morbidity. The commonest pregnancy related dermatoses was polymorphic eruption of pregnancy seen in 22% patients, prurigo of pregnancy was seen in 7% patients, pemphigoid gestationis was seen in 3% patients, pruritic folliculitis of pregnancy was seen in 2% patients and intrahepatic cholestasis was seen in 1% of patients. It was seen that the skin disorders were commonest in the third trimester (60%, followed by 31% patients in second trimester and 9% patients in first trimester.

  10. Prevalence of various dermatoses in school children

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    Rao Gatha

    1999-01-01

    Full Text Available In a school survey of 1161 children from 2 different schools studying in 1st to 5th standard between the age of 6 and 12 years, 890 (76.65% students were found to have disorders of skin and its appendage. Most of the dermatoses were asymptomatic and were diagnosed in the routine examination. Nevoid conditions were seen in 255 (21.96% students. Communicable dermatoses were noted in 221 (19% students and nutritional deficiencies were seen in 78 (6.71% students. Rest of the students had miscellaneous condition. Health education and good personal hygiene will definitely help to improve the health status of the school children.

  11. Molecular biological aspects of acquired bullous diseases

    DEFF Research Database (Denmark)

    Dabelsteen, Erik

    1998-01-01

    of the bullous lesions. In this article, updated topics of the immune-mediated bullous lesions which involve oral mucosa and skin are reviewed. Pemphigus antigens, which are desmosomal-associated proteins and belong to the cadherin superfamily of cell adhesion proteins, have been isolated, and their genes have...

  12. Dermatoses neutrofílicas: parte II Neutrophilic dermatoses: part II

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    Fernanda Razera

    2011-04-01

    Full Text Available Neste artigo são abordadas as dermatoses neutrofílicas, complementando o artigo anterior (parte I. São apresentadas e comentadas as seguintes dermatoses: pustulose subcórnea de Sneddon-Wilkinson, dermatite crural pustulosa e atrófica, pustulose exantemática generalizada aguda, acroder matite contínua de Hallopeau, pustulose palmoplantar, acropustulose infantil, bacteride pustular de Andrews e foliculite pustulosa eosinofílica. Uma breve revisão das dermatoses neutrofílicas em pacientes pediátricos também é realizada.This article addresses neutrophilic dermatoses, thus complementing the previous article (part I. The following dermatoses are introduced and discussed: subcorneal pustular dermatosis (Sneddon-Wilkinson disease, dermatitis cruris pustulosa et atrophicans, acute generalized exanthematous pustulosis, continuous Hallopeau acrodermatitis, palmoplantar pustulosis, infantile acropustulosis, Andrews' pustular bacteride and eosinophilic pustular folliculitis. A brief review of neutrophilic dermatoses in pediatric patients is also conducted.

  13. Linear IgA bullous dermatosis induced by interferon-alpha 2a.

    Science.gov (United States)

    Kocyigit, P; Akay, B N; Karaosmanoglu, N

    2009-07-01

    Linear Ig A bullous dermatosis (LABD) is an acquired autoimmune subepidermal blistering disorder with linear deposits of IgA along the basement membrane zone. Its cause is unclear, although it appears to have an immune-mediated basis. Idiopathic, systemic disorder-related, and rarely drug-induced forms of LABD have been described. We describe a case of LABD associated with interferon-alpha 2A used for the treatment of Kaposi's sarcoma.

  14. Drug-induced linear IgA bullous dermatosis simulating toxic epidermal necrolysis.

    Science.gov (United States)

    Nasr, Joanna; Ammoury, Alfred; Chouairy, Camil; Mégarbané, Halal; El Habr, Constantin

    2014-01-01

    Linear IgA bullous dermatosis (LAD) is an autoimmune subepidermal blistering disorder. LAD may be either idiopathic or drug related; the most common drug being vancomycin. The clinical presentations of both idiopathic and drug-related LAD are variable and may mimic other blistering disorders. We report a case of a 76-year-old man known to have a renal cell carcinoma who presented a vancomycin-induced LAD that clinically mimicked toxic epidermal necrolysis (TEN).

  15. Linear IGA bullous dermatosis in Tunisian children : 31 cases

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    Kharfi Monia

    2011-01-01

    Full Text Available Background: Linear IgA bullous dermatosis (LAD of children is relatively frequent in Africa. Aim: We undertook this study to evaluate the frequency of this disease among autoimmune bullous diseases (AIBDs in Tunisian children. Materials and Methods: We present a 32-year retrospective study (January 1976 to December 2007. Children with chronic acquired bullous diseases seen at the Charles Nicolle Hospital of Tunis and for who direct immunofluorescence (DIF of the perilesional skin demonstrated linear IgA immunoglobulin deposits were included in the study population. Results: Thirty-one children with LAD were selected representing 65.9% of all AIBDs of children selected in the same period, with a mean age of 5.5 years and a sex ratio (M/F of 2.4. Most of the children had generalized eruption (28/31, more profuse on the face, pelvic region, buttocks and limbs. Mucosal lesions happened in only four children (12.9%. The mean duration of the disease was 14 months. DIF demonstrated linear IgA deposits along the dermal-epidermal junction in all patients. IgG, IgM, and complement were also seen (20/31. Indirect immunofluorescence was negative in 67% of cases. Eight patients responded to dapsone; however, prednisone had to be added in seven children to control the disease and erythromycin in four others. A long-term remission period was achieved in 76.1% of patients. Conclusion: This study confirms that LAD is the most common AIBD in children in Tunisia which frequently occurs in preschool-aged males. Independently of the used drug, a long-term remission is frequently observed.

  16. Bullous leukemia cutis mimicking facial cellulitis*

    Science.gov (United States)

    Caldato, Luciana de Sales; Britto, Juliana de Sousa; Niero-Melo, Ligia; Miot, Hélio Amante

    2016-01-01

    Bullous leukemia cutis is an uncommon clinical manifestation of cutaneous infiltration by leukemic cells, from B-cell chronic lymphocytic leukemia. We present the case of a 67-year-old, female, chronic lymphocytic leukemia patient. She was taking chlorambucil and developed facial edema with erythema and warmth, misjudged as facial cellulitis. Two days later, she developed bullous lesions in the arms, legs, neck and face. The histopathology of facial and bullous lesions confirmed leukemia cutis. All lesions disappeared following the administration of rituximab combined with cycles of fludarabine and cyclophosphamide. Although soft tissue infections are common complications in patients undergoing chemotherapy, leukemia cutis can also resemble cellulitis. PMID:27192532

  17. [Linear IgA bullous dermatosis].

    Science.gov (United States)

    Lorette, Gérard; Georgesco, Gabriella

    2010-10-01

    The linear IgA bullous dermatosis can have various aspects involving erythema and bullous lesions. It is a rare disease. Two peaks of frequency are noticed in children before puberty and in adults around 60 years of age. The histological and immunological characterisation is infraepidermal bullous lesions and linear deposits of IgA along the dermoepidermal basement membrane. There are some targets antigens. There is often a medical condition that seems to trigger. The link with drugs in particular with vancomycin was established. The mainstay of treatment is dapsone generally associated with steroids.

  18. Impaired quality of life in patients with systemic sclerosis compared to the general population and chronic dermatoses

    OpenAIRE

    Bretterklieber, Agnes; Painsi, Clemens; Avian, Alexander; Wutte, Nora; Aberer, Elisabeth

    2014-01-01

    Background Systemic sclerosis (SSc) is a rare and potentially life threatening autoimmune disorder. The burden of disease compared to other dermatoses is unknown. The purpose of this study was to assess both the quality of life in patients with SSc and the variables that are associated with poor quality of life. Forty-one patients with systemic sclerosis (29 limited, 2 diffuse, 10 undifferentiated forms) were assessed with respect to their health status and compared to published data for the ...

  19. Bullous Systemic Lupus Erythematosus and Lupus Nephritis in a Young Girl

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    Tooba Momen

    2016-11-01

    Full Text Available Bullous systemic lupus erythematosus (BSLE is an autoimmune blistering disease occurring in patients with systemic lupus erythematosus (SLE. It is a rare disease, especially in children. A 14-year-old girl initially presented with fatigue, generalized vesiculobullous skin lesions, and ulcers over the hard palate and oral mucosa. Clinical investigations revealed hematuria and proteinuria, a high erythrocyte sedimentation rate and titer of antinuclear antibody, and anti-double-stranded DNA. Skin biopsy findings were suggestive of BSLE. A renal biopsy confirmed the features of class V lupus nephritis. Based on the clinical features and investigations, a diagnosis of BSLE with nephritis was made. She received methylprednisolone pulse therapy and hydroxychloroquine; however, it did not alleviate the vesiculobullous eruption, so treatment with dapsone started and resulted in the dramatic disappearance of the lesions. Interruption of dapsone due to hemolysis did not aggravate the bullous disease. During follow-up, she had multiple flare-ups of disease and nephritis without rebound of bullous lesions. BSLE is a rare presentation of SLE in children. Differentiating it from other skin bullous diseases and SLE with blister is important for the correct management. The unusual presentation of this disease may delay the diagnosis and therefore requires a high index of clinical suspicion.

  20. Evidence-based treatments for pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid: A systematic review

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    Sanjay Singh

    2011-01-01

    Full Text Available Background: Pemphigus, bullous pemphigoid, and epidermolysis bullosa acquisita are autoimmune diseases of skin associated with considerable morbidity and sometimes mortality. There is no cure for these diseases. Aims: To summarize evidence-based treatments for these diseases by performing a systematic review. Methods: The research protocol included the following steps: identification of databases to be searched, defining search strategy, searching the databases for references, first-stage screening of the abstracts, second-stage screening of full texts of articles identified after the first-stage screening, data extraction from the identified articles after second-stage screening, quality appraisal of the studies using the Delphi list, and summarizing the findings. Results: No randomized controlled trials of interventions in pemphigus vegetans, pemphigus erythematosus, and epidermolysis bullosa acquisita could be found. After the second-stage screening, 12 randomized controlled trials were analyzed, which included patients with pemphigus vulgaris or pemphigus vulgaris and pemphigus foliaceus, and 7 which included patients with bullous pemphigoid. Conclusions: Number of high-quality randomized controlled trials conducted on pemphigus and bullous pemphigoid is small. Oral corticosteroid along with a steroid-sparing agent appears to be the most effective treatment for pemphigus. Azathioprine may be most effective as a steroid-sparing agent. Topical corticosteroid therapy (as studied is effective for bullous pemphigoid and appears to be superior to oral corticosteroid for extensive disease. Some suggestions about future research are made.

  1. A Screening Study on Dermatoses in Pregnancy

    Science.gov (United States)

    Tharini, GK

    2017-01-01

    Introduction Pregnancy produces many cutaneous changes, some of which are specifically related to pregnancy (dermatoses of pregnancy), some are modifiable by pregnancy and others that are common are named physiologic. These physiologic skin changes, usually do not impair the health of the mother or the fetus but some of them can be cosmetically significant and of importance to the dermatologist. Aim The present study was undertaken to find out the prevalence of the physiological and pathological skin changes in pregnancy, and to correlate the prevalence of the major cutaneous changes and diseases in relation to different trimesters of pregnancy and with gravidity. Materials and Methods A cross-sectional study was conducted during the period of August 2008 to August 2010. Ethical clearance was sought from Institutional Ethical Committee. Five hundred pregnant women were randomly selected, irrespective of the duration of pregnancy and gravidity. Detailed history and complete dermatological examination was done. Results were tabulated and analysed. Statistical analysis was done by Fisher’s exact test and Chi square test. Results Physiological skin changes were seen in 94.8% of cases, with pigmentary changes being more common (90.8%). Specific dermatoses of pregnancy were observed in 14% of cases with pruritus gravidarum being the most common (10.4%). Prevalence of infection was found to 30.8% with fungal infection being the most common (23.8%). Exacerbations of systemic lupus erythematosus and neurofibromatosis was observed. Pigmentary changes, striae gravidarum and specific dermatoses of pregnancy were observed in statistically significant proportion in primigravidas and during third trimester. Conclusion This study emphasizes that the prevalence of physiological skin changes (94.8%) was much higher than specific dermatoses (14%), stressing the fact that in most instances, the skin problems during pregnancy needs only reassurance. But meticulous observation and

  2. AUTOIMMUNE EPIDERMAL BLISTERING DISEASES

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    Ana Maria Abreu Velez

    2013-11-01

    Full Text Available Autoimmune bullous skin diseases (ABDs are uncommon, potentially fatal diseases of skin and mucous membranes which are associated with deposits of autoantibodies and complement against distinct molecules of the epidermis and dermal/epidermal basement membrane zone (BMZ. These autoantibodies lead to a loss in skin molecular integrity, which manifests clinically as formation of blisters or erosions. In pemphigus vulgaris, loss of adhesion occurs within the epidermis. The pioneering work of Ernst H. Beutner, Ph.D. and Robert E. Jordon, M.D. confirmed the autoimmune nature of these diseases. Walter F. Lever, M.D. contributed significantly to our understanding of the histopathologic features of these diseases. Walter Lever, M.D. and Ken Hashimoto, M.D. contributed electron microscopic studies of these diseases, especially in pemphigus vulgaris and bullous pemphigoid. In bullous pemphigoid (BP, linear IgA bullous dermatosis, epidermolysis bullosa acquisita (EBA and dermatitis herpetiformis (DH, loss of adhesion takes place within or underneath the BMZ. Classic EBA demonstrates extensive skin fragility; DH is commonly associated with gluten-sensitive enteropathy, and manifests clinically with pruritic papulovesicles on the extensor surfaces of the extremities and the lumbosacral area. The clinical spectrum of bullous pemphigoid includes tense blisters, urticarial plaques, and prurigo-like eczematous lesions. Pemphigoid gestationis mostly occurs during the last trimester of pregnancy, and mucous membrane pemphigoid primarily involves the oral mucosa and conjunctivae and leads to scarring. Linear IgA bullous dermatosis manifests with tense blisters in a „cluster of jewels”-like pattern in childhood (chronic bullous disease of childhood and is more clinically heterogeneous in adulthood. Many of the autoantigens in these disorders are known and have been well characterized. ABDs may be influenced by both genetic and exogenous factors. The diagnoses of

  3. Study of dermatoses in kidney transplant patients Estudo das dermatoses em pacientes transplantados renais

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    Alexandre Moretti de Lima

    2013-06-01

    Full Text Available BACKGROUND: The increasing in the number of kidney transplant recipients has favored, more frequently than before, the emergence of dermatoses and warranted their study through subsequent publications. OBJECTIVES: to evaluate the frequency of dermatoses in kidney transplant recipients. METHODS: kidney transplant recipients with suspected dermatoses between March 1st 2009 and June 30th 2010. RESULTS: 53 patients (28 males and 25 females, aged between 22 and 69 (mean age = 45 years were evaluated. Most of them came from the cities of Ceilândia, Samambaia and São Sebastião/DF, and had already been transplanted for 5 to 10 years before (37.7%; 62.3% were recipients of living donors and 83% were prednisone-treated. The most prevalent dermatoses were of fungal (45.3% and viral (39.6% etiologies. Among the non-melanoma malignant neoplasms, the basal cell carcinoma prevailed (six cases, in spite of the low incidence. Concerning fungal dermatoses, 12 cases of onychomycosis, five of pityriasis versicolor and four of pityrosporum folliculitis were reported. For diagnosis, in most cases (64.2%, laboratory examinations (mycological and histopathological were performed. CONCLUSION: cutaneous manifestations in kidney transplant recipients are generally secondary to immunosuppression. The infectious dermatoses, especially those of fungal origin, are frequently found in kidney transplant recipients and their occurrence increases progressively according to the time elapsed from the transplantation, which makes follow-up important. FUNDAMENTOS: o crescente aumento do número dos transplantados renais tem favorecido o aparecimento mais frequente das dermatoses e permitido o estudo em sucessivos trabalhos. OBJETIVOS: avaliar a frequência das dermatoses em pacientes transplantados renais. MÉTODOS: captação de pacientes transplantados renais durante o período de 1° de março de 2009 a 30 de junho de 2010 com suspeita de dermatoses. RESULTADOS : foram

  4. Eosinophils in Autoimmune Diseases

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    Daniela Čiháková

    2017-04-01

    Full Text Available Eosinophils are multifunctional granulocytes that contribute to initiation and modulation of inflammation. Their role in asthma and parasitic infections has long been recognized. Growing evidence now reveals a role for eosinophils in autoimmune diseases. In this review, we summarize the function of eosinophils in inflammatory bowel diseases, neuromyelitis optica, bullous pemphigoid, autoimmune myocarditis, primary biliary cirrhosis, eosinophilic granulomatosis with polyangiitis, and other autoimmune diseases. Clinical studies, eosinophil-targeted therapies, and experimental models have contributed to our understanding of the regulation and function of eosinophils in these diseases. By examining the role of eosinophils in autoimmune diseases of different organs, we can identify common pathogenic mechanisms. These include degranulation of cytotoxic granule proteins, induction of antibody-dependent cell-mediated cytotoxicity, release of proteases degrading extracellular matrix, immune modulation through cytokines, antigen presentation, and prothrombotic functions. The association of eosinophilic diseases with autoimmune diseases is also examined, showing a possible increase in autoimmune diseases in patients with eosinophilic esophagitis, hypereosinophilic syndrome, and non-allergic asthma. Finally, we summarize key future research needs.

  5. Linear IgA Bullous Dermatosis: A Rare Clinicopathologic Entity with an Unusual Presentation.

    Science.gov (United States)

    Chaudhari, Soham; Mobini, Narciss

    2015-10-01

    Linear immunoglobulin A bullous dermatosis is a rare autoimmune mucocutaneous disorder caused by immunoglobulin A autoantibodies produced against several different antigens in the basement membrane zone. Clinically, it is characterized by tense vesicles or bullae, which on histopathological exam demonstrate subepidermal blister with a predominantly neutrophilic infiltrate. A smooth, linear pattern of immunoglobulin A deposition in the basement membrane zone on direct immunofluorescence is considered the gold standard for establishing a diagnosis. Treatment consists of dapsone or sulfapyridine. The authors report a 60-year-old woman who presented with pruritic erythematous patches and plaques on her trunk, back, and legs without blisters, who was diagnosed with eczema for several months with no response to prior treatments. A biopsy was performed, which was consistent with linear immunoglobulin A bullous dermatosis and later confirmed by direct immunofluorescence studies. The authors present this case to increase awareness of this rare disease, which could manifest in a nonclassical, nonblistering fashion.

  6. Linear IgA bullous dermatosis: a retrospective study of 23 patients in Denmark.

    Science.gov (United States)

    Lings, Kristina; Bygum, Anette

    2015-04-01

    Linear IgA bullous dermatosis (LAD) is an autoimmune, chronic bullous disease affecting primarily young children and adults. Studies on LAD are relatively sparse and from Scandinavia we could only find a few case reports. Therefore we decided to conduct a retrospective investigation of patients seen at our department since 1972. A total of 23 patients were identified; 7 children (F:M ratio 0.75) and 16 adults (F:M ratio 0.78). Mean age at disease onset in the two age groups were 2.7 and 56.8 years. Estimated incidence rate in our region: 0.67 per million per year. The most commonly used treatment modalities were corticosteroids, dapsone and sulphapyridine.

  7. Dermatoses in cement workers in southern Taiwan.

    Science.gov (United States)

    Guo, Y L; Wang, B J; Yeh, K C; Wang, J C; Kao, H H; Wang, M T; Shih, H C; Chen, C J

    1999-01-01

    Construction workers are known to have occupational dermatoses. The prevalence of such dermatoses was unknown in Taiwanese construction workers. The objective of this study was to determine the work exposure, prevalence of skin manifestations, and sensitivity to common contact allergens in cement workers of southern Taiwan. A total of 1147 current regular cement workers were telephone-interviewed about skin problems during the past 12 months, work exposure, and personal protection. Among those interviewed, 166 were examined and patch tested with common contact allergens. A high % of cement workers reported skin problems in the past 12 months. More men (13.9%) reported skin problems possibly related to work than women (5.4%). Prevalence was associated with lower use of gloves, duration of work as cement worker, and more time in jobs involving direct manual handling of cement, especially tiling. A high % of dermatitis was noted in the 166 workers examined, which correlated with reported skin problems. On patch testing, construction workers had a high frequency of sensitivity to chromate. Sensitivity to chromate or cobalt was associated with reported skin problems, or dorsal hand dermatitis on examination. These workers' dermatitis was under-diagnosed and inadequately managed. It is concluded that cement workers in southern Taiwan had a high prevalence of skin problems related to cement use. Protective measures, work practice, and physician education should be improved to prevent or manage such problems.

  8. Development of bullous pemphigoid during treatment of psoriatic onycho-pachydermo periostitis with ustekinumab.

    Science.gov (United States)

    Nakayama, Chihiro; Fujita, Yasuyuki; Watanabe, Mika; Shimizu, Hiroshi

    2015-10-01

    Ustekinumab is a human monoclonal antibody that specifically binds to the p40 subunit of interleukin (IL)-12 and IL-23, inhibiting the activity of both cytokines, thereby blocking the T-helper (Th)1 and Th17 inflammatory pathways. While biologic agents have dramatically changed the strategies of psoriasis treatment, increasing cases of autoimmune diseases during the use of such agents have been reported. We experienced a case of bullous pemphigoid occurring during treatment of a rare variant of psoriatic arthritis, psoriatic onycho-pachydermo periostitis with ustekinumab. Only six cases of autoimmune blistering diseases during treatment with biologic agents have ever been reported including our case, and we herein review the published work of these cases. Dermatologists must be attentive to the possibility of autoimmune blistering diseases during ustekinumab treatment.

  9. Caspase-1-Independent IL-1 Release Mediates Blister Formation in Autoantibody-Induced Tissue Injury through Modulation of Endothelial Adhesion Molecules

    NARCIS (Netherlands)

    Sadeghi, Hengameh; Lockmann, Anike; Hund, Anna-Carina; Samavedam, Unni K. S. R. L.; Pipi, Elena; Vafia, Katerina; Hauenschild, Eva; Kalies, Kathrin; Pas, Hendrikus; Jonkman, Marcel F.; Iwata, Hiroaki; Recke, Andreas; Schon, Michael P.; Zillikens, Detlef; Schmidt, Enno; Ludwig, Ralf J.

    2015-01-01

    Although reports documented aberrant cytokine expression in autoimmune bullous dermatoses (AIBDs), cytokine-targeting therapies have not been established in these disorders. We showed previously that IL-6 treatment protected against tissue destruction in experimental epidermolysis bullosa acquisita

  10. Aberrant Expression and Secretion of Heat Shock Protein 90 in Patients with Bullous Pemphigoid

    Science.gov (United States)

    Tukaj, Stefan; Kleszczyński, Konrad; Vafia, Katerina; Groth, Stephanie; Meyersburg, Damian; Trzonkowski, Piotr; Ludwig, Ralf J.; Zillikens, Detlef; Schmidt, Enno; Fischer, Tobias W.; Kasperkiewicz, Michael

    2013-01-01

    The cell stress chaperone heat shock protein 90 (Hsp90) has been implicated in inflammatory responses and its inhibition has proven successful in different mouse models of autoimmune diseases, including epidermolysis bullosa acquisita. Here, we investigated expression levels and secretory responses of Hsp90 in patients with bullous pemphigoid (BP), the most common subepidermal autoimmune blistering skin disease. In comparison to healthy controls, the following observations were made: (i) Hsp90 was highly expressed in the skin of BP patients, whereas its serum levels were decreased and inversely associated with IgG autoantibody levels against the NC16A immunodominant region of the BP180 autoantigen, (ii) in contrast, neither aberrant levels of circulating Hsp90 nor any correlation of this protein with serum autoantibodies was found in a control cohort of autoimmune bullous disease patients with pemphigus vulgaris, (iii) Hsp90 was highly expressed in and restrictedly released from peripheral blood mononuclear cells of BP patients, and (iv) Hsp90 was potently induced in and restrictedly secreted from human keratinocyte (HaCaT) cells by BP serum and isolated anti-BP180 NC16A IgG autoantibodies, respectively. Our results reveal an upregulated Hsp90 expression at the site of inflammation and an autoantibody-mediated dysregulation of the intracellular and extracellular distribution of this chaperone in BP patients. These findings suggest that Hsp90 may play a pathophysiological role and represent a novel potential treatment target in BP. PMID:23936217

  11. Aberrant expression and secretion of heat shock protein 90 in patients with bullous pemphigoid.

    Directory of Open Access Journals (Sweden)

    Stefan Tukaj

    Full Text Available The cell stress chaperone heat shock protein 90 (Hsp90 has been implicated in inflammatory responses and its inhibition has proven successful in different mouse models of autoimmune diseases, including epidermolysis bullosa acquisita. Here, we investigated expression levels and secretory responses of Hsp90 in patients with bullous pemphigoid (BP, the most common subepidermal autoimmune blistering skin disease. In comparison to healthy controls, the following observations were made: (i Hsp90 was highly expressed in the skin of BP patients, whereas its serum levels were decreased and inversely associated with IgG autoantibody levels against the NC16A immunodominant region of the BP180 autoantigen, (ii in contrast, neither aberrant levels of circulating Hsp90 nor any correlation of this protein with serum autoantibodies was found in a control cohort of autoimmune bullous disease patients with pemphigus vulgaris, (iii Hsp90 was highly expressed in and restrictedly released from peripheral blood mononuclear cells of BP patients, and (iv Hsp90 was potently induced in and restrictedly secreted from human keratinocyte (HaCaT cells by BP serum and isolated anti-BP180 NC16A IgG autoantibodies, respectively. Our results reveal an upregulated Hsp90 expression at the site of inflammation and an autoantibody-mediated dysregulation of the intracellular and extracellular distribution of this chaperone in BP patients. These findings suggest that Hsp90 may play a pathophysiological role and represent a novel potential treatment target in BP.

  12. Advances in understanding and managing bullous pemphigoid [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Cathy Y. Zhao

    2015-11-01

    Full Text Available Bullous pemphigoid (BP is the commonest subtype of autoimmune blistering disease in most countries of the world. It occurs most frequently in elderly patients and is characterised clinically by large, tense blisters in the skin preceded by urticarial plaques and pruritus. Immunopathologically, it is characterised by autoantibodies directed against the 180 kD antigen (BP180 and the 230 kD antigen (BP230. New knowledge regarding BP is being continually uncovered. This article reviews the recent advances in BP, including newer diagnostic tests, standardised outcome measures and emerging therapeutic options, as well as the evidence supporting their use.

  13. Development of bullous pemphigoid during the haemodialysis of a young man: case report and literature survey.

    Science.gov (United States)

    Osipowicz, Katarzyna; Kalinska-Bienias, Agnieszka; Kowalewski, Cezary; Wozniak, Katarzyna

    2017-02-01

    Haemodialysis is the most frequent form of renal replacement therapy (RRT) in patients with end-stage renal disorder (ESRD). Patients with ESRD frequently develop skin problems, mainly xerosis, pruritus and hyperpigmentation, as well as bullous diseases, mainly porphyria or pseudoporphyria and, in some cases, bullous pemphigoid (BP). BP is the most common autoimmune sub-epidermal blistering disease, and it predominantly affects elderly people. Clinically, BP is characterised by generalised pruritic, bullous eruptions and urticaria-like lesions. Usually, BP is an idiopathic disorder; however, in some cases, underlying internal disorders are present, like diabetes or neurological disorders. Herein, we present a 33-year-old man with ESRD, maintained on haemodialysis, who developed BP. There are only six cases with BP provoked by the placement of a fistula for haemodialysis. BP in the current patient was confirmed by direct immunofluorescence (DIF) and indirect immunofluorescence using BIOCHIP. The patient responded promptly to tertracycline and 0·05% clobetasol propionate lesionally. However, the relationship between BP and the fistula for haemodialisys still remains unknown. It is highly likely that the skin injury associated with fistula placement was responsible for the alteration of the basement membrane zone (BMZ) and the stimulation of the immune system, leading to BP development. To explain the real role of fistula placement as a provocative factor in BP, other such cases are required for assessment.

  14. Occupational hand dermatoses in hospital cleaning personnel.

    Science.gov (United States)

    Singgih, S I; Lantinga, H; Nater, J P; Woest, T E; Kruyt-Gaspersz, J A

    1986-01-01

    Hospital cleaning personnel were examined for occupational dermatoses. 356 persons were included in the study. The age ranged from 20 to 63 years with a mean of 40.1 years. The period prevalence rate of moderate and severe eczema was 12% (10% in men and 19% in women). In 88%, the eczema was of a duration longer than 2 years. Positive patch tests were found in 10% of men and 53% of women with eczema. The main allergens were nickel, cobalt, chromate and rubber chemicals. Positive tests to cleaning agents were rare. One case of contact allergy to sodium dichloro-iso-cyanurate and one to lysol were diagnosed. Irritant factors played a major rôle in most cases (92%). Fungus infection as a cause or complication in hand eczema should not be left out of consideration: in 2 persons, a mycosis of hands and/or fingernails was diagnosed.

  15. Role of sebaceous glands in inflammatory dermatoses.

    Science.gov (United States)

    Shi, Vivian Y; Leo, Michael; Hassoun, Lauren; Chahal, Dev S; Maibach, Howard I; Sivamani, Raja K

    2015-11-01

    Skin is an important interface between the host and its environment. Inflammatory dermatoses often have disrupted skin barrier function, rendering patients more susceptible to allergenic triggers leading to an exaggerated immune response. The skin surface lipid film, an important component of the skin barrier, comprises a mixture of keratinocyte and sebaceous gland-derived lipids. Recent evidence demonstrated that defective keratinocyte lipid synthesis predisposes for the development of atopic dermatitis. However, the important role of sebaceous gland-derived lipids in skin inflammatory diseases may be underrecognized. This overview focuses on the importance of the contribution of sebaceous glands to barrier function. Sebaceous gland alteration may play a role in the pathogenesis of common skin diseases including acne vulgaris, atopic dermatitis, psoriasis, rosacea, and seborrheic dermatitis.

  16. Tropical and exotic dermatoses and ulcers.

    Science.gov (United States)

    Rathnayake, Deepani; Sinclair, Rodney

    2014-09-01

    Tropical dermatoses and ulcers, although essentially unique to tropical and subtropical areas, are occasionally seen in Australian general practice on returning travellers and migrants from endemic countries. This article will discuss important causes of tropical and exotic ulcers occasionally seen in Australia. As tropical ulcers may mimic many other causes of skin ulceration and nodules, a history of recent travel should arouse clinical suspicion. The time frame since exposure to the causative organism is an important feature in the diagnostic process. For example, pyodermas and cutaneous larva migrans present a few days after contact with the causative agents, whereas leishmaniasis, cutaneous tuberculosis, atypical mycobacterial diseases (swimming pool granulomas) and tropical mycosis take weeks to months to appear.

  17. Childhood-onset bullous systemic lupus erythematosus.

    Science.gov (United States)

    Lourenço, D M R; Gomes, R Cunha; Aikawa, N E; Campos, L M A; Romiti, R; Silva, C A

    2014-11-01

    Bullous systemic lupus erythematosus has rarely been described in pediatric lupus population and the real prevalence of childhood-onset bullous systemic lupus erythematosus has not been reported. From January 1983 to November 2013, 303 childhood-onset SLE (c-SLE) patients were followed at the Pediatric Rheumatology Unit of the Childreńs Institute of Hospital das Clínicas da Faculdade de Medicina Universidade da Universidade de São Paulo, three of them (1%) diagnosed as childhood-onset bullous systemic lupus erythematosus. All three cases presented tense vesiculobullous lesions unassociated with lupus erythematosus lesions, with the median duration of 60 days (30-60). All patients fulfilled bullous systemic lupus erythematosus criteria. Two had nephritis and serositis and presented specific autoantibodies. The histological pattern demonstrated subepidermal blisters with neutrophils-predominant infiltrates within the upper dermis. Direct immunofluorescence (DIF) showed deposits of IgG and complement along the epidermal basement membrane, in the presence or absence of IgA and/or IgM. A positive indirect immunofluorescence on salt-split skin demonstrating dermal binding was observed in two cases. All of them had moderate/severe disease activity at diagnosis with median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) of 18 (14-24). Two patients received dapsone and one with severe nephritis received immunosuppressive drugs. In conclusion, in the last 30 years the prevalence of bullous lupus in childhood-onset lupus population was low (1%) in our tertiary University Hospital. A diagnosis of SLE should always be considered in children with recurrent tense vesiculobullous lesions with or without systemic manifestations. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  18. Dermatoses em renais cronicos em terapia dialitica

    Directory of Open Access Journals (Sweden)

    Luis Alberto Batista Peres

    2014-03-01

    Full Text Available Objetivo: As desordens cutâneas e das mucosas são comuns em pacientes em hemodiálise a longo prazo. A diálise prolonga a expectativa de vida, dando tempo para a manifestação destas anormalidades. Os objetivos deste estudo foram avaliar a prevalência de problemas dermatológicos em pacientes com doença renal crônica (DRC em hemodiálise. Métodos: Cento e quarenta e cinco pacientes com doença renal crônica em hemodiálise foram estudados. Todos os pacientes foram completamente analisados para as alterações cutâneas, de cabelos, mucosas e unhas por um único examinador e foram coletados dados de exames laboratoriais. Os dados foram armazenados em um banco de dados do Microsolft Excel e analisados por estatística descritiva. As variáveis contínuas foram comparadas pelo teste t de Student e as variáveis categóricas utilizando o teste do qui-quadrado ou o teste Exato de Fischer, conforme adequado. Resultados: O estudo incluiu 145 pacientes, com idade média de 53,6 ± 14,7 anos, predominantemente do sexo masculino (64,1% e caucasianos (90,0%. O tempo médio de diálise foi de 43,3 ± 42,3 meses. As principais doenças subjacentes foram: hipertensão arterial em 33,8%, diabetes mellitus em 29,6% e glomerulonefrite crônica em 13,1%. As principais manifestações dermatológicas observadas foram: xerose em 109 (75,2%, equimose em 87 (60,0%, prurido em 78 (53,8% e lentigo em 33 (22,8% pacientes. Conclusão: O nosso estudo mostrou a presença de mais do que uma dermatose por paciente. As alterações cutâneas são frequentes em pacientes em diálise. Mais estudos são necessários para melhor caracterização e manejo destas dermatoses.

  19. Linear IgA Bullous Dermatosis Secondary to Infliximab Therapy in a Patient with Ulcerative Colitis.

    Science.gov (United States)

    Hoffmann, Jochen; Hadaschik, Eva; Enk, Alexander; Stremmel, Wolfgang; Gauss, Annika

    2015-01-01

    Linear IgA bullous disease (LABD) is a rare vesiculobullous autoimmune skin disorder whose etiology and pathogenesis are not completely understood. Its occurrence has been related to malignancies, inflammatory diseases and several drugs. This report describes a 49-year-old Caucasian male with a 14-year history of ulcerative colitis who received infliximab to treat the refractory course of his bowel disease. During induction therapy with infliximab, he developed LABD. Treatment with infliximab was discontinued, and the skin lesions were successfully treated with oral steroids and dapsone. Considering the close chronological relation between administration of the tumor necrosis factor-α inhibitor and onset of the skin disease, we hypothesize that this is the first reported case of infliximab-induced LABD. Similar to psoriasis, it may represent a 'paradoxical' autoimmune reaction triggered by anti-tumor necrosis factor-α therapy.

  20. Mediators of Mast Cells in Bullous Pemphigoid and Dermatitis Herpetiformis

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    Agnieszka Zebrowska

    2014-01-01

    Full Text Available Bullous pemphigoid (BP and dermatitis herpetiformis (DH are skin diseases associated with inflammation. However, few findings exist concerning the role of mast cells in autoimmune blistering disease. Skin biopsies were taken from 27 BP and 14 DH patients, as well as 20 healthy individuals. Immunohistochemistry was used to identify the localization and mast cell expression of TNFα and MMP9 in skin lesions and perilesional skin. The serum concentrations of TNFα, MMP9, chymase, tryptase, PAF, and IL-4 were measured by immunoassay. TNFα and MMP9 expression in the epidermis and in inflammatory influxed cells in the dermis was detected in skin biopsies from patients. Although these mediators were found to be expressed in the perilesional skin of all patients, the level was much lower than that in lesional skin. Increased serum PAF levels were observed in BP patients. Mast cells may play an essential role in activating inflammation, which ultimately contributes to the tissue damage observed in BP and DH. Our findings suggest that differences in the pattern of cytokine expression directly contribute to variations in cellular infiltration in DH and BP.

  1. Localized linear IgA/IgG bullous dermatosis.

    Science.gov (United States)

    Shimizu, Satoko; Natsuga, Ken; Shinkuma, Satoru; Yasui, Chikako; Tsuchiya, Kikuo; Shimizu, Hiroshi

    2010-11-01

    Linear IgA/IgG bullous dermatosis (LAGBD) is an auto-immune blistering disease characterized by the local accumulation of IgA- and IgG-class anti-basement membrane autoantibodies. It typically presents as a generalized pruritic vesiculobullous eruption. No cases of localized LAGBD have yet been reported. We report a case of a 78-year-old man with LAGBD localized to the perianal area. The patient complained of suffering from persistent ulcers around the anus for more than 3 years. Physical examination revealed several blisters and ulcers up to 2-cm in diameter around the anus. No lesions were found elsewhere on the body. Histological analysis of a skin biopsy revealed subepidermal blistering, while direct immunofluorescence showed the linear deposition of IgA and IgG antibodies at the dermoepidermal junction. Indirect immunofluorescence of normal human skin whose layers had been separated using 1M NaCl showed the binding of both IgA and IgG to the epidermal side. Immunoblotting demonstrated the presence of circulating IgA and IgG autoantibodies that bound to a 120-kDa protein. This is the first case of localized LAGBD whose skin lesions were restricted to the perianal region.

  2. Dermatoses due to Indian cultural practices.

    Science.gov (United States)

    Gupta, Divya; Thappa, Devinder Mohan

    2015-01-01

    A wide prevalence of socio-religious and cultural practices in the Asian subcontinent often leads to multitude of skin diseases which may be missed by the dermatologists because of a lack of awareness. 'Henna' use causes IgE-mediated hypersensitivity reactions and contact dermatitis. 'Kumkum' application can result in pigmented contact dermatitis and lichen planus pigmentosus. Sticker 'bindis' and 'alta' induce contact leukoderma. Irritant and allergic contact dermatitis occurs after playing with 'Holi' colors. Threading and drawstring dermatitis lead to koebnerization of pre-existing dermatoses, infections and even squamous cell carcinoma of skin. Mild irritant reactions and contact sensitization occur secondary to balm and hair oil use. 'Mudichood' represents the comedogenic effect of hair oils combined with occlusion and humidity. Aromatherapy oils can cause contact dermatitis and photosensitive reactions. Heavy metal and steroid toxicity along with severe cutaneous adverse effects like erythroderma can occur as a consequent to the use of alternative medicines. Squamous cell carcinoma due to chronic heat exposure from the heating device "kangri" is seen in Kashmiris. Prayer nodules in Muslims and traction alopecia in Sikhs illustrate how religious practices can negatively affect the skin. With increasing globalization and migration, the practice of indigenous customs and traditions is no longer limited to regional territories, making it imperative for the dermatologists to be acquainted with the cutaneous side effects they can cause.

  3. Dermatoses Due to Indian Cultural Practices

    Science.gov (United States)

    Gupta, Divya; Thappa, Devinder Mohan

    2015-01-01

    A wide prevalence of socio-religious and cultural practices in the Asian subcontinent often leads to multitude of skin diseases which may be missed by the dermatologists because of a lack of awareness. ‘Henna’ use causes IgE-mediated hypersensitivity reactions and contact dermatitis. ‘Kumkum’ application can result in pigmented contact dermatitis and lichen planus pigmentosus. Sticker ‘bindis’ and ‘alta’ induce contact leukoderma. Irritant and allergic contact dermatitis occurs after playing with ‘Holi’ colors. Threading and drawstring dermatitis lead to koebnerization of pre-existing dermatoses, infections and even squamous cell carcinoma of skin. Mild irritant reactions and contact sensitization occur secondary to balm and hair oil use. ‘Mudichood’ represents the comedogenic effect of hair oils combined with occlusion and humidity. Aromatherapy oils can cause contact dermatitis and photosensitive reactions. Heavy metal and steroid toxicity along with severe cutaneous adverse effects like erythroderma can occur as a consequent to the use of alternative medicines. Squamous cell carcinoma due to chronic heat exposure from the heating device “kangri” is seen in Kashmiris. Prayer nodules in Muslims and traction alopecia in Sikhs illustrate how religious practices can negatively affect the skin. With increasing globalization and migration, the practice of indigenous customs and traditions is no longer limited to regional territories, making it imperative for the dermatologists to be acquainted with the cutaneous side effects they can cause. PMID:25657390

  4. Dermatoses due to indian cultural practices

    Directory of Open Access Journals (Sweden)

    Divya Gupta

    2015-01-01

    Full Text Available A wide prevalence of socio-religious and cultural practices in the Asian subcontinent often leads to multitude of skin diseases which may be missed by the dermatologists because of a lack of awareness. ′Henna′ use causes IgE-mediated hypersensitivity reactions and contact dermatitis. ′Kumkum′ application can result in pigmented contact dermatitis and lichen planus pigmentosus. Sticker ′bindis′ and ′alta′ induce contact leukoderma. Irritant and allergic contact dermatitis occurs after playing with ′Holi′ colors. Threading and drawstring dermatitis lead to koebnerization of pre-existing dermatoses, infections and even squamous cell carcinoma of skin. Mild irritant reactions and contact sensitization occur secondary to balm and hair oil use. ′Mudichood′ represents the comedogenic effect of hair oils combined with occlusion and humidity. Aromatherapy oils can cause contact dermatitis and photosensitive reactions. Heavy metal and steroid toxicity along with severe cutaneous adverse effects like erythroderma can occur as a consequent to the use of alternative medicines. Squamous cell carcinoma due to chronic heat exposure from the heating device "kangri" is seen in Kashmiris. Prayer nodules in Muslims and traction alopecia in Sikhs illustrate how religious practices can negatively affect the skin. With increasing globalization and migration, the practice of indigenous customs and traditions is no longer limited to regional territories, making it imperative for the dermatologists to be acquainted with the cutaneous side effects they can cause.

  5. Prioritizing dermatoses: rationally selecting guideline topics.

    Science.gov (United States)

    Borgonjen, R J; van Everdingen, J J E; van de Kerkhof, P C M; Spuls, Ph I

    2015-08-01

    Clinical practice guideline (CPG) development starts with selecting appropriate topics, as resources to develop a guideline are limited. However, a standardized method for topic selection is commonly missing and the way different criteria are used to prioritize is not clear. To select and prioritize dermatological topics for CPG development and elucidate criteria dermatologists find important in selecting guideline topics. All 410 dermatologists in the Netherlands were asked to create a top 20 of dermatological topics for which a guideline would be desirable, regardless of existing guidelines. They also rated, on a 5-point Likert scale, 10 determinative criteria derived from a combined search in literature and across (inter)national guideline developers. Top 20 topics received scores ranging from 0.01 to 0.2 and combined scores yielded a total score. The 118 surveys (response 29%) identified 157 different topics. Melanoma, squamous cell carcinoma, basal cell carcinoma, psoriasis and atopic dermatitis are top priority guideline topics. Venous leg ulcer, vasculitis, varicose veins, urticaria, acne, Lyme borreliosis, cutaneous lupus erythematosus, pruritus, syphilis, lymphoedema, decubitus ulcer, hidradenitis suppurativa, androgenic alopecia and bullous pemphigoïd complete the top 20. A further 15 topics have overlapping confidence intervals. Mortality and healthcare costs are regarded as less important criteria in topic selection (P topics. Respondents mostly agree with (inter)national guideline programmes and literature concerning the criteria important to selecting guideline topics. © 2014 European Academy of Dermatology and Venereology.

  6. Bullous impetigo caused by Streptococcus salivarius: a case report.

    OpenAIRE

    Brook, I

    1980-01-01

    A 19-month-old child presented with bullous impetigo around the perineal region, penis, and left foot. Streptococcus salivarius was the only isolate recovered from the lesions. The child was treated with parenteral penicillin, debridement of the bulli, and local application of silver sulphadiazine cream. This case of bullous impetigo illustrates another aspect of the pathogenicity of Strep. salivarius.

  7. Bullous Systemic Lupus Erythematosus Associated with Esophagitis Dissecans Superficialis

    Science.gov (United States)

    Jaffe, Eric A.

    2015-01-01

    Bullous systemic lupus erythematosus is one of the rare autoantibody mediated skin manifestation of systemic lupus erythematosus (SLE) demonstrating subepidermal blistering with neutrophilic infiltrate histologically. We present a case of a 40-year-old Hispanic female who presented with a several months' history of multiple blistering pruritic skin lesions involving the face and trunk, a photosensitive rash over the face and neck, swelling of the right neck lymph node, and joint pain involving her elbows and wrist. Her malady was diagnosed as bullous systemic lupus erythematosus based on the immunological workup and biopsy of her skin lesions. The patient also complained of odynophagia and endoscopy revealed esophagitis dissecans superficialis which is a rare endoscopic finding characterized by sloughing of the esophageal mucosa. The bullous disorders typically associated with esophagitis dissecans superficialis are pemphigus and rarely bullous pemphigoid. However, this is the first reported case of bullous systemic lupus erythematosus associated with esophagitis dissecans superficialis. PMID:25821624

  8. A newborn with bullous pemphigoid associated with linear IgA bullous dermatosis.

    Science.gov (United States)

    Akin, M A; Gunes, T; Akýn, L; Ohyama, B; Kontas, O; Hashimoto, T

    2009-06-01

    A 16-day-old boy was admitted to our clinic with localized blisters on the neck, cheeks, earlobes, and oral cavity and with erythema on the toes, in addition to poor weight gain and respiratory distress. A physical examination revealed several erythematous plaques with tense bullae, multiple vesicles, and erosions on the left toes, neck, earlobes, and face as well as erosive lesions on the anterior part of the oral cavity, lips, and buccal mucosae. A bronchoscopic examination revealed bullous lesions in the upper respiratory tract and on the epiglottis. A skin biopsy suggested a diagnosis of bullous pemphigoid (BP). Because of the severe mucosal involvement, further investigations including various immunological techniques were performed. The case was diagnosed as BP associated with linear IgA bullous disease (LAD). Complete remission without any scarring was achieved after three weeks of oral methyl prednisolone treatment. A correct differential diagnosis of bullous diseases is important for determining the prognosis and expected response to treatment. To our knowledge, this is the first case of BP associated with LAD reported in literature.

  9. Clinicoepidemiological study of pigmented purpuric dermatoses

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    Lata Sharma

    2012-01-01

    Full Text Available Background: Pigmented purpuric dermatoses (PPD are a group of vascular disorders with varied manifestations which cause concern and are resistant to treatment. The literature is still lacking in clinicoepidemiological studies. Aim: To study the epidemiology, etiological, host and environmental factors, clinical manifestations, its variations, and the type prevalent in this part of the world. Materials and Methods: All cases of PPD were selected for the study from Skin and Venereal Disease, Out Patient Department between January 2008 and June 2009. Their history, examination, hematological investigations, and, in a few, histopathology findings were also recorded and data obtained were evaluated statistically. Results: There were 100 cases of PPD of total 55 323 patients (0.18%. There were 79 males and 21 females between 11 and 66 years. They were working as police men, security guards, barber, chemist, teachers, students, farmers, businessmen, and housewives. In a majority, there was a history of prolonged standing in day-to-day work. Purpuric, brownish pigmented, lichenoid or atrophic lesions were seen depending upon the type of PPD on lower parts of one or both lower limbs. Blood investigations were normal. Schamberg′s disease was seen in ninety five, Lichen aureus in three, lichenoid dermatosis and Majocchi′s disease in one case each. Discussion: Three clinical types of PPD were diagnosed which may represent different features of the same disease. Cell-mediated immunity, immune complexes, capillary fragility, gravitational forces, venous hypertension, focal infection, clothing, contact allergy to dyes, and drug intake have been incriminating factors in the past. Patient′s occupation and environmental factors may also be considered contributory in precipitating the disease. Conclusions: The study revealed the problem of PPD in this geographical area, its magnitude, clinical presentation, the type prevalent, and possible aggravating

  10. Clinicopathological evaluation of non-parasitic dermatoses in canines

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    M. J. Sindha

    2015-11-01

    Full Text Available Aim: The present study has been carried out to detect non-parasitic dermatoses in canines brought at the Nandini Veterinary Hospital, Surat. Materials and Methods: The current investigation was carried out on skin scrapping, skin biopsy specimens, blood, and serum samples of 210 freshly registered cases of dogs with dermatological afflictions. Dogs found healthy on clinical examination were used as control animals (n=15. The incidence of non-parasitic dermatoses has been recorded as per age, breed, and sex of dogs. For bacterial isolation, the pus/exudates samples were collected from 40 cases of pyoderma and streaked onto brain-heart infusion agar while 13 skin scrapping samples were inoculated on Sabouraud’s dextrose agar with chloramphenicol for isolation of fungi. The organisms were identified on the basis of gross and microscopic observation of cultural growth on media. The blood and sera samples were also collected to note alteration in hematology and biochemical parameters, respectively. Tissue samples from lesions were collected and subsequently preserved in 10% neutral buffered formalin for histopathology. Results: Out of 210 cases of dermatoses, 60 cases were of non-parasitic dermatoses, i.e., 28.57%. Of these, bacterial skin infections (pyoderma were found to be the predominant at 80.00%, followed by other non-parasitic dermatological disorders, i.e., 11.67% and fungal skin infection, i.e., 8.33%. The dogs belonging to age group 1-3 years showed greater susceptibility to non-parasitic dermatological conditions. Breed wise incidence of pyoderma was found more in the Pomeranian breed (20.83%, whereas fungal skin affections were found to be higher in mongrel breed (60.00% and 42.86%, respectively. Male dogs showed greater involvement in bacterial, fungal, and other non-parasitic dermatoses. Bacteriological culture examination of 40 pus swabs resulted in the growth of 39 bacterial isolates. Mycological culture of skin scrapings from 13

  11. A Clinico - Aetiological Study Of Dermatoses In Paediatric Age Group

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    Ghosh Sadhan K

    1995-01-01

    Full Text Available Five hundred patients of the age group 0-12 years were studied for different types of dermatoses. Pyoderma (35.6%, scabies (22.4% and eczema (17.6% were the most common dermatological conditions, followed by molluscum contagiosum (4.6%, popular urticaria with insect bite (4%, vitiligo (3.4%, miliaria (2.8%, nevus (1.6%. Other dermatoses (8% were pityriasis rosea, wart, chicken pox, herpes zoster, acne vulgaris, leprosy, angular stomatitis, pruritus vulvae, psoriasis, candidiasis, condylomatalata, fixed drug relation, tinea capitis and corporis, phrynoderma, alopecia areata, phimosis, geographic tongue, trichotillomania, canitis, pediculosis, hypertrophic scar and pityriasis versicolor.

  12. Staphylococcal bullous impetigo in a neonate

    Science.gov (United States)

    Duggal, Shalini Dewan; Bharara, Tanisha; Jena, Pragnya Paramita; Kumar, Avinash; Sharma, Abha; Gur, Renu; Chaudhary, Sanjay

    2016-01-01

    An otherwise healthy, full-term neonate presented at day 15 of life to the pediatric emergency with generalized papulo-pustular rash for 2 d. This was finally diagnosed as bullous impetigo caused by Staphylococcus aureus (S. aureus). The skin lesions decreased significantly after starting antibiotic therapy and drainage of blister fluid. There was no recurrence of the lesions on follow-up. This case of generalized pustular eruption due to S. aureus in a neonate is reported, as it poses a diagnostic dilemma and can have serious consequences if left untreated. PMID:27458596

  13. Bullous Mastocytosis Mimicking Congenital Epidermolysis Bullosa

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    Julio Cesar Salas-Alanis

    2014-05-01

    Full Text Available A 2-month-old female infant was referred to DebRA Mexico from the Regional Children's Hospital because of a generalized dermatosis from birth characterized by multiple blisters and erosions on the trunk, face and limbs, associated with minor trauma. A skin biopsy showing subepidermal blisters associated with a dermal infiltrate of Giemsa-positive cells and CD117-positive antibody was consistent with the diagnosis of bullous mastocytosis. Treatment with oral antihistamines, topical steroids, and antibiotics was initiated, leading to a remission of the lesions.

  14. Bullous pemphigoid autoantibodies directly induce blister formation without complement activation.

    Science.gov (United States)

    Ujiie, Hideyuki; Sasaoka, Tetsumasa; Izumi, Kentaro; Nishie, Wataru; Shinkuma, Satoru; Natsuga, Ken; Nakamura, Hideki; Shibaki, Akihiko; Shimizu, Hiroshi

    2014-11-01

    Complement activation and subsequent recruitment of inflammatory cells at the dermal/epidermal junction are thought to be essential for blister formation in bullous pemphigoid (BP), an autoimmune blistering disease induced by autoantibodies against type XVII collagen (COL17); however, this theory does not fully explain the pathological features of BP. Recently, the involvement of complement-independent pathways has been proposed. To directly address the question of the necessity of the complement activation in blister formation, we generated C3-deficient COL17-humanized mice. First, we show that passive transfer of autoantibodies from BP patients induced blister formation in neonatal C3-deficient COL17-humanized mice without complement activation. By using newly generated human and murine mAbs against the pathogenic noncollagenous 16A domain of COL17 with high (human IgG1, murine IgG2), low (murine IgG1), or no (human IgG4) complement activation abilities, we demonstrate that the deposition of Abs, and not complements, is relevant to the induction of blister formation in neonatal and adult mice. Notably, passive transfer of BP autoantibodies reduced the amount of COL17 in lesional mice skin, as observed in cultured normal human keratinocytes treated with the same Abs. Moreover, the COL17 depletion was associated with a ubiquitin/proteasome pathway. In conclusion, the COL17 depletion induced by BP autoantibodies, and not complement activation, is essential for the blister formation under our experimental system. Copyright © 2014 by The American Association of Immunologists, Inc.

  15. Linear immunoglobulin A/immunoglobulin G bullous dermatosis associated with Vogt-Koyanagi-Harada disease.

    Science.gov (United States)

    Yanagihara, Shigeto; Mizuno, Nobuyuki; Naruse, Akiko; Tateishi, Chiharu; Tsuruta, Daisuke; Ishii, Masamitsu

    2011-08-01

    Vogt-Koyanagi-Harada disease is characterized by marked bilateral uveitis associated with symmetric vitiligo, alopecia, poliosis and dysacousia. Linear immunoglobulin (Ig)A bullous dermatosis (LABD) is characterized by small, tense, subepidermal bullae caused by IgA type autoantibody targeting the basal lamina. LABD patients sometimes show coexistence of IgG type autoantibody, termed linear IgA/IgG bullous dermatosis (LAGBD). We reported a 35-year-old Japanese male case of combined LAGBD and Vogt-Koyanagi-Harada disease. His human leukocyte antigen typing was -A24, B52, C*1202, DR*1502, DQ*0601. Immunoblot revealed that patient sera reacted to both 180- and 230-kDa proteins at the IgA and IgG level. Because Vogt-Koyanagi-Harada disease and LABD are reported to be associated with other autoimmune diseases, it is probable that Vogt-Koyanagi-Harada disease and LAGBD in our case may be associated with each other in the pathomechanism. However, we cannot exclude the possibility of this being mere coincidence.

  16. A Rare Case of Vancomycin-Induced Linear Immunoglobulin A Bullous Dermatosis

    Science.gov (United States)

    Swanson, Kurtis; Stromich, Jeremiah; Michalski, Basia M.; Olasz, Edit

    2017-01-01

    Linear IgA bullous dermatosis (LABD) is an autoimmune vesiculobullous disease, which is typically idiopathic but can also rarely be caused by medications or infections. Vancomycin is the most common drug associated with LABD. Lesions typically appear 24 hours to 15 days after the first dose of vancomycin. It is best characterized pathologically by subepidermal bulla (blister) formation with linear IgA deposition at the dermoepidermal junction. Here we report an 86-year-old male with a history of left knee osteoarthritis who underwent a left knee arthroplasty and subsequently developed a prosthetic joint infection. This infection was treated with intravenous vancomycin as well as placement of a vancomycin impregnated joint spacer. Five days following initiation of antibiotic therapy, he presented with a vesiculobullous eruption on an erythematous base over his trunk, extremities, and oral mucosa. The eruption resolved completely when intravenous vancomycin was discontinued and colchicine treatment was begun. Curiously, complete resolution occurred despite the presence of the vancomycin containing joint spacer. The diagnosis of vancomycin-induced linear IgA bullous dermatosis was made based on characteristic clinical and histopathologic presentations. PMID:28168063

  17. A Rare Case of Vancomycin-Induced Linear Immunoglobulin A Bullous Dermatosis

    Directory of Open Access Journals (Sweden)

    Pinky Jha

    2017-01-01

    Full Text Available Linear IgA bullous dermatosis (LABD is an autoimmune vesiculobullous disease, which is typically idiopathic but can also rarely be caused by medications or infections. Vancomycin is the most common drug associated with LABD. Lesions typically appear 24 hours to 15 days after the first dose of vancomycin. It is best characterized pathologically by subepidermal bulla (blister formation with linear IgA deposition at the dermoepidermal junction. Here we report an 86-year-old male with a history of left knee osteoarthritis who underwent a left knee arthroplasty and subsequently developed a prosthetic joint infection. This infection was treated with intravenous vancomycin as well as placement of a vancomycin impregnated joint spacer. Five days following initiation of antibiotic therapy, he presented with a vesiculobullous eruption on an erythematous base over his trunk, extremities, and oral mucosa. The eruption resolved completely when intravenous vancomycin was discontinued and colchicine treatment was begun. Curiously, complete resolution occurred despite the presence of the vancomycin containing joint spacer. The diagnosis of vancomycin-induced linear IgA bullous dermatosis was made based on characteristic clinical and histopathologic presentations.

  18. Bullous oral lichen planus: report of two cases

    Institute of Scientific and Technical Information of China (English)

    Berrin (U)NSAL; S. Elif G(U)LTEKIN; Erol BAL; Benay TOKMAN

    2003-01-01

    @@ Lichen planus is a common chronic mucocutaneous disease of unknown cause. Several types of lichen planus (reticular, atrophic, erosive and bullous) within the oral cavity have been described, among which the reticular and erosive types are the most common types,1,2 while the bullous type is rarely observed.1,3 Bullous lichen planus (BLP) lesions are commonly seen on the buccal mucosa, most frequently at the posterior areas adjacent to the second and third molar teeth. Less common localizations are gingiva and inner aspect of the lips.4 Bullae are generally short lived and leave ulcerated lesions on rupturing.1 The clinical diagnosis of bullous lichen planus is extremely difficult, and pathological examinations may be necessary to establish a definitive diagnosis.5

  19. Occurrence of hereditary bullous epidermolyses in Croatia.

    Science.gov (United States)

    Pavicić, Z; Kmet-Vizintin, P; Kansky, A; Dobrić, I

    1990-06-01

    To determine the occurrence of hereditary bullous epidermolyses (EB) in Croatia, Yugoslavia, from 1960 to 1987, cases were gathered from the hospital files of dermatologic and pediatric clinics and departments throughout the area. The diagnosis of EB type was made on the basis of clinical features, patients' histories, and light microscopy and electron microscopy findings. Fifty families with 58 patients were registered; 44 patients were examined personally by one of the authors. The most frequent type of EB in Croatia was recessive dystrophic EB Hallopeau-Siemens, occurring in 35 of the 58 individuals. Regional accumulation of cases within the Varazdin area was noted (13 patients). Prevalence of EB in Croatia is 0.956 cases per 100,000 inhabitants. One case of recessive dystrophic EB Hallopeau-Siemens occurred in about every 52,000 live births.

  20. The treatment of bullous rhegmatogenous retinal detachment.

    Science.gov (United States)

    Wong, D; Chignell, A H; Inglesby, D V; Little, B C; Franks, W

    1992-01-01

    We describe the results of a consecutive series of 97 cases of bullous superior retinal detachment treated by conventional surgery. The retinal detachments were characterized by either a single retinal break or multiple retinal breaks confined within 1 clock hour and no proliferative vitreoretinopathy. The surgery involved sequential drainage of subretinal fluid, injection of air, cryotherapy and the application of local explant. All cases would otherwise be suitable for pneumatic retinopexy. The anatomical success rate was 85.5% with a single operation and 97% with further procedures. We report on the complications encountered and appraise the advantages and disadvantages of this operation. Forty-five of the 97 cases had detachment of the macula for less than 2 weeks, and 35 of the 45 (80%) achieved a visual acuity of 6/18 or better. These visual results challenge the assertion that better visual outcome might be attained with pneumatic retinopexy.

  1. Extensive bullous lichen sclerosus et atrophicus*

    Science.gov (United States)

    Vukicevic, Jelica

    2016-01-01

    Lichen sclerosus et atrophicus is a chronic disease of unknown etiology characterized by atrophic and sclerotic plaques in both genital and extragenital regions. Extensive bullous lichen sclerosus et atrophicus (BLSA) is a severe variant of the disease with no widely accepted treatment. We present a 63-year-old woman with extensive extragenital, ivory-colored, atrophic plaques on her trunk and extremities and disseminated hemorrhagic bullae. The patient was unsuccessfully treated with standard topical corticosteroid therapy, doxycycline and chloroquine. According to the literature, there is little evidence of the efficacy of doxycycline and hydroxychloroquine in the treatment of BLSA. We report a rare case of extensive BLSA that is unresponsive to these drugs.

  2. A Case of Oropharyngeal Bullous Pemphigoid Presenting with Haemoptysis

    Directory of Open Access Journals (Sweden)

    C. M. Lee

    2015-01-01

    Full Text Available Objective. Bullous pemphigoid is well known for its cutaneous features; however in rare cases it may present with mucosal involvement. We report a case of bullous pemphigoid presenting with haemoptysis, initially presenting to the Ear, Nose and Throat Department for investigation. Methods. An 87-year-old lady was admitted with haemoptysis. She also complained of a spreading, pruritic, bullous rash, which first began three weeks previously. Initial investigations, which included nasendoscopy, revealed a normal nasal mucosa and a normal postnasal space. A large deroofed blister was observed on the soft palate. The presenting symptoms and signs raised the suspicion of an immunobullous disease including bullous pemphigoid. Conclusion. Bullous pemphigoid (BP is a subepidermal immunobullous disease that typically manifests in elderly patient populations. Although rare, BP can present in a mucocutaneous fashion akin to its more aggressive variant, mucous membrane pemphigoid (MMP. Differentiation of the two is based on clinical grounds, with the prevailing feature for the latter being the predominance of mucosal involvement, which may be extensive. The mainstay of treatment for bullous pemphigoid is steroid therapy, which may be administered both topically and systemically. A deeper understanding into the pathophysiology of the various immunobullous diseases may assist in our understanding of how the various disease entities manifest themselves.

  3. Nursing diagnoses in patients with immune-bullous dermatosis.

    Science.gov (United States)

    Brandão, Euzeli da Silva; Santos, Iraci Dos; Lanzillotti, Regina Serrão; Ferreira, Adriano Menis; Gamba, Mônica Antar; Azulay-Abulafia, Luna

    2016-08-15

    identify nursing diagnoses in patients with immune-bullous dermatosis. a quantitative and descriptive research, carried out in three institutions located in Rio de Janeiro and Mato Grosso do Sul, Brazil, using the Client Assessment Protocol in Dermatology during a nursing consultation. Simple descriptive statistics was used for data analysis. 14 subjects participated in the study, nine with a diagnosis of pemphigus vulgaris, pemphigus two and three of bullous pemphigoid. The age ranged between 27 and 82 years, predominantly females (11). 14 nursing diagnoses were discussed and identified from a clinical rationale in all study participants, representing the most common human responses in this sample. The application of the Assessment Protocol in Dermatology facilitated the comprehensive assessment, in addition to providing the identification of diagnostics according to the North American Nursing Diagnosis Association International. the nursing diagnoses presented confirm the necessity of interdisciplinary work during the care for this clientele. For better description of the phenomena related to the client in question, it is suggested the inclusion of two risk factors related in three diagnoses of this taxonomy. It is worth noting the contribution of the findings for the care, education and research in nursing in dermatology. identificar diagnósticos de enfermagem em clientes com dermatoses imunobolhosas. pesquisa quantitativa e descritiva, realizada em três instituições localizadas no Rio de Janeiro e no Mato Grosso do Sul-Brasil, aplicando o Protocolo de Avaliação do Cliente em Dermatologia, durante consulta de enfermagem. Utilizou-se a estatística descritiva simples para análise dos dados. participaram do estudo 14 sujeitos, nove com diagnóstico médico de pênfigo vulgar, dois de foliáceo e três de penfigoide bolhoso. A idade variou entre 27 e 82 anos, predominando 11 pessoas do sexo feminino. Foram discutidos 14 diagnósticos de enfermagem

  4. B cell epitope spreading: mechanisms and contribution to autoimmune diseases.

    Science.gov (United States)

    Cornaby, Caleb; Gibbons, Lauren; Mayhew, Vera; Sloan, Chad S; Welling, Andrew; Poole, Brian D

    2015-01-01

    While a variety of factors act to trigger or initiate autoimmune diseases, the process of epitope spreading is an important contributor in their development. Epitope spreading is a diversification of the epitopes recognized by the immune system. This process happens to both T and B cells, with this review focusing on B cells. Such spreading can progress among multiple epitopes on a single antigen, or from one antigenic molecule to another. Systemic lupus erythematosus, multiple sclerosis, pemphigus, bullous pemphigoid and other autoimmune diseases, are all influenced by intermolecular and intramolecular B cell epitope spreading. Endocytic processing, antigen presentation, and somatic hypermutation act as molecular mechanisms that assist in driving epitope spreading and broadening the immune response in autoimmune diseases. The purpose of this review is to summarize our current understanding of B cell epitope spreading with regard to autoimmunity, how it contributes during the progression of various autoimmune diseases, and treatment options available.

  5. Vulvar Skin Disorders throughout Lifetime: About Some Representative Dermatoses

    Directory of Open Access Journals (Sweden)

    Jean Doyen

    2014-01-01

    Full Text Available The objective of this paper is to present general considerations which should be kept in mind by clinicians in charge of women with vulvar diseases. Four representative vulvar dermatoses are described. Lichen simplex chronicus is a pathological condition related to chemical and mechanical irritant agents. Detrimental effects of these irritants, in the presence of other dermatoses, have to be considered when therapeutic responses are unsatisfactory. Lichen sclerosus is the most common vulvar dermatosis in elderly. However, it should be kept in mind that it may be diagnosed at any age. Lichen planus, in spite of sharing a similar range of etiological factors with lichen sclerosus, is a very distinct entity. Finally, Paget’s disease, although rare, is also described especially because of the challenge it represents both clinically and therapeutically.

  6. (Industrial dermatoses among the Belthatow brown coal miners)

    Energy Technology Data Exchange (ETDEWEB)

    Ruszczak, Z.; Bienias, L.; Proszyncka-Kuczynska, W.

    1981-01-01

    443 subjects were examined, 235 applying to the physician due to the occurrence of skin dermatoses (group I) and 208 workers referred for periodic examinations (group II). In group I skin dermatoses were found in 100, i.e. 85%, of the subjects and in group II in 99 i.e. 48% of the subjects. In both groups the most frequent dermatosis was feet skin inflammation, especially interdigital intertrigo. Oil acne was diagnosed in 15, ordinary acne in 23, pityriasis versicolor in 19. erythrasma in 10, and eczema and contact dermatitis in 7 subjects of the first group and 3 subjects of the other group. 204 subjects with feet skin pathologies underwent mycologic examinations and 36 subjects--also bacteriologic examinations. The results of those studies indicate that in 23% of the subjects, feet interdigital intertrigo results from mycologic infections. Bacterial infections may contribute to etiopathogenesis. Imidazole compounds are useful for the treatment and prevention of interdigital intertrigo.

  7. Dermatoses provocadas por plantas (fitodermatoses Dermatosis due to plants (phytodermatosis

    Directory of Open Access Journals (Sweden)

    Vitor Manoel Silva dos Reis

    2010-08-01

    Full Text Available As dermatoses causadas por plantas são relativamente comuns no nosso meio e podem ocorrer por diversos mecanismos patogênicos. São descritas dermatoses por trauma físico, por ação farmacológica, mediadas por IgE, por irritação, por ação conjunta da luz e por sensibilização. Também são descritas na introdução desta revisão as pseudofitodermatoses causadas por elementos veiculados pelas plantas e, por isso, aparentemente causadas pelas plantas.Dermatosis caused by plants is relatively common and may occur by various pathogenic mechanisms. Dermatitis due to physical trauma, pharmacological action, irritation, sensitization, mediated by IgE and induced by light are described. Pseudophytodermatosis caused by plant-delivered elements is also described in the introduction to this work.

  8. Occupational Dermatoses by Type of Work in Greece

    OpenAIRE

    Zorba, Eleni; Karpouzis, Antony; Zorbas, Alexandros; Bazas, Theodore; Zorbas, Sam; Alexopoulos, Elias; Zorbas, Ilias; Kouskoukis, Konstantinos; Konstandinidis, Theodoros

    2013-01-01

    Background To elucidate the relationship between seven occupational dermatoses (ODs) and 20 types of work in Greece. Methods This was a prevalence epidemiologic study of certain ODs among 4,000 workers employed in 20 types of enterprise, in 104 companies, in 2006–2012, using data from company medical records, questionnaires, occupational medical, and special examinations. The χ2 test was applied to reveal statistically significant relationships between types of enterprises and occurrence of O...

  9. Prevalence of Various Dermatoses in School Children of Anand District

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    Rita Vora, Nishit Bodiwala, Shivang Patel

    2012-01-01

    Full Text Available Introduction: Skin diseases are common in children. School survey is a useful yardstick as it is easy to conduct, less time consuming and large number of children of particular age group can be screened for presence of diseases. Method: This study was conducted in 48 schools (32 urban and semi urban schools,16 rural school of Anand district over a period of 5 years from Jan 2006 to Dec 2010. Total 26177 students comprising 15248 male and 10929 female from KG to 12th standard were examined. Result: Out of 26,177 students, 15.41 %( 4035 students were found to have skin disorders. Out of this, 18.14% (732 had infectious dermatoses, 79.60% (3212 had non-infectious dermatoses and 2.26%(91 had nutritional dermatoses. Discussion: The prevalence of skin disease (15.41% reported in our study is quite less than other studies. Infectious disease showed low incidence compared to other studies due to proper hygienic condition.

  10. Piperacillin-tazobactam-induced linear IgA bullous dermatosis presenting clinically as Stevens-Johnson syndrome/toxic epidermal necrolysis overlap.

    Science.gov (United States)

    Adler, N R; McLean, C A; Aung, A K; Goh, M S Y

    2017-04-01

    Linear IgA bullous dermatosis (LABD) is a subepidermal autoimmune bullous disease characterized by linear IgA deposition at the basement membrane zone, which is visualized by direct immunofluorescence. Patients with LABD typically present with widespread vesicles and bullae; however, this is not necessarily the case, as the clinical presentation of this disease is heterogeneous. LABD clinically presenting as Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) is an infrequent, yet well-described phenomenon. Most cases of LABD are idiopathic, but some cases are drug-induced. Multiple drugs have been implicated in the development of LABD. We report a case of piperacillin-tazobactam-induced LABD presenting clinically as SJS/TEN overlap. This is the first reported case of a strong causal association between piperacillin-tazobactam and the development of LABD.

  11. A case of vancomycin-associated linear IgA bullous dermatosis and IgA antibodies to the α3 subunit of laminin-332.

    Science.gov (United States)

    Zenke, Y; Nakano, T; Eto, H; Koga, H; Hashimoto, T

    2014-04-01

    Linear IgA bullous dermatosis (LABD) is a rare autoimmune bullous disease, which is defined by the histopathological finding of subepidermal vesicles with neutrophilic infiltration and linear IgA deposits in the basement membrane zone, revealed by immunofluorescence study. We present a case of LABD in which vancomycin (VCM) administration triggered LABD, and immunoblot analysis showed IgA antibodies reactive to the 145- and 165-kDa α3 subunits of laminin-332. This is the first report of VCM-associated LABD in which the target antigen was laminin-332. In the present case, we were compelled to continue administration of VCM along with systemic steroids, which eventually led to the attenuation of the symptoms, normalization of the serum IgA level, and negative results on both indirect immunofluorescence of 1 mol L(-1) NaCl-split skin and immunoblot analysis.

  12. Activation of Blood Coagulation in Two Prototypic Autoimmune Skin Diseases: A Possible Link with Thrombotic Risk.

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    Massimo Cugno

    Full Text Available Coagulation activation has been demonstrated in two prototypic autoimmune skin diseases, chronic autoimmune urticaria and bullous pemphigoid, but only the latter is associated with increased thrombotic risk. Two markers of coagulation activation (prothrombin fragment F1+2 and fibrin fragment D-dimer were measured by immunoenzymatic methods in plasma samples from 30 patients with active chronic autoimmune urticaria, positive for autologous serum skin test, 30 patients with active bullous pemphigoid and 30 healthy subjects. In skin biopsies, tissue factor expression was evaluated by both immunohistochemistry and in situ hybridization. F1+2 and D-dimer levels were higher in active chronic autoimmune urticaria (276.5±89.8 pmol/L and 5.56±4.40 nmol/L, respectively than in controls (145.2±38.0 pmol/L and 1.06±0.25 nmol/L; P=0.029 and P=0.011 and were much higher in active bullous pemphigoid (691.7±318.7 pmol/L and 15.24±9.09 nmol/L, respectively (P<0.0001. Tissue factor positivity was evident in skin biopsies of both disorders with higher intensity in bullous pemphigoid. F1+2 and D-dimer, during remission, were markedly reduced in both disorders. These findings support the involvement of coagulation activation in the pathophysiology of both diseases. The strong systemic activation of coagulation in bullous pemphigoid may contribute to increase the thrombotic risk and provides the rationale for clinical trials on anticoagulant treatments in this disease.

  13. A case of mixed bullous disease of epidermolysis bullosa acquisita and linear IgA bullous dermatosis.

    Science.gov (United States)

    Osawa, Masumi; Demitsu, Toshio; Toda, Sunao; Yokokura, Hideto; Umemoto, Naoka; Yamada, Tomoko; Yoneda, Kozo; Kakurai, Maki; Yoshida, Mariko; Hashimoto, Takashi

    2005-01-01

    A 75-year-old Japanese male visited us with bullous eruptions on the extremities. Physical examination revealed large bullae on the hands, lower legs and feet. The oral mucosa was also involved. Histology disclosed subepidermal blister with inflammatory cell infiltrates in the dermis. Direct immunofluorescence showed deposits of IgG and IgA at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient's IgG antibodies reacted with the dermal side of the split, while IgA antibodies reacted with the epidermal side. Immunoblotting showed that the patient's serum reacted with the NC1 domain of type VII collagen (290-kDa epidermolysis bullosa acquisita antigen) as well as the 120-kDa linear IgA bullous dermatosis antigen, LAD-1. Systemic prednisolone resulted in a favorable response. From the clinicopathological findings, the present case is not consistent with either epidermolysis bullosa acquisita or IgA bullous dermatosis. Therefore, we regarded the case as mixed bullous disease of epidermolysis bullosa acquisita and linear IgA bullous dermatosis. Such a case has not been previously reported.

  14. Dermatoses em pacientes com diabetes mellitus Skin lesions in diabetic patients

    National Research Council Canada - National Science Library

    N T Foss; D P Polon; M H Takada; M C Foss-Freitas; M C Foss

    2005-01-01

    ... predomínio de dermatofitoses (82,6%), seguido de grupo de dermatoses como acne e degeneração actínica (66,7%), piodermites (5%), tumores cutâneos (3%) e necrobiose lipoídica (1%). Entre as dermatoses mais comuns em...

  15. Serological Diagnosis of Autoimmune Blistering Diseases

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    Birgül Özkesici

    2016-03-01

    Full Text Available Autoimmune blistering diseases are a rare diseases, characterized by development of autoantibodies against the structural proteins of the epidermis or dermoepidermal junction, and blisters and erosions on skin and/or mucous membranes clinically. Clinical features are important guiding findings for suspicious of this group of diseases. The diagnosis is achieved by the evaluation together of clinical features, histological and immunological findings. The gold standard in the diagnosis of this group diseases are demonstration of tissue bound and/or circulating autoantibodies. Methods for this purpose are; direct and indirect immunofluorescence, Enzyme Linked Immunosorbent Assay (ELISA, immunoprecipitation and immunoblotting. The aim of this paper is to review serological diagnostic methods in the diagnosis of autoimmune bullous diseases and to present developments in recent years.

  16. Extent and pattern of paediatric dermatoses in rural areas of central India

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    Bhatia Vikas

    1997-01-01

    Full Text Available A house to house study was done on 666 children aged 0-14 years in 5 villages of Wardha district in Maharastra (Central India. 346 children (51.95% had one or more dermatoses. Infective dermatoses contributed 63.5% of all dermatoses, while noninfectious and nutritional deficiency dermatoses were responsibile for 21.2% and 15.2%, respectively. Pediculosis capitis was seen in 136 children (20.4%, followed by pyoderma in 107 (16.07% and dematophytosis in 44 (6.61%. Scabies was found in only 6 and 4 cases of leprosy were also delected. Pityriasis alba, pityriasis capitis amond non-infectious; hair and skin changes among nutritional deficiency dermatoses were leading presentations.

  17. Evidence for a role of eosinophils in blister formation in bullous pemphigoid.

    Science.gov (United States)

    de Graauw, E; Sitaru, C; Horn, M; Borradori, L; Yousefi, S; Simon, H-U; Simon, D

    2017-07-01

    Bullous pemphigoid (BP) is an autoimmune bullous disease of the skin characterized by subepidermal blister formation due to tissue-bound and circulating autoantibodies to the hemidesmosomal antigens BP180 and BP230. Although eosinophils and their toxic mediators are found abundantly in BP lesions, their role in blister formation has remained unclear. To investigate the role of eosinophils in the pathogenesis of BP with a specific focus on blister formation and to define conditions inducing dermal-epidermal separation (DES). In an ex vivo human model of BP, normal human skin cryosections were incubated with purified human peripheral blood eosinophils with or without activation in the presence or absence of BP autoantibodies, brefeldin A, diphenyleneiodonium, DNase or blocking F(ab')2 fragments to CD16, CD18, CD32 and CD64. Dermal-epidermal separation was assessed by light microscopy studies and quantified using Fiji software. Following activation with IL-5 and in the presence of BP autoantibodies, eosinophils induced separation along the dermal-epidermal junction of ex vivo skin. Dermal-epidermal separation was significantly reduced by blocking any of the following: Fcγ receptor binding (P = 0.048), eosinophil adhesion (P = 0.046), reactive oxygen species (ROS) production (P = 0.002), degranulation (P blister formation in the presence of BP autoantibodies. Dermal-epidermal separation by IL-5-activated eosinophils depends on adhesion and Fcγ receptor activation, requires elevated ROS production and degranulation and involves EET formation. Thus, targeting eosinophils may be a promising therapeutic approach for BP. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  18. Autoimmune epilepsy.

    Science.gov (United States)

    Greco, Antonio; Rizzo, Maria Ida; De Virgilio, Armando; Conte, Michela; Gallo, Andrea; Attanasio, Giuseppe; Ruoppolo, Giovanni; de Vincentiis, Marco

    2016-03-01

    Despite the fact that epilepsy is the third most common chronic brain disorder, relatively little is known about the processes leading to the generation of seizures. Accumulating data support an autoimmune basis in patients with antiepileptic drug-resistant seizures. Besides, recent studies show that epilepsy and autoimmune disease frequently co-occur. Autoimmune epilepsy is increasingly recognized in the spectrum of neurological disorders characterized by detection of neural autoantibodies in serum or spinal fluid and responsiveness to immunotherapy. An autoimmune cause is suspected based on frequent or medically intractable seizures and the presence of at least one neural antibody, inflammatory changes indicated in serum or spinal fluid or on MRI, or a personal or family history of autoimmunity. It is essential that an autoimmune etiology be considered in the initial differential diagnosis of new onset epilepsy, because early immunotherapy assures an optimal outcome for the patient.

  19. [Autoimmune hepatitis].

    Science.gov (United States)

    Ostojić, Rajko

    2003-01-01

    Autoimmune hepatitis is an unresolving, hepatocellular inflammation of unknown cause that is characterized by the presence of periportal hepatitis on histologic examination, tissue autoantibodies in serum, and hypergammaglobulinemia. By international consensus, the designation autoimmune hepatitis has replaced alternative terms for the condition. Three types of autoimmune hepatitis have been proposed based on immunoserologic findings. Type 1 autoimmune hepatitis is characterized by the presence of antinuclear antibodies (ANA) or smooth muscle antibodies (SMA) (or both) in serum. Seventy percent of patients with type 1 of autoimmune hepatitis are women. This type is the most common form and accounts for at least 80% of cases. Type 2 is characterized by the presence of antibodies to liver-kidney microsome type 1 (anti-LKM1) in serum. Patients with this type of autoimmune hepatitis are predominantly children. Type 3 autoimmune hepatitis is characterized by the presence of antibodies to soluble liver antigen (anti-SLA) in serum. There are no individual features that are pathognomonic of autoimmune hepatitis, and its diagnosis requires the confident exclusion of other conditions. The large majority of patients show satisfactory response to corticosteroid (usually prednisone or prednisolone) therapy. For the past 30 years it has been customary to add azathioprine as a "steroid sparing" agent to allow lower doses of steroids to be used and remission, once achieved, can be sustained in many patients with azathioprine alone after steroid withdrawal. Patients with autoimmune hepatitis who have decompensated during or after corticosteroid therapy are candidates for liver transplantation.

  20. Autoimmune hepatitis

    Science.gov (United States)

    ... PA: Elsevier Saunders; 2010:chap 88. Read More Autoimmune disorders Chronic thyroiditis (Hashimoto disease) Cirrhosis Glomerulonephritis Hemolytic anemia Liver cancer - hepatocellular carcinoma Mesenteric venous thrombosis Type ...

  1. Dermatoses of Pregnancy - Clues to Diagnosis, Fetal Risk and Therapy

    Science.gov (United States)

    2011-01-01

    The specific dermatoses of pregnancy represent a heterogeneous group of pruritic skin diseases that have been recently reclassified and include pemphigoid (herpes) gestationis, polymorphic eruption of pregnancy (syn. pruritic urticarial papules and plaques of pregnancy), intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. They are associated with severe pruritus that should never be neglected in pregnancy but always lead to an exact work-up of the patient. Clinical characteristics, in particular timing of onset, morphology and localization of skin lesions are crucial for diagnosis which, in case of pemphigoid gestationis and intrahepatic cholestasis of pregnancy, will be confirmed by specific immunofluorescence and laboratory findings. While polymorphic and atopic eruptions of pregnancy are distressing only to the mother because of pruritus, pemphigoid gestationis may be associated with prematurity and small-for-date babies and intrahepatic cholestasis of pregnancy poses an increased risk for fetal distress, prematurity, and stillbirth. Corticosteroids and antihistamines control pemphigoid gestationis, polymorphic and atopic eruptions of pregnancy; intrahepatic cholestasis of pregnancy, in contrast, should be treated with ursodeoxycholic acid. This review will focus on the new classification of pregnancy dermatoses, discuss them in detail, and present a practical algorithm to facilitate the management of the pregnant patient with skin lesions. PMID:21909194

  2. Histological evaluation of corneal scar formation in pseudophakic bullous keratopathy.

    Directory of Open Access Journals (Sweden)

    Ting Liu

    Full Text Available PURPOSE: To evaluate histological changes in the corneal stroma in pseudophakic bullous keratopathy. METHODS: Twenty-eight patients (28 eyes with pseudophakic bullous keratopathy underwent therapeutic penetrating keratoplasty at Shandong Eye Institute between January 2006 and November 2011. The patients were divided into two groups according to the duration of bullous keratopathy (1.0 year group, and three buttons from enucleated eyes with choroidal melanoma served as a control. In vivo confocal microscopy examination, hematoxylin-eosin, Masson's trichrome stain and Van Gieson staining were used for microscopic examination. The histological evaluation and scoring of the buttons for morphological changes, including the degree of stromal scars, neovascularization and inflammatory cells within the corneal buttons, were compared. To study the underlying mechanism, connective tissue growth factor (CTGF and TGF-β immunohistochemistry were performed. RESULTS: Confocal microscopy examination and histological evaluation and scoring of the buttons showed that compared with the 1.0 year group (P1.0 year group. CONCLUSIONS: During the progression of pseudophakic bullous keratopathy, stromal scars occurred more often in the patients that had a longer duration of disease. Cytokines such as CTGF and TGF-β1 may play a role in this pathological process and deserve further investigation.

  3. BULLOUS PEMPHIGOID SUCCESSFULLY CONTROLLED BY TETRACYCLINE AND NICOTINAMIDE

    NARCIS (Netherlands)

    KOLBACH, DN; REMME, JJ; BOS, WH; JONKMAN, MF; DEJONG, MCJM; PAS, HH; VANDERMEER, JB

    1995-01-01

    In 1986, Berk and Lorincz reported the efficacy of tetracycline and nicotinamide in the treatment of bullous pemphigoid (BP). In the present study of seven patients with BP, we found that a regimen of 2 g tetracycline combined with 2 g nicotinamide daily was effective in clearing the skin lesions. T

  4. Bullous reactions to bed bug bites reflect cutaneous vasculitis

    Science.gov (United States)

    This study evaluates bullous cutaneous reactions and sequential histopathology in an individual sensitized to bed bug bites in an effort to better understand the allergic response and histology associated with these bites. There was a progression of the inflammatory response across time ranging from...

  5. Scabies presenting with bullous pemphigoid-like lesions.

    Science.gov (United States)

    Ansarin, Habib; Jalali, Mir Hadi Aziz; Mazloomi, Shadi; Soltani-Arabshahi, Razieh; Setarehshenas, Roya

    2006-01-27

    A wide range of clinical manifestations may be seen in scabies, from classic pruritic papules and burrows to secondary features such as impetigo. Bullus lesions are a less frequent. Twenty cases of scabies presenting with bullae have been reported so far in the medical literature. Differentiating this subtype of scabies from the immunobullous disease bullus pemphigoid is a diagnostic challenge. A 42-year-old man was referred to our dermatology outpatient clinic with 3-month history of severe pruritus and tense blisters affecting mainly the lower trunk, arms and legs. An initial biopsy was suggestive for bullous pemphigoid. Close physical examination revealed small excoriated papules and a few burrows on borders of the hands and wrists. Skin scraping of the lesions on wrists was positive for Sarcoptes scabiei. Another biopsy specimen from a recent blister revealed subepidermal bullae with fibrin and inflammatory cells, particularly eosinophils. Direct immunofluorescence exam was negative. The patient was treated with lindane lotion followed by crotamiton cream with near complete resolution of the lesions. Scabies must be considered in patients presenting with recent onset of unexplained pruritic bullous lesions. Biopsy and immunofluorescence studies together with skin scrapings for Sarcoptes scabiei could help to differentiate these cases from bullous pemphigoid. Antiscabietic treatment results in resolution of bullous lesions in the affected patients.

  6. Bullous Pemphigoid as Pruritus in the Elderly A Common Presentation

    NARCIS (Netherlands)

    Bakker, Christiaan V.; Terra, Jorrit B.; Pas, Hendri H.; Jonkman, Marcel F.

    2013-01-01

    IMPORTANCE In the literature, patients with bullous pemphigoid have been reported to have itch without blisters. Clinical observations in these patients have varied from eczematous or urticarial to papular or nodular skin lesions. Here we investigated the spectrum of clinical variants. OBSERVATIONS

  7. Bullous impetigo associated with Abiotrophia defectiva in an immunocompetent adult.

    Science.gov (United States)

    Anderson, Heather M; Miller, Cathy; Kemp, Earl; Huntington, Mark K

    2012-07-01

    Infection of humans by Abiotrophia defectiva, a nutritionally variant streptococcus, most commonly takes the form of endocarditis, though a variety of other manifestations ranging from central nervous system abscesses to orthopaedic infections have been seen. We report here what we believe is the first case of bullous impetigo associated with this organism.

  8. Linear IgA bullous dermatosis following influenza vaccination.

    Science.gov (United States)

    Alberta-Wszolek, Lauren; Mousette, Alyse M; Mahalingam, Meera; Levin, Nikki A

    2009-11-15

    Linear IgA Bullous Dermatosis (LABD) is an immune-mediated subepidermal vesiculobullous eruption characterized by linear deposits of IgA at the basement membrane zone. Most cases are idiopathic but medications, infections, and malignancies have also been reported to induce LABD. We report the case of a 54-year-old woman who developed LABD shortly after receiving an influenza vaccination.

  9. Bullous Variant of Central Serous Chorioretinopathy: Expansion of Phenotypic Features Using Multimethod Imaging.

    Science.gov (United States)

    Balaratnasingam, Chandrakumar; Freund, K Bailey; Tan, Anna M; Mrejen, Sarah; Hunyor, Alex P; Keegan, David J; Dansingani, Kunal K; Dayani, Pouya N; Barbazetto, Irene A; Sarraf, David; Jampol, Lee M; Yannuzzi, Lawrence A

    2016-07-01

    To define the phenotypic characteristics of the bullous variant of central serous chorioretinopathy (CSC) using multimethod imaging. Retrospective, observational case series. Twenty-one eyes of 14 patients with bullous retinal detachment resulting from CSC (bullous CSC group) and 122 eyes of 84 patients with chronic CSC without bullous retinal detachment (nonbullous CSC group). We performed a retrospective review of clinical and multimethod imaging data of patients who sought treatment from the authors with bullous retinal detachment resulting from CSC between January 2010 and November 2015. Multimethod imaging comprised color photography, fluorescein angiography, fundus autofluorescence, and high-resolution optical coherence tomography. Consecutive cases of chronic CSC without bullous retinal detachment, seen during the same period, comprised a comparative group. Qualitative and quantitative characteristics of the choroid, retinal pigment epithelium, and retina were compared between the 2 groups. Mean age of the bullous CSC group was 53.8 years. There was no difference in age, visual acuity, corticosteroid use, or the proportion of white patients and men between the 2 groups (all P > 0.132). Peripheral nonperfusion occurred only in eyes with bullous retinal detachment (38% of cases). Retinal pigment epithelial tears were seen in 95% of eyes in the bullous group and none of the eyes in the nonbullous CSC group. The bullous CSC group demonstrated a greater number of pigment epithelial detachments (PEDs) and more eyes demonstrated PEDs with internal hyperreflectivity (both P features. Copyright © 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

  10. Autoimmune myelopathies.

    Science.gov (United States)

    Flanagan, Eoin P

    2016-01-01

    Autoimmune myelopathies are a heterogeneous group of immune-mediated spinal cord disorders with a broad differential diagnosis. They encompass myelopathies with an immune attack on the spinal cord (e.g., aquaporin-4-IgG (AQP4-IgG) seropositive neuromyelitis optica (NMO) and its spectrum disorders (NMOSD)), myelopathies occurring with systemic autoimmune disorders (which may also be due to coexisting NMO/NMOSD), paraneoplastic autoimmune myelopathies, postinfectious autoimmune myelopathies (e.g., acute disseminated encephalomyelitis), and myelopathies thought to be immune-related (e.g., multiple sclerosis and spinal cord sarcoidosis). Spine magnetic resonance imaging is extremely useful in the evaluation of autoimmune myelopathies as the location of signal change, length of the lesion, gadolinium enhancement pattern, and evolution over time narrow the differential diagnosis considerably. The recent discovery of multiple novel neural-specific autoantibodies accompanying autoimmune myelopathies has improved their classification. These autoantibodies may be pathogenic (e.g., AQP4-IgG) or nonpathogenic and more reflective of a cytotoxic T-cell-mediated autoimmune response (collapsin response mediator protein-5(CRMP5)-IgG). The presence of an autoantibody may help guide cancer search, assist treatment decisions, and predict outcome/relapse. With paraneoplastic myelopathies the initial goal is detection and treatment of the underlying cancer. The aim of immunotherapy in all autoimmune myelopathies is to maximize reversibility, maintain benefits (while preventing relapse), and minimize side effects.

  11. From autoimmunity to autoimmune disease

    NARCIS (Netherlands)

    Franco Salinas, G.

    2012-01-01

    This thesis describes several mechanisms in which T and B lymphocytes, together with the costimulatory molecules and cytokines that influence their behavior, are fundamental in the progression of autoimmunity to autoimmune disease. If these mechanisms can be understood in greater detail, more

  12. Role of IgE in autoimmunity.

    Science.gov (United States)

    Sanjuan, Miguel A; Sagar, Divya; Kolbeck, Roland

    2016-06-01

    There is accumulating evidence to suggest that IgE plays a significant role in autoimmunity. The presence of circulating self-reactive IgE in patients with autoimmune disorders has been long known but, at the same time, largely understudied. However, studies have shown that the increased IgE concentration is not associated with higher prevalence for atopy and allergy in patients with autoimmune diseases, such as systemic lupus erythematosus. IgE-mediated mechanisms are conventionally known to facilitate degranulation of mast cells and basophils and promote TH2 immunity, mechanisms that are not only central to mounting an appropriate defense against parasitic worms, noxious substances, toxins, venoms, and environmental irritants but that also trigger exuberant allergic reactions in patients with allergies. More recently, IgE autoantibodies have been recognized to participate in the self-inflicted damaging immune responses that characterize autoimmunity. Such autoimmune responses include direct damage on tissue-containing autoantigens, activation and migration of basophils to lymph nodes, and, as observed most recently, induction of type 1 interferon responses from plasmacytoid dendritic cells. The importance of IgE as a central pathogenic mechanism in autoimmunity has now been clinically validated by the approval of omalizumab, an anti-IgE mAb, for patients with chronic spontaneous urticaria and for the clinical benefit of patients with bullous pemphigoid. In this review we summarize recent reports describing the prevalence of self-reactive IgE and discuss novel findings that incriminate IgE as central in the pathogenesis of inflammatory autoimmune disorders.

  13. Autoimmune gastritis.

    Science.gov (United States)

    Kulnigg-Dabsch, Stefanie

    2016-10-01

    Autoimmune gastritis is a chronic inflammatory disease with destruction of parietal cells of the corpus and fundus of the stomach. The known consequence is vitamin B12 deficiency and, consequently, pernicious anemia. However, loss of parietal cells reduces secretion of gastric acid which is also required for absorption of inorganic iron; thus, iron deficiency is commonly found in patients with autoimmune gastritis. This usually precedes vitamin B12 deficiency and is found mainly in young women. Patients with chronic iron deficiency, especially those refractory to oral iron therapy, should therefore be evaluated for the presence of autoimmune gastritis.

  14. Lichen planus and other lichenoid dermatoses: Kids are not just little people.

    Science.gov (United States)

    Payette, Michael J; Weston, Gillian; Humphrey, Stephen; Yu, JiaDe; Holland, Kristen E

    2015-01-01

    Lichenoid dermatoses, a group of inflammatory skin conditions with characteristic clinical and histopathologic findings, range from common to rare. Classic lichen planus typically presents as pruritic, polygonal, violaceous flat-topped papules and plaques; many variants in morphology and location also exist. Other lichenoid dermatoses share similar clinical presentations and histopathologic findings. These include lichenoid drug eruption, lichen planus-like keratosis, lichen striatus, lichen nitidus, and keratosis lichenoides chronica. Epidemiologic characteristics vary among each lichenoid disorder. While classic lichen planus is considered a disease of adults, other lichenoid dermatoses may be more common in younger populations. The literature contains an array of reports on the variations in presentation and successful management of lichen planus and lichenoid dermatoses among diverse populations. Familiarity with the characteristics of each lichenoid dermatosis, rare or common within each patient population, is key to accomplishing timely recognition and effective management.

  15. Autoimmune hypophysitis.

    Science.gov (United States)

    Ezzat, S; Josse, R G

    1997-03-01

    Autoimmune (lymphocytic) hypophysitis has emerged as a distinct and specific clinical and pathological disease entity. Although relatively rare compared with other autoimmune endocrine diseases, nearly a hundred cases have been described. The condition is much more common in females (9:1) and appears to have a particular predilection for the pregnant and postpartum states. The anterior pituitary, and less often the neurohypophysis, appear to be the target for inflammatory autoimmune destruction. During the evolution of the disease process, pituitary hyperfunction (usually hyperprolactinemia) has been noted. This disease should now be included in the differential diagnosis of pituitary disorders, especially in females presenting with pituitary enlargement, particularly if symptoms occur in temporal relationship to pregnancy. The disease may form part of the spectrum of the polyglandular autoimmune endocrine disorders. (Trends Endocrinol Metab 1997;8:74-80). (c) 1997, Elsevier Science Inc.

  16. Autoimmune hepatitis.

    Science.gov (United States)

    Heneghan, Michael A; Yeoman, Andrew D; Verma, Sumita; Smith, Alastair D; Longhi, Maria Serena

    2013-10-26

    Autoimmune hepatitis is a disease of the hepatic parenchyma that can present in acute or chronic forms. In common with many autoimmune diseases, autoimmune hepatitis is associated with non-organ-specific antibodies in the context of hepatic autoimmunity. This dichotomy has made definition of a unifying hypothesis in the pathophysiology of the disease difficult, although data from the past 8 years have drawn attention to the role of regulatory T cells. Several triggers have been identified, and the disease arises in genetically susceptible individuals. Clinical and biochemical remission is achievable in up to 85% of cases. For the remaining patients, alternative immunosuppression strategies are an option. Liver transplantation provides an excellent outcome for patients with acute liver failure or complications of end-stage liver disease, including hepatocellular carcinoma. Variant or overlapping syndromes are worthy of consideration when unexpected disease features arise.

  17. Autoimmune disorders

    Science.gov (United States)

    ... as azathioprine, cyclophosphamide, mycophenolate, sirolimus, or tacrolimus. Targeted drugs called tumor necrosis factor (TFN) blockers can be used for some diseases. Outlook (Prognosis) The outcome depends on the disease. Most autoimmune diseases are chronic , but many can be controlled ...

  18. Autoimmune Hepatitis

    Science.gov (United States)

    ... the digestive tract Graves' disease, the most common cause of hyperthyroidism in the United States Hashimoto's disease, also called chronic lymphocytic thyroiditis or autoimmune thyroiditis, a form of chronic inflammation ... which causes irritation, scarring, and narrowing of the bile ducts ...

  19. Autoimmune pancreatitis

    Directory of Open Access Journals (Sweden)

    Davorin Dajčman

    2007-05-01

    Full Text Available Background: Autoimmune pancreatitis is a recently described type of pancreatitis of presumed autoimmune etiology. Autoimmune pancreatitis is often misdiagnosed as pancreatic cancer difficult, since their clinical presentations are often similar. The concept of autoimmune pancreatitis was first published in 1961. Since then, autoimmune pancreatitis has often been treated not as an independent clinical entity but rather as a manifestation of systemic disease. The overall prevalence and incidence of the disease have yet to be determined, but three series have reported the prevalence as between 5 and 6 % of all patients with chronic pancreatitis. Patient vary widely in age, but most are older than 50 years. Patients with autoimmune pancreatitis usually complain of the painless jaundice, mild abdominal pain and weight loss. There is no laboratory hallmark of the disease, even if cholestatic profiles of liver dysfunction with only mild elevation of amylase and lipase levels have been reported.Conclusions: Proposed diagnostic criteria contains: (1 radiologic imaging, diffuse enlargement of the pancreas and diffusely irregular narrowing of the main pancreatic duct, (2 laboratory data, elevated levels of serum ã-globulin and/or IgG, specially IgG4, or the presence of autoantibodies and (3 histopathologic examination, fibrotic change with dense lymphoplasmacytic infiltration in the pancreas. For correct diagnosis of autoimmune pancreatitis, criterion 1 must be present with criterion 2 and/or 3. Autoimmune pancreatitis is frequently associated with rheumatoid arthritis, Sjogren’s syndrome, inflammatory bowel disease, tubulointersticial nephritis, primary sclerosing cholangitis and idiopathic retroperitoneal fibrosis. Pancreatic biopsy using an endoscopic ultrasound-guided fine needle aspiration biopsy is the most important diagnostic method today. Treatment with corticosteroids leads to the and resolution of pancreatic inflamation, obstruction and

  20. Intertriginous bullous morphea: A clue for the pathogenesis?

    Directory of Open Access Journals (Sweden)

    Kavala Mukaddes

    2007-01-01

    Full Text Available Bullae occurring in lesions of morphea are uncommon. The cause of bullae formation in morphea is multifactorial, although lymphatic obstruction from the sclerodermatous process is considered the likeliest cause. Bullous morphea may be confused clinically with lichen sclerosus et atrophicus since both diseases may cause bullae in sclerodermatous plaques. A 69-year-old woman presented with a history of generalized morphea diagnosed 9 years earlier; and a 1-month history of pruritic bullae on her inframammary folds, axillary regions, lower abdomen, upper extremities and inguinal folds. Physical examination revealed multiple erythematous erosions, hemorrhagic vesicles and eroded bullae with slight scale or crusts overlying hypopigmented, indurated, shiny plaques. Skin biopsy revealed prominent edema in the papillary dermis, resulting in bulla formation and thickening of collagen fibers within the dermis. Direct immunofluorescence was negative. According to histologic and clinical features, the diagnosis of bullous morphea was established.

  1. Autoimmune hepatitis.

    Science.gov (United States)

    Strassburg, Christian P

    2010-10-01

    Autoimmune hepatitis is a chronic inflammatory disease of the liver with a dismal prognosis when left untreated. Key for the improvement of prognosis is a timely diagnosis before cirrhosis has developed. This is reached by the exclusion of other causes of hepatitis, elevated immunoglobulin G, autoantibody profile and histological assessment. Treatment achieves remission rates in 80% of individuals and consists of immunosuppression with corticosteroids and azathioprine. A recent randomised controlled multicenter trial has added budesonide to the effective treatment options in non-cirrhotic patients and leads to a reduction of unwanted steroid side effects. Autoimmune hepatitis is an autoimmune disease of unknown aetiology. Association studies of major histocompatibility complex and other genes demonstrate an influence of immunogenetics. However, apart from the autoimmune polyglandular syndrome type 1, in which 10% of patients suffer from an autoantibody-positive autoimmune hepatitis linked to mutations of the autoimmune regulator gene, there is no clear evidence for a hereditary aetiology of this disease. Copyright © 2010 Elsevier Ltd. All rights reserved.

  2. Histopathological analysis of vesicular and bullous lesions in Kaposi sarcoma

    Directory of Open Access Journals (Sweden)

    Kandemir Nilüfer

    2012-08-01

    Full Text Available Abstract Background In this study, the clinical and morphological features of vesiculobullous lesions observed in Kaposi sarcoma are analyzed, and the features of bullous Kaposi sarcoma cases are emphasized. Methods A total of 178 biopsy materials of 75 cases diagnosed as classic-type cutaneous Kaposi sarcoma were reviewed. Twenty-five cases showing vesiculobullous features were included in the study. Tumor, epidermis, dermis, and clinical data regarding these cases was evaluated. Results Vesicular changes were observed in 21 (12% out of 178 lesions of the 75 cases, while bullous changes were present in only 4 (2%. In all cases where vesicular and bullous changes were detected, tumor, epidermis, and dermis changes were similar. All cases were nodular stage KS lesions, whereas hyperkeratosis and serum exudation in the epidermis, marked edema in the dermis, and enlarged lymphatic vessels and chronic inflammatory response were observed. Conclusions Our findings suggest that changes in vascular resistance occurring during tumor progression are the most important factors comprising vesiculobullous morphology. Virtual slides The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1646397188748474

  3. Annular bullous lesions with atypical erythema multiforme in leprosy.

    Science.gov (United States)

    Shah, Aishani; Mahajan, Rashmi; Ninama, Kishan; Bilimoria, Freny

    2014-09-01

    Erythema nodosum leprosum (ENL) is an immune complex-mediated reaction that may complicate the course of multibacillary leprosy. Bullous lesions in Type II reaction, though reported, are exceedingly rare. We report the case of a 32 year old female patient who presented initially at our OPD with erythema nodosum. Cutaneous examination revealed impaired sensation over dorsum of right foot and thickened right lateral popliteal nerve. Slit skin smear (SSS) from ear lobes revealed AFB with a bacteriological index of 2+. She was started on MDT, tablet ofloxacin 200 mg twice a day, and 30 mg oral prednisolone. Two months later, she presented with generalised pruritus, large target lesions over the back, and hemorrhagic bullae over lower extremities and annular pattern of bullae, over both arms. A SSS was repeated which was positive for AFB. Histopathology from bullous lesions was consistent with ENL. Direct Immunofluorescence (DIF) study was negative. Our patient improved rapidly after she was started on thalidomide 100 mg twice daily, with withdrawal of ofloxacin. Erythema Multiforme (EMF) and annular bullous lesions have been reported in patients on treatment with ofloxacin. This case is being presented due to the unusual and varied manifestation of Type II lepra reaction in a 34 year old female patient.

  4. [Bullous rash around a peritoneal dialysis catheter exit site].

    Science.gov (United States)

    Chasset, F; Pecquet, C; Cury, K; Sesé, L; Moguelet, P; Francès, C; Gharbi, C; Senet, P

    2015-01-01

    Cutaneous eruption around a peritoneal dialysis (PD) catheter exit site is a rare complication. Herein we report a case of bullous eruption; we discuss the diagnostic approach and the related therapeutic implications. A 63-year-old man presented a bullous periumbilical eruption two months after initiation of PD. Cultures of laboratory samples ruled out an infectious origin and systemic corticosteroids initiated at 0.5mg/kg for suspected eosinophilic peritonitis produced significant improvement of the cutaneous eruption. Recurrence of the bullous eruption was observed upon dose-reduction of the corticosteroid. Skin histology showed a clinical picture of eczema and direct immunofluorescence was negative. Patch testing was carried out using the European Standard Battery comprising antiseptic, cosmetic and plastic series; a semi-open test was performed with the dressing used for PD, and ROAT was carried out on the povidone iodine (Betadine™) 10% used for topical care during PD. The patch testing and ROAT were positive (++), confirming contact dermatitis due to Betadine™. The eruption totally disappeared on substitution of Betadine™ by chlorhexidine for topical antisepsis of the PD catheter, thus enabling PD to be continued rather than instituting hemodialysis. Allergic contact dermatitis around a PD catheter is a rare and little-known complication. In the present case, ROAT testing showed sensitization to Betadine™ and enabled an alternative antisepsis solution to be found, allowing PD to be continued. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  5. Bullous pemphigoid: role of complement and mechanisms for blister formation within the lamina lucida.

    Science.gov (United States)

    Iwata, Hiroaki; Kitajima, Yasuo

    2013-06-01

    Bullous pemphigoid (BP), an autoimmune subepidermal blistering skin disease, demonstrates tense blisters with or without widespread erythema, blistering along the lamina lucida, immunoglobulin G and/or complement deposits at the basement membrane zone, and the presence of circulating autoantibodies against hemidesmosomal molecules. These autoantibodies usually react against 180-kDa and/or 230-kDa proteins, designated as BP180 and BP230, respectively. The precise blistering mechanisms after autoantibodies bind to antigens are not fully understood. Immune complexes are thought to initially activate the complement cascade, which may induce activation of proteases and/or cytokines and cause dermal-epidermal separation. However, why does separation run specifically within the lamina lucida in a space as narrow as 500 nm wide? This review mainly focuses on the possible mechanisms of BP-specific blistering and how separation occurs along the lamina lucida, based on existing evidence. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  6. Risk of Death in Bullous Pemphigoid: A Retrospective Database Study in Finland.

    Science.gov (United States)

    Försti, Anna-Kaisa; Jokelainen, Jari; Timonen, Markku; Tasanen, Kaisa

    2016-08-23

    Bullous pemphigoid (BP) is an autoimmune skin disease of elderly people, which is associated with increased mortality. The aim of this study was to investigate the standardized mortality ratio (SMR) for BP in Finland, and concomitant comorbidities and medications. This was a retrospective database study of all cases of BP diagnosed at the Department of Dermatology, Oulu University Hospital, Finland, between 1985 and 2012. A total of 198 immunologically confirmed cases of BP were found. One-year mortality was 16.7%, and SMR 7.56 (95% confidence interval (CI) 4.98-10.14). The most common comorbidities were cardiovascular diseases (76.3%) and neurodegenerative diseases (40.9%). Malignancies (8.6%) were associated with increased mortality (hazard ratio = 2.4, 95% CI 1.1-5.5, p = 0.047). A novel finding was that polypharmacy was very common in patients with BP, and the higher the number of drugs, the greater the mortality. In conclusion, the mortality for BP in Finland is 7.6-fold that of a reference population, and malignancies and polypharmacy are associated with increased mortality.

  7. Quality of life, depression, anxiety and loneliness in patients with bullous pemphigoid. A case control study*

    Science.gov (United States)

    Kouris, Anargyros; Platsidaki, Eftychia; Christodoulou, Christos; Armyra, Kalliopi; Korkoliakou, Panagiota; Stefanaki, Christina; Tsatovidou, Revekka; Rigopoulos, Dimitrios; Kontochristopoulos, George

    2016-01-01

    Background Bullous pemphigoid (BP) is a chronic, autoimmune blistering skin disease that affects patients' daily life and psychosocial well-being. Objective The aim of the study was to evaluate the quality of life, anxiety, depression and loneliness in BP patients. Methods Fifty-seven BP patients and fifty-seven healthy controls were recruited for the study. The quality of life of each patient was assessed using the Dermatology Life Quality Index (DLQI) scale. Moreover, they were evaluated for anxiety and depression according to the Hospital Anxiety Depression Scale (HADS-scale), while loneliness was measured through the Loneliness Scale-Version 3 (UCLA) scale. Results The mean DLQI score was 9.45±3.34. Statistically significant differences on the HADS total scale and in HADS-depression subscale (p=0.015 and p=0.002, respectively) were documented. No statistically significant difference was found between the two groups on the HADS-anxiety subscale. Furthermore, significantly higher scores were recorded on the UCLA Scale compared with healthy volunteers (p=0.003). Conclusion BP had a significant impact on quality of life and the psychological status of patients, probably due to the appearance of unattractive lesions on the skin, functional problems and disease chronicity. PMID:27828632

  8. Refractory linear IgA bullous dermatosis successfully treated with mycophenolate sodium.

    Science.gov (United States)

    Marzano, Angelo V; Ramoni, Stefano; Spinelli, Diana; Alessi, Elvio; Berti, Emilio

    2008-01-01

    Linear IgA bullous dermatosis (LABD) is a rare, blistering autoimmune disease characterized by linear deposits of IgA at the basement membrane zone (BMZ), with the possible presence of circulating IgA anti-BMZ antibodies. LABD of childhood is usually self-healing, while in adults it follows a more prolonged course and refractory cases may rarely occur. The first-line treatment for LABD is dapsone in monotherapy or in combination with systemic corticosteroids, but various therapeutic approaches have been used in non-responder patients. We report two adult patients with refractory LABD successfully treated with enteric-coated mycophenolate sodium (EC-MPS), a recently introduced formulation of mycophenolic acid (MPA). MPA is an immunosuppressive agent that acts by inhibiting monophosphate dehydrogenase, a key enzyme in the novo synthesis of purines. Based on the present cases, we indicate EC-MPS as being a safe and effective adjuvant therapy in the treatment of LABD when dapsone or the other steroid-sparing drugs fail. It seems to offer an improved gastric side effect profile in comparison with the classic formulation of MPA, namely its ester mycophenolate mofetil (MMF).

  9. Role of methotrexate in the treatment of bullous pemphigoid in the elderly.

    Science.gov (United States)

    Patton, Timothy; Korman, Neil

    2008-01-01

    Bullous pemphigoid (BP) is an autoimmune blistering disease that commonly occurs in the elderly. Immunosuppressive medications are effective at controlling the disease in the majority of cases. Mortality can occur as a consequence of severe disease or as a result of the therapies that are frequently employed as treatment. Commonly employed therapies include systemic corticosteroids, azathioprine and mycophenolate mofetil. In a small subset of patients, these first- and second-line therapies do not control disease or are not tolerated by the patients. Optional therapies include nicotinamide (niacinamide), tetracycline, intravenous immunoglobulin, cyclophosphamide, dapsone and methotrexate. The majority of BP patients are elderly, and several considerations need to be taken into account before a specific therapy is chosen. Methotrexate provides several advantages in the elderly population in terms of practicality, cost and tolerability. Several retrospective and prospective studies have evaluated its effectiveness in the treatment of BP in the elderly population. The results of these studies indicate that methotrexate is an effective therapy for BP and is a reasonable option for treatment in the elderly population, although maybe not as a first- or second-line therapy.

  10. Bullous Pemphigoid With a Dual Pattern of Glomerular Immune Complex Disease

    NARCIS (Netherlands)

    Hoorn, Ewout J.; Taams, Noor E.; Hurskainen, Tiina; Salih, Mahdi; Weening, Jan J.; Jonkman, Marcel F.; Pas, Hendri H.; Schreurs, Marco W. J.

    2016-01-01

    A 75-year-old man presented with a blistering skin disease and nephrotic syndrome. Bullous pemphigoid was diagnosed by linear immunoglobulin G (IgG) and C3 staining along the basement membrane zone of a skin biopsy specimen and by the presence of circulating IgG recognizing the 180-kDa bullous pemph

  11. Bullous lesions as a manifestation of systemic lupus erythematosus in two Mexican teenagers

    Directory of Open Access Journals (Sweden)

    López-Corella Eduardo

    2010-07-01

    Full Text Available Abstract Rarely, systemic lupus erythematosus (SLE presents with bullous lesions due to severe edema and hydropic degeneration of the basal layer, or as a subepidermal blistering disease. Here, we describe two Mexican teenagers, one with SLE with blisters and another with bullous SLE. We also discuss the mechanisms and clinical implications of lesion formation in patients with SLE and bullae.

  12. Bullous Pemphigoid With a Dual Pattern of Glomerular Immune Complex Disease

    NARCIS (Netherlands)

    Hoorn, Ewout J.; Taams, Noor E.; Hurskainen, Tiina; Salih, Mahdi; Weening, Jan J.; Jonkman, Marcel F.; Pas, Hendri H.; Schreurs, Marco W. J.

    A 75-year-old man presented with a blistering skin disease and nephrotic syndrome. Bullous pemphigoid was diagnosed by linear immunoglobulin G (IgG) and C3 staining along the basement membrane zone of a skin biopsy specimen and by the presence of circulating IgG recognizing the 180-kDa bullous

  13. IgA autoantibodies in the pemphigoids and linear IgA bullous dermatosis

    NARCIS (Netherlands)

    Horvath, Barbara; Niedermeier, Andrea; Podstawa, Eva; Mueller, Ralf; Hunzelmann, Nicolas; Karpati, Sarolta; Hertl, Michael

    2010-01-01

    Background: Patients with bullous pemphigoid (BP), mucous membrane pemphigoid (MMP) and pemphigoid gestationis (PG) have IgG antibodies against BP180 and BP230, components of the hemidesmosomes. Patients with linear IgA bullous dermatosis (LABD) have IgA autoantibodies against a 97/120-kDa protein w

  14. Autoimmun hypophysitis

    DEFF Research Database (Denmark)

    Krarup, Therese; Hagen, Claus

    2010-01-01

    Autoimmune hypophysitis (AH) - often referred to as lymphocytic hypophysitis - is a rare disease that affects the pituitary gland and causes inflammation. The disease enlarges the pituitary gland and the clinical presentations are lack of pituitary function and headaches. AH is mostly seen in women...... during pregnancy or postpartum, but also occurs in males and children. AH is often associated with other autoimmune diseases, most frequently with Hashimoto's thyroiditis. The symptoms are caused by enlargement of the pituitary gland and disturbances of the hormone function. Treatment is either...

  15. Autoimmune disease

    Institute of Scientific and Technical Information of China (English)

    2005-01-01

    2005164 Optimal cut-point of glutamic acid decar-boxylase antibody (GAD-Ab) for differentiating two subtypes of latent autoimmune diabetes in adults (LADA). LI Xia(李霞), et al. Dept Endocrinol, 2nd Xiangya Hosp, Central South Univ, Changsha, 410011. Chin J Diabetes, 2005;13(1) :34-38. Objective: To investigate the optimal cut-point of glutamate decarboxylase antibody (GAD-Ab) for differentiating two subtypes of latent autoimmune diabetes in adults (I. ADA). Methods: The frequency

  16. Ribosomal protein s6-ps240 is expressed in lesional skin from patients with autoimmune skin blistering diseases

    Directory of Open Access Journals (Sweden)

    Ana Maria Abreu Velez

    2013-01-01

    Full Text Available Background: The in situ signaling transduction within skin biopsies from patients affected by autoimmune skin blistering diseases is not well-characterized. Aim : In autoimmune skin blistering diseases, autoantibodies seem to trigger several intracellular signaling pathways and we investigated the presence of the phosphorylated form of ribosomal protein S6-pS240 within autoimmune skin blistering diseases biopsies. Materials and Methods: We utilized immunohistochemistry to evaluate the presence of S6-pS240 in lesional skin biopsies of patients affected by autoimmune skin blistering diseases including patients with an endemic and nonendemic pemphigus foliaceus (non EPF, with bullous pemphigoid (BP, pemphigus vulgaris (PV, dermatitis herpetiformis (DH, and the respective controls. Results: Most autoimmune bullous skin diseases biopsies stained positive for S6-pS240 around lesional blisters, including adjacent areas of the epidermis; and within upper dermal inflammatory infiltrates, and/or mesenchymal-endothelial cell junctions within the dermis. Conclusions: We document that S6-pS240 is expressed in lesional areas of skin biopsies from patients with autoimmune skin blistering diseases, as well as on eccrine glands and piloerector muscles. Thus, the role of this molecule in autoimmune skin blistering diseases warrants further study.

  17. Stress coping mechanisms in patients with chronic dermatoses

    Directory of Open Access Journals (Sweden)

    Korabel, Hanna

    2013-09-01

    Full Text Available Objective. The results of numerous studies of today confirm that persons suffering from psychosomatic disorders are not able to effectively cope with stress. The experience of stress is also frequently combined with the occurrence or aggravation of various skin diseases. The goal of our study was to identify the predominantways of coping with stress in the group of patients with chronic dermatoses.Methods. The group under study included patients receiving treatment in the Dermatology Clinic of Collegium Medicum, Jagiellonian University. They were either hospitalized patients or those who came for control examinations at the Outpatient Clinic. Evaluation of the forms of coping with stress was conducted with the help of the Endler and Parker Questionnaire – CISS.Results. They significantly more often apply the style of coping focused on avoiding (p-value= 0.0056. It also turned out that the patients in the dermatological groups manifested a constant tendency to get involved in vicarious activities (p-value=0.0247.Discussion. The results of the presented study indicate that there is a statistically significant difference between the patients with dermatological disorders and those in the control group as regards their ways of coping with stress.Conclusion. The results obtained in the discussed study may be a starting point for designing a complex support for the patients with skin diseases. The therapeutic technique that may prove helpful for this group of patients is the cognitive-behavioral therapy (CTB.

  18. Autoimmune sialadenitis

    NARCIS (Netherlands)

    Guntinas-Lichius, O.; Vissink, A.; Ihrler, S.

    2010-01-01

    Using the European-American classification criteria the diagnosis of autoimmune sialadenitis in Sjogren's syndrome can generally be easily established or excluded. In addition, sonography performed by the ENT physician is helpful in diagnosing and especially in follow-up screening for MALT lymphomas

  19. Case Report of Bullous Pemphigoid following Fundus Fluorescein Angiography

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    Goktug Demirci

    2010-05-01

    Full Text Available Purpose: To report a first case of bullous pemphigoid (BP following intravenous fluorescein for fundus angiography. Clinical Features: A 70-year-old male patient was admitted to the intensive care unit with BP and sepsis. He reported a history of fundus fluorescein angiography with a pre-diagnosis of senile macular degeneration 2 months prior to presentation. At that time, fluorescein extravasated at the antecubital region. Following the procedure, pruritus and erythema began at the wrists bilaterally, and quickly spread to the entire body. The patient also reported a history of allergy to human albumin solution (Plamasteril®; Abbott 15 years before, during bypass surgery. On dermatologic examination, erythematous patches were present on the scalp, chest and anogenital region. Vesicles and bullous lesions were present on upper and lower extremities. On day 2 of hospitalization, tense bullae appeared on the upper and lower extremities. The patient was treated with oral methylprednisolone 48 mg (Prednol®; Mustafa Nevzat, topical clobetasol dipropionate 0.05% cream (Dermovate®; Glaxo SmithKline, and topical 4% urea lotion (Excipial Lipo®; Orva for presumptive bullous pemphigoid. Skin punch biopsy provided tissue for histopathology, direct immunofluorescence examination, and salt extraction, which were all consistent with BP. After 1 month, the patient was transferred to the intensive care unit with sepsis secondary to urinary tract infection; he died 2 weeks later from sepsis and cardiac failure. Conclusions: To our knowledge, this is the first reported case of BP following fundus fluorescein angiography in a patient with known human albumin solution allergy. Consideration should be made to avoid fluorescein angiography, change administration route, or premedicate with antihistamines in patients with known human albumin solution allergy. The association between fundus fluorescein angiography and BP should be further investigated.

  20. Pseudo-Kaposi sarcoma (acroangiodermatitis): occurring after bullous erysipelas.

    Science.gov (United States)

    Kutlubay, Zekayi; Yardimci, Gürkan; Engin, Burhan; Demirkesen, Cuyan; Aydin, Övgü; Khatib, Rashid; Tuzun, Yalçın

    2015-05-18

    Pseudo-Kaposi sarcoma is a benign reactive vascular proliferative disorder, which can be seen at any age. It occurs when the chronic venous pressure changes result in vascular proliferation in the upper and mid dermis. This disease is divided into two subtypes: the most frequent subtype is the Mali type and seen in early ages. The Mali type is seen in chronic venous insufficiency and in those patients with arteriovenous shunts. The rare subtype is the Stewart-Bluefarb type. This disease must be distinguished from Kaposi sarcoma because of their clinical resemblance. Herein, we present a patient with pseudo-Kaposi sarcoma, which developed after bullous erysipelas.

  1. Bullous lesions, sweat gland necrosis and rhabdomyolysis in alcoholic coma

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    Neelakandhan Asokan

    2014-01-01

    Full Text Available A 42-year-old male developed hemorrhagic bullae and erosions while in alcohol induced coma. The lesions were limited to areas of the body in prolonged contact with the ground in the comatose state. He developed rhabdomyolysis, progressing to acute renal failure (ARF. Histopathological examination of the skin showed spongiosis, intraepidermal vesicles, and necrosis of eccrine sweat glands with denudation of secretory epithelial lining cells. With supportive treatment and hemodialysis, the patient recovered in 3 weeks time. This is the first reported case of bullous lesions and sweat gland necrosis occurring in alcohol-induced coma complicated by rhabdomyolysis and ARF.

  2. Updates on the management of autoimmune blistering diseases.

    Science.gov (United States)

    Hooten, Joanna N; Hall, Russell P; Cardones, Adela R

    2014-01-01

    Autoimmune blistering diseases are rare, but potentially debilitating cutaneous disorders characterized by varying degrees of mucosal and cutaneous bullae formation. Topical therapy is appropriate for mild and even some moderate disease activity, but systemic treatment can be considered for more extensive involvement. Corticosteroids remain the first-line systemic therapy for patients with moderate to severe bullous pemphigoid and pemphigus vulgaris. While the use of systemic steroids has dramatically reduced mortality from these two autoimmune blistering disorders, treatment is also associated with multiple side effects, especially when used long-term. Steroid sparing agents, therefore, are invaluable in inducing long-term remission while minimizing steroid associated side effects. Treatment must be tailored to the individual patient's condition, and several other factors must be carefully considered in choosing appropriate therapy: 1) diagnosis, 2) severity of the condition and body site affected, 3) presence of comorbidities, and 4) ability to tolerate systemic therapy.

  3. [Autoimmune channelopathies].

    Science.gov (United States)

    Michaud, M; Delrieu, J; Astudillo, L

    2011-12-01

    Autoimmune channelopathies are rare neuromuscular diseases that have been characterized clinically for several decades but for which the evidence of associated antibodies has only been recently demonstrated. Ion channels have an important role of activation, inhibition and regulation in neuromuscular transmission. Myasthenia gravis, generally associated with the presence of anti-acetylcholine receptor antibody, is the best-known channelopathy. Other anti-channel antibodies, including voltage-dependent, are associated with several neurological diseases, as illustrated by anti-voltage-gated calcium channels found in Lambert-Eaton myasthenic syndrome and paraneoplastic cerebellar ataxia, and anti-voltage-gated potassium channels found in neuromyotonia, Morvan's syndrome and limbic encephalitis. The treatment of autoimmune channelopathies is logically based on corticosteroids, immunosuppressant drugs, intravenous immunoglobulins and plasmapheresis. Copyright © 2011 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  4. A case of crusted scabies with a bullous pemphigoid-like eruption and nail involvement.

    Science.gov (United States)

    Nakamura, Eri; Taniguchi, Hiroko; Ohtaki, Noriko

    2006-03-01

    We report a case of a 71-year-old man infected at a nursing home who developed a bullous pemphigoid-like eruption with nail involvement. He was diagnosed by his family doctor as suffering from eczema and was treated with topical corticosteroids, then blisters started appearing. He was next diagnosed as suffering from bullous pemphigoid and treated with oral prednisolone, which worsened his condition. He was finally diagnosed as having crusted scabies with bullous pemphigoid-like eruptions and nail involvement at our clinic. He was then prescribed oral ivermectin (two doses of 12 mg ivermectin with a 1-week interval) and topical lindane (1%gamma-BHC in petrolatum) for scabies with 5% salicylic acid in plastibase as an additional treatment for the crusted lesions on his soles. He showed remarkable improvement in 2 weeks, and his nails showed complete recovery after 7 weeks of occlusive dressing treatment with 1%gamma-BHC. One and a half years later, the patient showed no sign of a recurrence of scabies. The histology of a blister taken from this patient was similar to that of bullous pemphigoid. Direct immunofluorescence showed immunoglobulin (Ig)G and C3 deposition at the dermoepidermal junction similar to that of bullous pemphigoid, but indirect immunofluorescence was negative. The bullous symptoms of this patient were considered to be due to the scabies, because the patient recovered completely after receiving treatment for scabies. Indirect immunofluorescent study is important to distinguish between scabies with blister formation and true bullous pemphigoid.

  5. Dermatoses em pacientes com AIDS: estudo de 55 casos. Uberlândia, MG, Brasil

    Directory of Open Access Journals (Sweden)

    GOMIDES MABEL DUARTE ALVES

    2002-01-01

    Full Text Available Foram estudados 55 pacientes com a síndrome da imunodeficiência adquirida (Aids, triados ao serviço de Dermatologia, apresentando doenças cutâneo-mucosas, algumas de caráter oportunista. As dermatoses são freqüentes nestes doentes e, às vezes, de difícil diagnóstico pelo caráter atípico das lesões. OBJETIVOS: Analisar a freqüência e apresentação clínica das dermatoses relacionadas à Aids. MÉTODOS: Cinqüenta e cinco pacientes com Aids e lesões tegumentares foram estudados, de modo transversal, no Serviço de Dermatologia da Universidade Federal de Uberlândia, de 1995 a 1997. Foram realizadas biopsias e culturas diversas para elucidação diagnóstica. RESULTADOS: Foram encontradas 116 dermatoses, com predomínio das fúngicas (78%, seguidas pelas virais (40%, eritêmato-escamosas (27%, pápulo-pruríticas (18%, causadas por drogas (10%, neoplásicas (9% e outras afecções cutâneo-mucosas (7%. A maioria dos pacientes apresentou mais de uma dermatose (67%. CONCLUSÕES: Confirmou-se a maior freqüência de dermatoses fúngicas, seguidas pelas virais, na Aids. Observou-se a necessidade de propedêutica bem elaborada para o diagnóstico preciso das dermatoses, devido à sua apresentação atípica em grande número de doentes. O exame dermatológico e a biopsia das lesões tiveram grande relevância na suspeita do diagnóstico de Aids.

  6. A clinical study of dermatoses in diabetes to establish its markers

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    Dependra Kumar Timshina

    2012-01-01

    Full Text Available Background: Cutaneous manifestations of diabetes mellitus generally appear subsequent to the development of the disease, but they may be the first presenting signs and in some cases they may precede the primary disease manifestation by many years. Aims : T0 he aim of our study was to study the spectrum of dermatoses in diabetics, to know the frequency of dermatoses specific to diabetes mellitus (DM, and to establish the mucocutaneous markers of DM. Material and Methods: The study was conducted at a diabetic clinic and our department between September 2008 and June 2010. Two hundred and twenty-four diabetic patients were included in the study group and those with gestational diabetes were excluded. Healthy age- and sex-matched individuals were taken as controls. Results: The male to female ratio was 1 : 1.21. Type 2 DM was seen in 89.7% and type 1 DM in 10.3% of the patients. Dermatoses were seen in 88.3% of the diabetics compared to 36% in non-diabetic controls (P<0.05. Cutaneous infections were the most common dermatoses followed by acanthosis nigricans and xerosis in diabetics. Type 2 DM was found to have an increased risk of complications than type 1 DM. Complications of diabetes were seen in 43.7% of the diabetic cases. Diabetic dermopathy, loss of hair over the legs, diabetic foot ulcer, and so on, were found to be the cutaneous markers of DM in our group of cases. Conclusion: Dermatoses were more common in diabetics than non-diabetics. Cutaneous infections formed the largest group of dermatoses in DM.

  7. Dermatoses in the first 72 h of life: A clinical and statistical survey

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    Vanessa Zagne

    2011-01-01

    Full Text Available Background: The neonatal period is generally regarded as the first 28 days of extrauterine life. Skin disorders are commonly seen in the neonatal period, most of which are transient and limited to the first days or weeks of life. In spite of being so frequent, these transient conditions usually escape dermatologist′s observations, and consequently few have been studied. Aims: The study was designed to identify the dermatoses in the first 72 h of life; to report the relationship among the most common dermatoses with the newborn′s features; and to verify how often dermatoses are reported by the neonatologist. Methods: The authors carried out a cross-sectional study on newborn′s dermatoses in a brazilian public hospital, including 203 healthy neonates, until 72 h of age, with skin disorders. Results: Out of 34 different skin diagnosed conditions, hypertrichosis lanuginosa, Mongolian spot, sebaceous hyperplasia, epidermal hyperpigmentation, erythema toxicum, and salmon patch were the most frequent ones. The dermatoses with statistical significance were: Mongolian spot and epidermal hyperpigmentation with the non-white newborns; erythema toxicum and cutis marmorata with the white newborns; salmon patch with the female sex; physiologic desquamation with the cesarean section; acrocyanosis with the first pregnancy birth and Bohn′s nodules with the vaginal delivery. Conclusions: Thirty-four different types of dermatological alterations were identified in the healthy newborn within 3 days of life at the Maternity School of the Federal University of Rio de Janeiro. Underreporting of dermatoses with serious medical significance shows the importance of a dermatologist in the neonatal unit of a hospital.

  8. Autoimmun pankreatitis

    DEFF Research Database (Denmark)

    Fjordside, Eva; Novovic, Srdan; Schmidt, Palle Nordblad;

    2015-01-01

    Autoimmune pancreatitis (AIP) is a rare inflammatory disease. AIP has characteristic histology, serology and imaging findings. Two types of AIP exist, type 1, which is a part of the systemic immunoglobulin G4-related disease, and type 2, which is only localized to the pancreas. Patients with type 1...... are predominantly older men, have involvement of other organs and more often experience relapse than patients with type 2. Both types respond well to steroid treatment. The most important differential diagnose is pancreatic cancer....

  9. PSEUDOSINDACTILIA EN EPIDERMÓLISIS BULLOSA Pseudosyndactylia in bullous epidermolysis

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    Enríque Vergara Amador

    2009-01-01

    Full Text Available La epidermólisis bullosa comprende un grupo de patologías que se caracterizan por la fragilidad de la piel, formación de ampollas, y en las formas distróficas, la formación de pseudosindactilias. Debido a lo exótico de la entidad y la dificultad para el enfoque de tratamiento, se presenta un caso clínico con desarrollo de pseudosindactilias, característico usualmente de las formas distróficas. Se describe en aspectos clínicos de la entidad, el tratamiento quirúrgico de las pseudosindactilias y de las contracturas en las manos, y se discuten aspectos de la enfermedad y de los tipos de tratamientoBullous epidermolysis comprises a group of pathologies characterized by skin fragility, formation of bullae and, in its dystrophic forms, by the formation of pseudosyndactylias. Due to the exotic nature of the disease and the difficulty in focusing its treatment, a case is presented in which pseudosyndactylias developed, a usual characteristic of the dystrophic forms. This article addresses clinical aspects of bullous epidermolysis, surgical treatment of pseudosyndactilia and hand contractures and other aspects of the disease and its treatment

  10. Accidental bullous phototoxic reactions to bergamot aromatherapy oil.

    Science.gov (United States)

    Kaddu, S; Kerl, H; Wolf, P

    2001-09-01

    Oil of bergamot is an extract from the rind of bergamot orange (Citrus aurantium ssp bergamia) that has a pleasant, refreshing scent; until a few years ago it had been widely used as an ingredient in cosmetics but was restricted or banned in most countries because of certain adverse effects. More recently, oil of bergamot preparations have been gaining renewed popularity in aromatherapy. Oil of bergamot possesses photosensitive and melanogenic properties because of the presence of furocoumarins, primarily bergapten (5-methoxypsoralen [5-MOP]). However, 5-MOP is also potentially phototoxic and photomutagenic. Despite its increasing application, there are only a few recent reports of phototoxic reactions to bergamot aromatherapy oil. We describe two patients with localized and disseminated bullous phototoxic skin reactions developing within 48 to 72 hours after exposure to bergamot aromatherapy oil and subsequent ultraviolet exposure. One patient (case 2) had no history of direct contact with aromatherapy oil but developed bullous skin lesions after exposure to aerosolized (evaporated) aromatherapy oil in a sauna and subsequent UVA radiation in a tanning salon. This report highlights the potential health hazard related to the increasing use of psoralen-containing aromatherapy oils.

  11. Nursing diagnoses in patients with immune-bullous dermatosis

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    Euzeli da Silva Brandão

    Full Text Available ABSTRACT Objective: identify nursing diagnoses in patients with immune-bullous dermatosis. Method: a quantitative and descriptive research, carried out in three institutions located in Rio de Janeiro and Mato Grosso do Sul, Brazil, using the Client Assessment Protocol in Dermatology during a nursing consultation. Simple descriptive statistics was used for data analysis. Results: 14 subjects participated in the study, nine with a diagnosis of pemphigus vulgaris, pemphigus two and three of bullous pemphigoid. The age ranged between 27 and 82 years, predominantly females (11. 14 nursing diagnoses were discussed and identified from a clinical rationale in all study participants, representing the most common human responses in this sample. The application of the Assessment Protocol in Dermatology facilitated the comprehensive assessment, in addition to providing the identification of diagnostics according to the North American Nursing Diagnosis Association International. Conclusion: the nursing diagnoses presented confirm the necessity of interdisciplinary work during the care for this clientele. For better description of the phenomena related to the client in question, it is suggested the inclusion of two risk factors related in three diagnoses of this taxonomy. It is worth noting the contribution of the findings for the care, education and research in nursing in dermatology.

  12. Nursing diagnoses in patients with immune-bullous dermatosis 1

    Science.gov (United States)

    Brandão, Euzeli da Silva; dos Santos, Iraci; Lanzillotti, Regina Serrão; Ferreira, Adriano Menis; Gamba, Mônica Antar; Azulay-Abulafia, Luna

    2016-01-01

    ABSTRACT Objective: identify nursing diagnoses in patients with immune-bullous dermatosis. Method: a quantitative and descriptive research, carried out in three institutions located in Rio de Janeiro and Mato Grosso do Sul, Brazil, using the Client Assessment Protocol in Dermatology during a nursing consultation. Simple descriptive statistics was used for data analysis. Results: 14 subjects participated in the study, nine with a diagnosis of pemphigus vulgaris, pemphigus two and three of bullous pemphigoid. The age ranged between 27 and 82 years, predominantly females (11). 14 nursing diagnoses were discussed and identified from a clinical rationale in all study participants, representing the most common human responses in this sample. The application of the Assessment Protocol in Dermatology facilitated the comprehensive assessment, in addition to providing the identification of diagnostics according to the North American Nursing Diagnosis Association International. Conclusion: the nursing diagnoses presented confirm the necessity of interdisciplinary work during the care for this clientele. For better description of the phenomena related to the client in question, it is suggested the inclusion of two risk factors related in three diagnoses of this taxonomy. It is worth noting the contribution of the findings for the care, education and research in nursing in dermatology. PMID:27533274

  13. UNUSUAL CLINICOPATHOLOGICAL AND IMMUNOLOGICAL PRESENTATION OF CHRONIC BULLOUS DERMATOSIS OF CHILDHOOD (LINEAR IGA DERMATOSIS)

    OpenAIRE

    Al-Saif Fahad; Al-Rikabi Ammar

    2011-01-01

    Linear IgA bullous dermatosis is a rare sulfone-responsive subepidermal blistering disorder of unknown etiology in which smooth linear deposits of IgA are found in the basement membrane zone. Chronic bullous dermatosis of childhood is equivalent to linear IgA disease of adulthood and is characterized by an abrupt onset of large, widespread and tense bullae on a normal or erythematous base. In this case, we describe an unusual presentation of chronic bullous dermatosis in a 14-month-old Saudi ...

  14. Bullous disorders as a manifestation of immune reconstitution inflammatory syndrome: A series of three cases

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    Rama Das

    2013-01-01

    Full Text Available Bullous disorders such as pemphigus vulgaris, bullous pemphigoid after the initiation of highly active antiretroviral therapy in certain human immunodeficiency virus reactive individuals have been described in this case series as a manifestation of an immune reconstitution inflammatory syndrome. This phenomenon should be suspected in individuals who present with bullous lesions within 3-8 weeks after initiation of therapy despite of improved immunological response. Strong clinical suspicion, through clinical examination, appropriate laboratory investigation such as CD4 T-cell count, histopathological examinations with H and E stain, direct immunofluorescence test are required for diagnosis.

  15. Eosinophil cationic protein levels parallel coagulation activation in the blister fluid of patients with bullous pemphigoid.

    Science.gov (United States)

    Tedeschi, A; Marzano, A V; Lorini, M; Balice, Y; Cugno, M

    2015-04-01

    Bullous pemphigoid (BP) is an autoimmune blistering disease due to autoantibodies against two hemidesmosomal antigens, namely BP180 and BP230, and characterized by coagulation activation both at cutaneous and systemic levels. Skin-infiltrating eosinophils contribute to bulla formation and, upon activation, are supposed to initiate the coagulation cascade. The aim of this study was to investigate whether the activation of eosinophils and coagulation are linked in BP. We evaluated the correlation between eosinophil cationic protein (ECP) levels and concentrations of the prothrombotic markers F1 + 2 and D-dimer in blister fluid and blood samples of 30 BP patients. Thirty healthy subjects were used as normal controls. ECP, F1 + 2 and D-dimer plasma levels were significantly higher in BP patients than in normal subjects. A significant correlation was found between ECP plasma levels and blood eosinophil count (r = 0.54, P = 0.002). F1 + 2 plasma levels positively correlated with disease severity, expressed as the percentage of body surface area involved (r = 0.36, P = 0.048). A striking increase in ECP (288.8 ± 45.2 ng/mL), F1 + 2 (31 409.9 ± 2929.4 pmol/L) and D-dimer levels (342 798.3 ± 44 206 ng/mL) was found in blister fluid from BP patients. In blister fluid, ECP levels were significantly higher than in peripheral blood (P blister fluids from BP patients and correlate with markers of coagulation activation, supporting the view that eosinophils initiate the coagulation cascade at skin level. © 2014 European Academy of Dermatology and Venereology.

  16. Three-year observation of the patients with bullous pemphigoid: 29 cases

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    Özlem Ekiz

    2013-12-01

    Full Text Available Background and Design: Bullous pemphigoid (BP is a chronic, autoimmune, subepidermal blistering disease. The aim of this study was to determine the clinico-epidemiologic data’s of patients diagnosed as BP in our clinic and compare these data with other studies in our country and in the world. Material and Method: We researched the documents of BP cases diagnosed by clinical and histopathological examinations from January 2009 to January 2013, retrospectively. We evaluated clinical and dermographical characteristics of all patients such as age, sex, existence of oral mucosal lesions, association of pruritus, duration of disease, presence of triggering factors, features of skin lesions, results of direct immunofluorescence (DIF investigation, drug intake, recurrence and mortality rates and causes of detected mortality. Results: Of the 29 patients included in the study %48,3 were female, %51,7 were male. The average age was 70,17 and the mean duration of disease was 15.41 months. In five cases oral mucosal involvement and in 24 cases pruritus was detected. Drugs in three cases and malignity in one case were assessed as triggering factors. The results of DIF investigation revealed the linear IgG and C3 deposition at the dermoepidermal junction in 12 cases, C3 deposition in 10 cases. The initial treatment was topical corticosteroid in 11, tetracycline and nicotinamide combination in six, systemic corticosteroid in 10 individuals. After initial therapy recurrence appeared in 7 patients in one year. Mortality during hospitalization was observed in 2 cases. Conclusions: We presented the clinical and epidemiological features of patients diagnosed as BP in our clinics in the last three years. Because of having different ethnical originated, heterogeneous population of our city, we believe that our investigation will contribute to having opinion about epidemiology of BP in our country. Nevertheless, prospective, long term and multicentre studies based on

  17. Autoimmune liver disease panel

    Science.gov (United States)

    Liver disease test panel - autoimmune ... Autoimmune disorders are a possible cause of liver disease. The most common of these diseases are autoimmune hepatitis and primary biliary cholangitis (formerly called primary biliary cirrhosis). This group of tests ...

  18. Penfigóide bolhoso no lactente Bullous pemphigoid in childhood

    Directory of Open Access Journals (Sweden)

    Ada Lobato Quattrino

    2007-02-01

    Full Text Available O penfigóide bolhoso é doença que comumente acomete pessoas idosas. Desde o primeiro caso na infância, confirmado por imunofluorescência em 1970, cerca de 50 casos foram relatados e poucos ocorreram em crianças com menos de um ano de idade. Apresentase caso de penfigóide bolhoso em lactente de quatro meses de idade e discutem-se as características clínicas e imunopatológicas dessa rara bulose da infância.Bullous pemphigoid typically affects the elderly. Since the first description of bullous pemphigoid in childhood in 1970, about 50 cases have been reported, but only 10 were reported in children under the first year of age. We report a case of bullous pemphigoid in a 4-month-old girl and discuss the clinical and immunopathological features of this rare bullous disease of infancy.

  19. Bullous pemphigoid. Occurrence in a patient with mycosis fungoides receiving PUVA and topical nitrogen mustard therapy

    Energy Technology Data Exchange (ETDEWEB)

    Patterson, J.W.; Ali, M.; Murray, J.C.; Hazra, T.A.

    1985-04-01

    A 57-year-old woman with mycosis fungoides developed blisters within cutaneous plaques while receiving PUVA therapy and topical nitrogen mustard. Direct and indirect immunofluorescence studies showed the findings of bullous pemphigoid. Her bullous disease was controlled after cessation of these therapies and institution of prednisone and methotrexate. During the 5 months following completion of a course of electron-beam therapy, she has been free of the cutaneous manifestations of both diseases. Previous instances of PUVA-related pemphigoid have occurred in psoriatics. The role of ultraviolet light in the induction of pemphigoid is discussed, particularly with regard to its possible interaction with the altered skin of psoriasis or mycosis fungoides. Some of the rare cases of bullous mycosis fungoides might actually have represented ultraviolet-unmasked bullous pemphigoid.

  20. Freqüência de dermatoses infecciosas em 208 pacientes transplantados renais

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    Vettorato Gérson

    2003-01-01

    Full Text Available FUNDAMENTOS: Nos pacientes transplantados renais, a imunossupressão crônica acarreta maior suscetibilidade às dermatoses infecciosas. OBJETIVOS: avaliar a freqüência de dermatoses infecciosas em 208 pacientes transplantados renais no período de 12 meses e verificar a relação entre sua ocorrência e o período de tempo transcorrido desde o transplante. MÉTODO: 208 transplantados renais de uma população de 720 pacientes foram submetidos a exame dermatológico no período de um ano, tendo sido realizados exames anatomopatológico micológico, bacteriológico e/ou cultural das lesões suspeitas. RESULTADOS: a freqüência de dermatoses infecciosas nessa população foi de 89,4%. As infecções fúngicas, virais, bacterianas e parasitárias mais freqüentes foram pitiríase versicolor (17,8%, verruga vulgar (32,2%, foliculite (4,3% e escabiose (3,8%. CONCLUSÃO: as dermatoses infecciosas são freqüentes nos pacientes transplantados renais, e sua ocorrência aumenta progressivamente conforme o tempo transcorrido a partir do transplante, sendo importante o acompanhamento dermatológico desses pacientes.

  1. Tolerance and safety of superficial chemical peeling with salicylic acid in various facial dermatoses

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    Iqbal Zafar

    2005-03-01

    Full Text Available BACKGROUND: Chemical peeling is a skin-wounding procedure that may have some potentially undesirable side-effects. AIMS: The present study is directed towards safety concerns associated with superficial chemical peeling with salicylic acid in various facial dermatoses. METHODS: The study was a non-comparative and a prospective one. Two hundred and sixty-eight patients of either sex, aged between 10 to 60 years, undergoing superficial chemical peeling for various facial dermatoses (melasma, acne vulgaris, freckles, post-inflammatory scars/pigmentation, actinic keratoses, plane facial warts, etc. were included in the study. Eight weekly peeling sessions were carried out in each patient. Tolerance to the procedure and any undesirable effects noted during these sessions were recorded. RESULTS: Almost all the patients tolerated the procedure well. Mild discomfort, burning, irritation and erythema were quite common but the incidence of major side-effects was very low and these too, were easily manageable. There was no significant difference in the incidence of side-effects between facial dermatoses (melasma, acne and other pigmentary disorders. CONCLUSION: Chemical peeling with salicylic acid is a well tolerated and safe treatment modality in many superficial facial dermatoses.

  2. [Autoimmune pancreatitis].

    Science.gov (United States)

    Beyer, G; Menzel, J; Krüger, P-C; Ribback, S; Lerch, M M; Mayerle, J

    2013-11-01

    Autoimmune pancreatitis is a relatively rare form of chronic pancreatitis which is characterized by a lymphoplasmatic infiltrate with a storiform fibrosis and often goes along with painless jaundice and discrete discomfort of the upper abdomen. Clinically we distinguish between two subtypes, which differ in terms of their histology, clinical picture and prognosis. Type 1 autoimmune pancreatitis is the pancreatic manifestation of the IgG4-associated syndrome which also involves other organs. About one third of the patients can only be diagnosed after either histological prove or a successful steroid trail. Type 2 is IgG4-negative with the histological picture of an idiopathic duct centric pancreatitis and is to higher degree associated with inflammatory bowel disease. A definitive diagnosis can only be made using biopsy. Usually both forms show response to steroid treatment, but in type 1 up to 50 % of the patients might develop a relapse. The biggest challenge and most important differential diagnosis remains the discrimination of AIP from pancreatic cancer, because also AIP can cause mass of the pancreatic head, lymphadenopathy and ductal obstruction. This article summarizes recent advances on epidemiology, clinical presentation, diagnostic strategy, therapy and differential diagnosis in this relatively unknown disease.

  3. Autoimmune hepatitis

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    F Motamed

    2014-04-01

    Full Text Available Autoimmune hepatitis is (AIH is a chronic hepatitis that occurs in children and adults of all ages. It is characterized by immunologic and autoimmune features, including circulating auto antibodies and high serum globulin concentrations. It was first described in the 1950s by term of chronic active hepatitis. It has 2 types with different auto antibodies. Diagnosis is based upon serologic and histologic findings and exclusion of other forms of chronic liver disease.   A scoring system should be used in assessment based upon: 1 Auto anti bodie titer 2 Serum IgG level  3 Liver histology 4 Absence of viral and other causes of hepatitis. Clear indications for treatment: 1   rise of aminotrasferases 2   clinical symptoms of liver disease 3   histological features in liver biopsy 4   Children with AIH initial treatment involve glucocorticoid with or without azathioprine. For patients with fulminant hepatitis liver transplantation, should be kept in mind.   Remission is defined by: 1   Resolution of symptoms 2   Normalization of serum trasaminases 3   Normalization of serum bilirubin and gamma globuline levels. 4   Improvement in liver histology 5   Treatment is continued for at least 2-5 years, glucocorticoids are with drawn first, by tapering over six weeks. Azathioprine will be with drawn.  

  4. Outbreak of staphylococcal bullous impetigo in a maternity ward linked to an asymptomatic healthcare worker.

    Science.gov (United States)

    Occelli, P; Blanie, M; Sanchez, R; Vigier, D; Dauwalder, O; Darwiche, A; Provenzano, B; Dumartin, C; Parneix, P; Venier, A G

    2007-11-01

    An outbreak of staphylococcal bullous impetigo occurred over a period of five months in a maternity ward involving seven infected and two colonised neonates. The skin lesions were due to epidermolytic toxin A-producing Staphylococcus aureus. Infection control measures were implemented and a retrospective case-control study performed. Contact with an auxiliary nurse was the only risk factor for cases of bullous impetigo (Pimpetigo and the auxiliary nurse was reassigned to an adult ward.

  5. PREVALENCE OF PAEDIATRIC DERMATOSES IN THE AGE GROUP OF 5-14 YEARS AT A TERTIARY CARE CENTER IN SALEM

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    Prasanna Kanniah Baskara

    2017-01-01

    Full Text Available BACKGROUND Skin diseases in pediatric age group are common all over the world. Pediatric dermatoses require separate view from adult dermatoses as there are differences in their clinical presentation and treatment. OBJECTIVE The aim of our study was to determine the prevalence of pediatric dermatoses attending our tertiary care center. MATERIALS AND METHODS This was a cross-sectional study conducted in 100 consecutive children with dermatoses between 5-14 years of age attending dermatology outpatient department at Vinayaka Missions Kirupananda Variyar medical college, Salem. The study was conducted over a period of 6 months from February 2016 to August 2016. Demographic parameters, detailed history, clinical features and diagnosis were recorded and analyzed. RESULTS In our short term study, we examined 100 pediatric cases with 106 dermatoses. The incidence of infections and infestations (43.39% was more prevalent in our study. The most common non-infectious dermatoses in our study was insect bite reactions (17.9%. Dermatitis and eczema (9.39%, disorders of sweat and sebaceous glands (6.6%, pigmentary disorders (5.66%, disorders of hair and nails (3.77%, genetic disorders (2.88%, immune and allergic disorders (2.8%, psoriasis (2.8%, nutritional disorders (0.9%, Polymorphic light eruption (0.9%, pearly penile papule (0.9%, aphthous ulcer (0.9% and pityriasis rosea (0.9% were the other dermatoses seen in the study.CONCLUSION Fungal infections (tinea versicolor and tinea corporis, scabies and insect bite reactions were the common dermatoses observed in our study. Most of the pediatric patients attending our hospital came from rural areas belonging to low socioeconomic strata. Health education, proper sanitation and improved nutrition will help to reduce the incidence of pediatric dermatoses.

  6. Bleb Point: Mimicker of Pneumothorax in Bullous Lung Disease

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    Gelabert, Christopher

    2015-05-01

    Full Text Available In patients presenting with severe dyspnea, several diagnostic challenges arise in distinguishing the diagnosis of pneumothorax versus several other pulmonary etiologies like bullous lung disease, pneumonia, interstitial lung disease, and acute respiratory distress syndrome. Distinguishing between large pulmonary bullae and pneumothorax is of the utmost importance, as the acute management is very different. While multiple imaging modalities are available, plain radiographs may be inadequate to make the diagnosis and other advanced imaging may be difficult to obtain. Ultrasound has a very high specificity for pneumothorax. We present a case where a large pulmonary bleb mimics the lung point and therefore inaccurately suggests pneumothorax. [West J Emerg Med. 2015;16(3:447–449.

  7. Figurate and bullous eruption in association with breast carcinoma.

    Science.gov (United States)

    Watsky, K L; Orlow, S J; Bolognia, J L

    1990-05-01

    We describe a patient with two coexistent cutaneous eruptions: (1) trauma-induced bullae of the distal extremities and elbows and (2) multiple concentric gyrate lesions on the trunk and extremities, some of which became bullous. The gyrate lesions were stationary and nonpruritic. Biopsy of both types of lesions showed a subepidermal blister and a minimal inflammatory infiltrate. Direct immunofluorescence revealed linear deposition of IgG and C3 at the dermoepidermal junction and indirect immunofluorescence was negative. By immunoelectron microscopy, these immune deposits were localized to the lower lamina lucida. The eruption was not controlled despite high-dose (80 mg/d) oral administration of prednisone and required the addition of an oral administration of methotrexate (20 mg weekly). On further evaluation, an intraductal mammary carcinoma was detected. Following radiation therapy, the methotrexate and prednisone therapy were tapered without recurrence of the eruption during a follow-up period of 18 months.

  8. Sulfasalazine-induced linear immunoglobulin A bullous dermatosis with DRESS.

    Science.gov (United States)

    Hernández, N; Borrego, L; Soler, E; Hernández, J

    2013-05-01

    Linear immunoglobulin (Ig) A dermatosis is an immune-mediated bullous disease characterized by linear deposits of IgA along the basal membrane. While usually idiopathic, it can occasionally be induced by drug exposure. We report the case of a 60-year-old woman with rheumatoid arthritis being treated with sulfasalazine who developed linear IgA dermatosis and drug rash with eosinophilia and systemic symptoms (DRESS). The dermatosis and associated symptoms resolved following withdrawal of the drug and treatment with systemic corticosteroids for 2 months. This is the first report of sulfasalazine-induced linear IgA dermatosis in association with DRESS and we believe that sulfasalazine should be added to the list of drugs that can cause linear IgA dermatosis.

  9. Intestinal-borne dermatoses significantly improved by oral application of Escherichia coli Nissle 1917

    Science.gov (United States)

    Manzhalii, Elina; Hornuss, Daniel; Stremmel, Wolfgang

    2016-01-01

    AIM: To evaluate the effect of oral Escherichia coli (E. coli) Nissle application on the outcome of intestinal-borne dermatoses. METHODS: In a randomized, controlled, non-blinded prospective clinical trial 82 patients with intestinal-borne facial dermatoses characterized by an erythematous papular-pustular rash were screened. At the initiation visit 37 patients entered the experimental arm and 20 patients constituted the control arm. All 57 patients were treated with a vegetarian diet and conventional topical therapy of the dermatoses with ointments containing tetracycline, steroids and retinoids. In the experimental arm patients received a one month therapy with oral E. coli Nissle at a maintenance dose of 2 capsules daily. The experimental group was compared to a non-treatment group only receiving the diet and topical therapy. The primary outcome parameter was improvement of the dermatoses, secondary parameters included life quality and adverse events. In addition the immunological reaction profile (IgA, interleucin-8 and interferon-α) was determined. Furthermore the changes of stool consistency and the microbiota composition over the time of intervention were recorded. RESULTS: Eighty-nine percent of the patients with acne, papular-pustular rosacea and seborrhoic dermatitis responded to E. coli Nissle therapy with significant amelioration or complete recovery in contrast to 56% in the control arm (P 107 CFU/g stool) was observed in 79% and 63% of the patients, respectively (P 0.05). Accordingly, stool consistency, color and smell normalized in the E. coli Nissle treated patients. CONCLUSION: E. coli Nissle protects the mucus barrier by overgrowth of a favorable gut microbiota with less immunoreactive potential which finally leads to clinical improvement of intestinal borne dermatoses. PMID:27340358

  10. Pattern of dermatoses among nicobarese in a community health camp at Nancowry, Andaman and Nicobar Islands

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    Radhakrishnan Subramaniyan

    2016-01-01

    Full Text Available Background: The Andaman and Nicobar Islands are a group of islands to the east of the Indian mainland. The Nicobar district in its southern part includes the Nancowry group. Very little is known about the dermatoses in this remote region and hence, this study was carried out at a community medical camp held in Kamorta on November 12, 2014. Aims: To study the pattern of dermatoses in Nicobarese attending a community medical camp at Nancowry.Subjects and Methods: All Nicobarese, predominantly mongoloid, attending a multi-specialty community medical camp at Kamorta on November 12, 2014, were initially seen by a general practitioner. Persons with dermatologic complaints or the presence of skin lesions were then seen by a single dermatologist. Results: A total of 375 patients were seen. Out of these, 113 cases (30.13% had a skin disorder. Females comprised 50.44% and males 49.56% of the cases. The mean age was 21.28 years. The most common dermatoses were infections and infestations comprising 53 cases (46.9% of which fungal infections were seen in 25 cases (22.12%, pyodermas in 12 cases (10.62%, scabies in 9 cases (7.96%, warts in 4 cases (3.54%, 1 case each of molluscum contagiosum, herpes zoster, and pediculosis capitis (0.88% followed by eczema in 20 cases (17.70%, acne in 13 cases (11.5%, papular urticaria in 9 cases (7.96%, and psoriasis in 3 cases (2.65%. Miscellaneous dermatoses made up the rest of the 15 cases (13.27%. Conclusion: The pattern of dermatoses seen among the Nicobarese is quite similar with respect to the prevalence of infections in other regions of India, especially humid regions such as Assam, coastal Karnataka, and Kolkata and much higher than arid regions such as the deserts of Rajasthan.

  11. Becker nevus with vitiligo and lichen planus: Cocktail of dermatoses

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    Sanjeev Gupta

    2010-07-01

    Full Text Available Context: Becker nevus has been reported to be associated with lichen planus (LP in isolated case reports in past. The association of LP and vitiligo has been noted in few cases and has been attributed to a common autoimmune etiology. The coexistence of lichen planus, vitiligo and Becker nevus has not been reported so far. Case Report: A thirty five years old male presented with lesions of Becker nevus along with vitiligo and Lichen planus coexisting at one place on right side of the chest. Vitiligo and Becker nevus could not be treated. Lichen planus was confirmed histopathologically. We were able to treat lichen planus with topical potent steroids, tacrolimus and systemic antihistamines. The vitiligo lesion in our case was resistant to treatment. Conclusion: This case is being reported for the rare occurrence of three different well defined skin conditions in our patient and reviews the possible known etiological factors for their coexistence.

  12. Increased Activity and Apoptosis of Eosinophils in Blister Fluids, Skin and Peripheral Blood of Patients with Bullous Pemphigoid.

    Science.gov (United States)

    Engmann, Judith; Rüdrich, Urda; Behrens, Georg; Papakonstantinou, Eleni; Gehring, Manuela; Kapp, Alexander; Raap, Ulrike

    2017-04-06

    Bullous pemphigoid (BP) is an autoimmune blistering skin disease that is more common in elderly individuals. The aim of this study was to determine the functional activity of eosinophils in patients with BP compared with healthy donors. Blood, skin and blister-derived eosinophils were strongly activated in patients with BP, seen by increased surface expression of CD69 compared with controls. CD11b was also increased in BP blood eosinophils, which may explain the striking accumulation of eosinophils in BP (1×106 per ml blister fluid). Furthermore, CCL26 was expressed by activated eosinophils in BP skin and in blister fluid. BP eosinophils also released IL-6, IL-8 and IL-1α in BP blister fluids. Apoptosis in cultivated BP eosinophils was increased and accompanied by enhanced surface externalization of CD95. Caspase 3 positive eosinophils in lesional BP skin and blister fluid also showed the initiation of apoptosis. These results reveal novel pathophysiological aspects of BP, with a strong activation pattern and increased apoptosis of eosinophils in the peripheral blood, skin and blister fluids.

  13. Role of IgE in bullous pemphigoid: a review and rationale for IgE directed therapies.

    Science.gov (United States)

    Messingham, K N; Pietras, T A; Fairley, J A

    2012-06-01

    Bullous pemphigoid (BP) is an autoimmune blistering disorder that is characterized by elevated total serum IgE and both IgG and IgE class autoantibodies directed against the hemidesmosomal proteins BP180 and BP230. In BP, IgE is found at the basement membrane zone and coating mast cells in lesional skin. IgE binding to immune cells is mediated through its high affinity receptor, FcεRI on the surface of mast cells, basophils and eosinophils. In BP lesions, IgE binding is thought to be a critical step in the activation of these cells. Models of the disease have demonstrated that BP IgE can replicate the early stages of BP lesion formation. These findings suggest that IgE inhibition may be a therapeutic approach for BP. Omalizumab is a humanized monoclonal antibody that inhibits IgE binding to FcεRI and is currently FDA-approved for the treatment of severe allergic asthma. To date, two case reports have each described the efficacy of omalizumab in a patient with severe recalcitrant BP. These studies are the first to provide clear evidence of the contribution of IgE autoantibodies in the pathogenesis of human BP and suggest that omalizumab may provide an additional therapeutic tool for treatment.

  14. Diaminodiphenyl Sulfone-Induced Hemolytic Anemia and Alopecia in a Case of Linear IgA Bullous Dermatosis.

    Science.gov (United States)

    Akasaka, Eijiro; Kayo, Sato-Jin; Nakano, Hajime; Ishii, Norito; Hashimoto, Takashi; Sawamura, Daisuke

    2015-01-01

    Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is an autoimmune mucocutaneous disease characterized by subepidermal blistering induced by IgA autoantibodies against several autoantigens in the basal membranous zone of the skin and mucosal tissue. Although diaminodiphenyl sulfone (DDS), also known as dapsone, is generally recognized as the first-line therapy for LABD, DDS can induce several severe side effects. We present a Japanese case of LABD with DDS-induced hemolytic anemia and alopecia. In the present case, the DDS-induced hemolytic anemia and hair loss made the DDS monotherapy difficult. When DDS is used in LABD patients with iron deficiency anemia (IDA), hemolytic anemia is concealed by IDA. It is thus necessary to carefully and frequently examine the laboratory data to find the signs of DDS-induced hemolytic anemia. Even though there is no literature on DDS-induced alopecia, alopecia was reported as one of the side effects of DDS in an FDA report, and, in our case, hair loss was improved after reducing its dosage. We have to recognize that alopecia is one of the side effects of DDS and that careful management is needed in order not to overlook the adverse side effects of DDS when treating LABD patients.

  15. Diaminodiphenyl Sulfone-Induced Hemolytic Anemia and Alopecia in a Case of Linear IgA Bullous Dermatosis

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    Eijiro Akasaka

    2015-07-01

    Full Text Available Linear immunoglobulin A (IgA bullous dermatosis (LABD is an autoimmune mucocutaneous disease characterized by subepidermal blistering induced by IgA autoantibodies against several autoantigens in the basal membranous zone of the skin and mucosal tissue. Although diaminodiphenyl sulfone (DDS, also known as dapsone, is generally recognized as the first-line therapy for LABD, DDS can induce several severe side effects. We present a Japanese case of LABD with DDS-induced hemolytic anemia and alopecia. In the present case, the DDS-induced hemolytic anemia and hair loss made the DDS monotherapy difficult. When DDS is used in LABD patients with iron deficiency anemia (IDA, hemolytic anemia is concealed by IDA. It is thus necessary to carefully and frequently examine the laboratory data to find the signs of DDS-induced hemolytic anemia. Even though there is no literature on DDS-induced alopecia, alopecia was reported as one of the side effects of DDS in an FDA report, and, in our case, hair loss was improved after reducing its dosage. We have to recognize that alopecia is one of the side effects of DDS and that careful management is needed in order not to overlook the adverse side effects of DDS when treating LABD patients.

  16. Dermatoses em pacientes com AIDS: estudo de 55 casos. Uberlândia, MG, Brasil

    OpenAIRE

    GOMIDES MABEL DUARTE ALVES; BERBERT ALCEU LUIZ CAMARGO VILLELA; MANTESE SÔNIA ANTUNES DE OLIVEIRA; ROCHA ADENIR; FERREIRA MARCELO SIMÃO; BORGES AERCIO S.

    2002-01-01

    Foram estudados 55 pacientes com a síndrome da imunodeficiência adquirida (Aids), triados ao serviço de Dermatologia, apresentando doenças cutâneo-mucosas, algumas de caráter oportunista. As dermatoses são freqüentes nestes doentes e, às vezes, de difícil diagnóstico pelo caráter atípico das lesões. OBJETIVOS: Analisar a freqüência e apresentação clínica das dermatoses relacionadas à Aids. MÉTODOS: Cinqüenta e cinco pacientes com Aids e lesões tegumentares foram estudados, de modo transversal...

  17. Evaluation of Obesity-Associated Dermatoses in Obese and Overweight Individuals

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    Nurhan Döner

    2011-09-01

    Full Text Available Background and Design: Although the effects of obesity on the skin are known, there are no adequate studies on this subject. To evaluate obesity-related dermatoses in obese and overweight people and to compare the obtained results with those of individuals with body mass index (BMI lower than 25 kg/m2.Material and methods: A total of 500 (mean age: 43.6±12.8; 318 female, 182 male volunteer patients aged 18 years and older, who were treated in inpatient clinics except dermatology, and their relatives were included in the study. The subjects were grouped according to BMI: less than 25 kg/m2 (controls, n = 120, 25-29.9 kg/m2 (overweights, n=130, and greater than 30 kg/m2 (obeses, n=250. The participants were examined dermatologically and all known skin diseases associated with obesity were noted. Results: The total number of dermatoses was statistically higher in obeses and overweights than in controls, as well as in obeses than in overweights. The five most frequently seen dermatoses in all groups were: varicose vein, tinea pedis, striae distensae, acrochordon and onychomycosis, respectively. There were statistically significant differences: between obeses and controls with regard to varicose vein, tinea pedis, striae distensae, acrochordon, onychomycosis, acanthosis nigricans and intertrigo; between overweights and controls with respect to varicose vein, tinea pedis, striae distensae, acrochordon and onychomycosis; between obeses and overweights with regard to striae distensae, acanthosis nigricans and onychomycosis. A positive correlation was detected between BMI and degree of plantar hyperkeratosis in all participants; between BMI and hirsutism score, severity of androgenetic alopecia (AGA and cellulite in women. Conclusion: While acanthosis nigricans and AGA in females were associated with obesity, the other dermatoses were related with not only with obesity but also with increasing weight gain.

  18. Psoralen plus ultraviolet A (PUVA soaks and UVB TL01 treatment for chronic hand dermatoses

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    Lisbeth Jensen

    2012-02-01

    Full Text Available Chronic eczematous hand dermatoses with and without contact allergies are complex diseases, which makes it a challenge to select the best treatment and obtain an optimal patient experience and a satisfactory treatment result. The aim of this study was to evaluate retrospectively the clinical effect and patient experience of local treatment with psoralen plus ultraviolet A (PUVA soaks and TL01 phototherapy for severe chronic hand dermatoses, and also to evaluate the quality of life for the subgroup of patients with allergic contact dermatitis including Compositae allergy. A retrospective evaluation of results for 94 consecutive patients having received a total of 121 treatment courses with local PUVA soaks or TL01 phototherapy for one of the following diagnoses (n=number of treatment courses: psoriasis (n=19, hyperkeratotic hand eczema (n=27, Pustulosis Palmoplantaris (PPP (n=22, vesicular eczema (n=16, Compositae dermatitis (n=24, and allergic contact dermatitis (n=13. Moreover, semi-structured interviews with 6 selected patients having multiple contact allergies including Compositae allergy were used to evaluate quality of life. As a result, we found that PUVA soaks has good effect in patients with psoriasis and hyperkeratotic hand eczema and local phototherapy for chronic hand dermatoses is a useful treatment option in selected cases.

  19. Pattern of Pediatric Dermatoses in a Tertiary Care Hospital of Western Nepal

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    Yogesh Poudyal

    2016-01-01

    Full Text Available Pediatric dermatoses are one of the most common presentations in a dermatology clinic and reflect the health and hygiene status of children. The incidence and severity of these skin lesions are influenced by geographical area, seasonal and cultural factors, and socioeconomic status. This study was done to show the prevalence of different pediatric dermatoses in a tertiary care hospital of Western Nepal. Chart reviews of children aged one day to 17 years, presenting to Universal Medical College Teaching Hospital, Nepal, from 1 September 2014 to 30 august 2015, were done. Descriptive analysis and two-sided chi-square test were done. Among 23992 patients visiting the dermatology outpatient department (OPD, 5398 (22.5% were of pediatric age groups (male/female: 1.2/1; most of them belonged to young teens and teenagers (n=3308; 61.3%. Three most common dermatoses were fungal infections (18.5%, eczema (14.4%, and acne (10.1%. Fungal infections (n=653; 65.4% and acne (n=284; 51.9% were common in males, whereas eczema (n=402; 51.7% was more common in females. Fungal infection (P<0.001, eczema (P<0.001, pigmentation disorders (P<0.001, and acne (P<0.01 were significantly more during summer, while scabies was more in winter (P<0.001. Dermatophytosis, pyoderma, and warts comprised frequently occurring fungal, bacterial, and viral infections, respectively.

  20. Pregnancy Dermatoses%妊娠期特异性皮肤病

    Institute of Scientific and Technical Information of China (English)

    敖俊红; 杨蓉娅

    2011-01-01

    The nomcnclature and nosologic classification of pregnancy dermatoses are still confusing,around which the dispute had been existing for a long time.According to the new classification system.the diseases were classified into five classes.which known as pemphigoid gestationis,intrahepatic cholestasis of pregnancy, impetigo herpetiformis, polymorphic eruption of pregnancy and papular dermatoses of pregnancy(prurigo of pregnancy.pruritic folliculitis of pregnancy and atopic eruption of pregnancy).The review focused on the advances in the following issues about pregnancy dermatoses:classification, characterization of clinical manifestations, diagnosis and management, prognosis and impacts on fetals.%妊娠期特异性皮肤病的命名及分类一直存在争议,最新的分类将其分为妊娠性类天疱疮、妊娠肝内胆汁淤积、疱疹样脓疱病、妊娠性多形疹、妊娠丘疹性皮肤病(包括妊娠痒疹、妊娠瘙痒性毛囊炎及妊娠特应性皮肤病)五类.本文主要介绍妊娠期特异性皮肤病的分类、临床特点、诊断和鉴别诊断、治疗、预后及对胎儿的影响等方面的研究进展.

  1. Dermatoses among paddy field workers - A descriptive, cross-sectional pilot study

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    Shenoi Shrutakirthi

    2005-01-01

    Full Text Available BACKGROUND: Paddy farming is one of the main occupations in coastal South India. Dermatological problems in paddy field workers have not received much attention. AIM : The purpose of this study was to study the dermatoses of the exposed parts of the body, viz. face, hands, and feet, in paddy field workers. METHODS: Three hundred and forty-one workers were questioned and clinical findings noted. Scrapings for bacterial and fungal examination were taken by random selection. RESULTS: Seventy-three per cent had work-related itching. Melasma was the commonest facial lesion (41.1%. The main problems on the hands were hyperkeratosis (26.4%, nail dystrophy (15.2% and paronychia (8.8%. Common feet dermatoses included nail dystrophy (57.1%, pitted keratolysis (42.5% and fissuring (23.5%. Common aerobic and anaerobic bacteria isolated from pitted keratolysis and intertrigo were Klebsiella and Clostridium species. Aspergillus species were the commonest fungus grown from intertrigo. CONCLUSIONS: Occupational dermatoses are common in paddy field workers.

  2. Immunohistological evaluation of feline herpesvirus-1 infection in feline eosinophilic dermatoses or stomatitis.

    Science.gov (United States)

    Lee, Meichet; Bosward, Katrina L; Norris, Jacqueline M

    2010-02-01

    This study used immunohistochemistry (IHC) and histopathology to evaluate the presence of feline herpesvirus-1 (FHV-1) in feline cases of 'eosinophilic granuloma complex' (EGC) or other eosinophilic dermatoses or stomatitis, diagnosed at the Veterinary Pathology Diagnostic Service, University of Sydney between January 1996 and June 2008. Two of the 30 cases (6.6%) examined showed positive immunoreactivity to FHV-1 using IHC. Intranuclear inclusion bodies were also detected on histopathological examination of haematoxylin and eosin stained sections of both cases but were very difficult to find. Therefore, FHV-1 is uncommonly associated with EGC or other eosinophilic dermatoses or stomatitis in Sydney. However, misdiagnosis as an EGC lesion or other eosinophilic dermatoses may occur if inclusion bodies are overlooked or absent on histopathology and this may significantly decrease the chance of a favourable treatment outcome. FHV-1 should be considered in cats with severe ulcerative cutaneous or oral lesions, unresponsive to corticosteroid treatment, with or without concurrent or historical signs of upper respiratory tract or ocular disease more typical of FHV-1. IHC may be helpful in differentiating FHV-1 dermatitis or stomatitis from other eosinophilic lesions, which is of vital clinical and therapeutic importance.

  3. The autoimmune tautology.

    Science.gov (United States)

    Anaya, Juan-Manuel

    2010-01-01

    Although autoimmune diseases exhibit contrasting epidemiological features, pathology, and clinical manifestations, three lines of evidence demonstrate that these diseases share similar immunogenetic mechanisms (that is, autoimmune tautology). First, clinical evidence highlights the co-occurrence of distinct autoimmune diseases within an individual (that is, polyautoimmunity) and within members of a nuclear family (that is, familial autoimmunity). Second, physiopathologic evidence indicates that the pathologic mechanisms may be similar among autoimmune diseases. Lastly, genetic evidence shows that autoimmune phenotypes might represent pleiotropic outcomes of the interaction of non-specific disease genes.

  4. Bullous variant of familial biphasic lichen amyloidosis: A unique combination of three rare presentations

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    Vijayalaxmi Veerabasappa Suranagi

    2015-01-01

    Full Text Available A 55-year-old man presented with multiple, itchy papules and macules on the trunk and extremities. Histopathologic examination of biopsy specimens taken from three different lesions showed a subepidermal blister with amyloid deposits in the dermal papillae. No systemic disease or involvement of other organs was detected. The clinical and histological findings were compatible with a bullous variant of lichen amyloidosis (LA. Primary cutaneous localized amyloidosis usually presents with papular, macular or nodular lesions. Bullous lesions associated with LA are very rare. Furthermore, patient had seven other members in the family with similar lesions, which is also a rare occurrence. We report a case with a rare combination of biphasic, bullous variant of familial LA.

  5. Unusual clinicopathological and immunological presentation of chronic bullous dermatosis of childhood (linear IgA dermatosis

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    Al-Saif Fahad

    2011-01-01

    Full Text Available Linear IgA bullous dermatosis is a rare sulfone-responsive subepidermal blistering disorder of unknown etiology in which smooth linear deposits of IgA are found in the basement membrane zone. Chronic bullous dermatosis of childhood is equivalent to linear IgA disease of adulthood and is characterized by an abrupt onset of large, widespread and tense bullae on a normal or erythematous base. In this case, we describe an unusual presentation of chronic bullous dermatosis in a 14-month-old Saudi girl. Histopathological examination revealed subepidermal cell poor blisters with linear deposition of IgA, IgG, IgM, and C3 along the dermoepidermal junction. The unusual clinical, histopathological and immunofluorescence findings in this patient are discussed, with an account on the differential diagnosis in such cases along with a detailed review of the relevant literature.

  6. Unusual clinicopathological and immunological presentation of chronic bullous dermatosis of childhood (linear IgA dermatosis).

    Science.gov (United States)

    Fahad, Al-Saif; Ammar, Al-Rikabi

    2011-01-01

    Linear IgA bullous dermatosis is a rare sulfone-responsive subepidermal blistering disorder of unknown etiology in which smooth linear deposits of IgA are found in the basement membrane zone. Chronic bullous dermatosis of childhood is equivalent to linear IgA disease of adulthood and is characterized by an abrupt onset of large, widespread and tense bullae on a normal or erythematous base. In this case, we describe an unusual presentation of chronic bullous dermatosis in a 14-month-old Saudi girl. Histopathological examination revealed subepidermal cell poor blisters with linear deposition of IgA, IgG, IgM, and C3 along the dermoepidermal junction. The unusual clinical, histopathological and immunofluorescence findings in this patient are discussed, with an account on the differential diagnosis in such cases along with a detailed review of the relevant literature.

  7. Autoimmune Inner Ear Disease

    Science.gov (United States)

    ... Find an ENT Doctor Near You Autoimmune Inner Ear Disease Autoimmune Inner Ear Disease Patient Health Information ... with a hearing loss. How Does the Healthy Ear Work? The ear has three main parts: the ...

  8. Perspectives on autoimmunity

    Energy Technology Data Exchange (ETDEWEB)

    Cohen, I.R.

    1987-01-01

    The contents of this book are: HLA and Autoimmunity; Self-Recognition and Symmetry in the Immune System; Immunology of Insulin Dependent Diabetes Mellitus; Multiple Sclerosis; Autoimmunity and Immune Pathological Aspects of Virus Disease; Analyses of the Idiotypes and Ligand Binding Characteristics of Human Monoclonal Autoantibodies to DNA: Do We Understand Better Systemic Lupus Erythematosus. Autoimmunity and Rheumatic Fever; Autoimmune Arthritis Induced by Immunization to Mycobacterial Antigens; and The Interaction Between Genetic Factors and Micro-Organisms in Ankylosing Spondylitis: Facts and Fiction.

  9. Prevalência de dermatoses pediátricas em um hospital universitário na região sudeste do Brasil Prevalence of pediatric dermatoses in a university hospital in southeastern Brazil

    Directory of Open Access Journals (Sweden)

    Flávia Regina Ferreira

    2011-06-01

    Full Text Available FUNDAMENTOS: Numerosas dermatoses afetam crianças, dependendo da idade, da região e da classe socioeconômica. OBJETIVO: Determinar a prevalência de dermatoses pediátricas em um hospital universitário, considerando-se o diagnóstico, a idade e o sexo. MÉTODOS: Estudo epidemiológico transversal realizado de julho de 2006 a dezembro de 2007. Análise dos prontuários de 264 pacientes do Ambulatório de Dermatologia Pediátrica de um hospital universitário nesse período. A variável dependente foi a existência ou não de dermatoses em crianças até os 19 anos de idade. Entre as variáveis independentes obtiveram-se: diagnóstico clínico, sexo e idade. RESULTADOS: Dos 264 prontuários analisados, observou-se maior prevalência de dermatoses alérgicas em 74 casos (28,0%, seguidas por dermatoses inflamatórias em 49 casos (18,6%, dermatoses pigmentares em 42 casos (15,9%, dermatoses infecciosas em 38 casos (14,4%, tumores benignos em 25 casos (9,5%, miscelânea em 14 casos (5,3%, genodermatoses em 12 casos (4,5% e afecções de anexos cutâneos em dez casos (3,8%. Os lactentes perfizeram 11,3% do total, os pré-escolares, 15,9%, os escolares, 48,8% e os adolescentes, 23,8%. Observou-se maior incidência de dermatoses alérgicas em pré-escolares em 15 casos (35,7%, em lactentes em dez casos (33,3% e em escolares em 39 casos (30,2%. Entre os adolescentes destacaram-se as dermatoses inflamatórias. O estudo não mostrou diferenças estatísticas entre sexo e faixa etária. CONCLUSÕES: O estudo do perfil epidemiológico facilita o diagnóstico das dermatoses pediátricas, incentivando a boa anamnese e a busca da prevençãoBACKGROUND: Numerous dermatoses affects children, depending on age, region and socioeconomic status. OBJECTIVE: To determine the prevalence of pediatric dermatoses at the Dermatology Department of a University Hospital, involving the diagnosis, age and sex. METHODS: Epidemiologic cross-sectional study carried out in the

  10. Bullous lung diseases as a risk factor for lung cancer: A case report

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    Nagorni-Obradović Ljudmila

    2016-01-01

    Full Text Available Introduction. A possible association between lung cancer and bullous lung disease has been suggested and recently supported by the results of genetic studies. Case report. A previously healthy 43-year-old man, smoker, was diagnosed with bullous lung disease at the age of 31 years. He was followed up for 12 years when lung cancer (adenocarcinoma was found at the site. In the meantime, he was treated for recurrent respiratory infections. Conclusion. There is the need for active approach in following up the patients with pulmonary bulla for potential development of lung cancer.

  11. Autoimmune Hemolytic Anemia.

    Science.gov (United States)

    Liebman, Howard A; Weitz, Ilene C

    2017-03-01

    Autoimmune hemolytic anemia is an acquired autoimmune disorder resulting in the production of antibodies directed against red blood cell antigens causing shortened erythrocyte survival. The disorders can present as a primary disorder (idiopathic) or secondary to other autoimmune disorders, malignancies, or infections. Treatment involves immune modulation with corticosteroids and other agents.

  12. Application of Mycobacterium Leprae-specific cellular and serological tests for the differential diagnosis of leprosy from confounding dermatoses.

    Science.gov (United States)

    Freitas, Aline Araújo; Hungria, Emerith Mayra; Costa, Maurício Barcelos; Sousa, Ana Lúcia Osório Maroccolo; Castilho, Mirian Lane Oliveira; Gonçalves, Heitor Sá; Pontes, Maria Araci Andrade; Duthie, Malcolm S; Stefani, Mariane Martins Araújo

    2016-10-01

    Mycobacterium leprae-specific serological and cell-mediated-immunity/CMI test were evaluated for the differential diagnosis of multibacillary/MB, and paucibacillary/PB leprosy from other dermatoses. Whole-blood assay/WBA/IFNγ stimulated with LID-1 antigen and ELISA tests for IgG to LID-1 and IgM to PGL-I were performed. WBA/LID-1/IFNγ production was observed in 72% PB, 11% MB leprosy, 38% dermatoses, 40% healthy endemic controls/EC. The receiver operating curve/ROC for WBA/LID-1 in PB versus other dermatoses showed 72.5% sensitivity, 61.5% specificity and an area-under-the-curve/AUC=0.75; 74% positive predictive value/PPV, 59% negative predictive value/NPV. Anti PGL-I serology was positive in 67% MB, 8% PB leprosy, 6% of other dermatoses; its sensitivity for MB=66%, specificity=93%, AUC=0.89; PPV=91%, NPV=72%. Anti-LID-1 serology was positive in 87% MB, 7% PB leprosy, all other participants were seronegative; 87.5% sensitivity for MB, 100% specificity, AUC=0.97; PPV=100%, NPV=88%. In highly endemic areas anti-LID-1/PGL-I serology and WBA/LID-1-represent useful tools for the differential diagnosis of leprosy from other confounding dermatoses.

  13. A 12-year retrospective review of bullous systemic lupus erythematosus in cutaneous and systemic lupus erythematosus patients.

    Science.gov (United States)

    Chanprapaph, K; Sawatwarakul, S; Vachiramon, V

    2017-01-01

    Objective The aim of this study was to investigate the clinical features, laboratory findings, systemic manifestations, treatment and outcome of patients with bullous systemic lupus erythematosus in a tertiary care center in Thailand. Methods We performed a retrospective review from 2002 to 2014 of all patients who fulfilled the diagnostic criteria for bullous systemic lupus erythematosus to evaluate for the clinical characteristics, extracutaneous involvement, histopathologic features, immunofluorescence pattern, serological abnormalities, internal organ involvement, treatments and outcome. Results Among 5149 patients with cutaneous lupus erythematosus and/or systemic lupus erythematosus, 15 developed vesiculobullous lesions. Ten patients had validation of the diagnosis of bullous systemic lupus erythematosus, accounting for 0.19%. Bullous systemic lupus erythematosus occurred after the diagnosis of systemic lupus erythematosus in six patients with a median onset of 2.5 months (0-89). Four out of 10 patients developed bullous systemic lupus erythematosus simultaneously with systemic lupus erythematosus. Hematologic abnormalities and renal involvement were found in 100% and 90%, respectively. Polyarthritis (40%) and serositis (40%) were less frequently seen. Systemic corticosteroids, immunosuppressants, antimalarials and dapsone offered resolution of cutaneous lesions. Conclusion Bullous systemic lupus erythematosus is an uncommon presentation of systemic lupus erythematosus. Blistering can occur following or simultaneously with established systemic lupus erythematosus. We propose that clinicians should carefully search for systemic involvement, especially hematologic and renal impairment, in patients presenting with bullous systemic lupus erythematosus.

  14. Prevalence, number and morphological types of multinucleated histiocytic giant cells in equine inflammatory dermatoses: a retrospective light microscopic study of skin-biopsy specimens from 362 horses.

    Science.gov (United States)

    Cohen, R D; Scott, D W; Erb, H N

    2009-04-01

    Multinucleated histiocytic giant cells (MHGC) are seen frequently in skin-biopsy specimens from horses with inflammatory dermatoses. However, the prevalence, number and morphological types of these cells have not been reported. To determine the prevalence, number and morphological types of MHGC in equine inflammatory dermatoses, and the association of these cells with specific conditions. Skin-biopsy specimens from 335 horses with inflammatory dermatoses and from 27 horses with normal skin were evaluated for the prevalence, number and morphological types of MHGC. The prevalence and number of MHGC were greater in granulomatous dermatoses than in nongranulomatous dermatoses. Infectious and noninfectious dermatoses were not different in terms of prevalence or morphological types of MHGC. Foreign-body MHGC were the predominant type in almost all cases. MHGC were not seen in normal skin. MHGC are seen in a wide variety of equine inflammatory dermatoses, especially those that are granulomatous. Number and morphological types of MHGC are of no apparent diagnostic significance. MHGC are frequently present in a wide variety of inflammatory dermatoses in the horse. Because the prevalence, number and morphological types of MHGC are of minimal diagnostic significance, special stains and tissue cultures are necessary to confirm specific diagnoses.

  15. Linear IgA and IgG bullous dermatosis.

    Science.gov (United States)

    Fernandes, Karina de Almeida Pinto; Galvis, Kely Hernández; Gomes, Anndressa Camillo da Matta Setubal; Nogueira, Osvania Maris; Felix, Paulo Antônio Oldani; Vargas, Thiago Jeunon de Sousa

    2016-01-01

    Childhood linear immunoglobulin A dermatosis is a rare autoimmune vesiculobullous disease. It results in linear deposition of autoantibodies (immunoglobulin A) against antigens in the basal membrane zone, leading to subepidermal cleavage. Additional depositions of immunoglobulin G and complement-3 might occur. It is still debated whether concomitant findings of immunoglobulins A and G should be considered a subtype of this dermatosis or a new entity. Further studies are needed to recognize this clinical variant.

  16. Linear IgA and IgG bullous dermatosis*

    Science.gov (United States)

    Fernandes, Karina de Almeida Pinto; Galvis, Kely Hernández; Gomes, Anndressa Camillo da Matta Setubal; Nogueira, Osvania Maris; Felix, Paulo Antônio Oldani; Vargas, Thiago Jeunon de Sousa

    2016-01-01

    Childhood linear immunoglobulin A dermatosis is a rare autoimmune vesiculobullous disease. It results in linear deposition of autoantibodies (immunoglobulin A) against antigens in the basal membrane zone, leading to subepidermal cleavage. Additional depositions of immunoglobulin G and complement-3 might occur. It is still debated whether concomitant findings of immunoglobulins A and G should be considered a subtype of this dermatosis or a new entity. Further studies are needed to recognize this clinical variant. PMID:28300887

  17. A prospective study of clinical profile in patients of palmoplantar dermatoses

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    Pragya A Nair

    2017-01-01

    Full Text Available Background: Palms and soles are the readily visible areas of the body that are affected in various dermatoses. This may have considerable concern to the patient and can cause diagnostic dilemma. Moreover, palmoplantar dermatoses also limit our day to day activities with a significant impact on quality of life. Aim: To study the clinical profile of patients suffering from palmoplantar dermatoses at a tertiary care centre. Patients and Methods: A prospective observational study was carried out from June 2014 to May 2015 in the Department of Dermatology, Venerology and Leprosy at a rural tertiary care centre after taking approval from the institutional ethical committee. In total, 202 cases were enrolled. The study was analyzed using Chi-square test. Results: A total of 202 patients were enrolled, among which 53.46% were males. The most common age group affected was 17–40 years (42.57%. Duration of less than 1 month was seen in 31.7% cases whereas 29.7% showed seasonal variation. The most common chief complaint was itching (69.8%. Palmoplantar psoriasis was the most common dermatosis with 28.22% cases, followed by keratinizing disorders with 26.72% cases. Palms were involved in 66.34% cases, soles in 69.30%, and both palms and soles in 37.12% cases. Other body parts were involved in 15.34% of the cases. Diabetes and hypertension were common comorbid conditions seen in 11.4% of the cases each. Conclusion: Palmoplantar psoriasis was the most common disorder affecting palms and soles followed by palmoplantar keratoderma.

  18. Non-Descemet’s stripping automated endothelial keratoplasty for bullous keratopathy secondary to iridoschisis

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    Minezaki T

    2013-07-01

    Full Text Available Teruumi Minezaki, Takaaki Hattori, Hayate Nakagawa, Shigeto Kumakura, Hiroshi GotoDepartment of Ophthalmology, Tokyo Medical University, Shinjukuku, Tokyo, JapanPurpose: To report a case of bullous keratopathy secondary to iridoschisis treated by non-Descemet's stripping automated endothelial keratoplasty (nDSAEK.Case report: A 79-year-old woman was referred to our hospital with loss of vision in the left eye. Slit lamp examination of her left eye showed a shallow anterior chamber with cataract and schisis in the inferior quadrant of iris stroma. Bullous keratopathy secondary to iridoschisis was diagnosed. Cataract surgery with iridectomy succeeded to deepen the anterior chamber and remove the floating iris leaf, although corneal edema remained. Four days later, nDSAEK was performed, which resolved corneal edema and restored visual acuity.Conclusion: The two-step surgery of cataract surgery plus iridectomy followed by nDSAEK may be an effective strategy for treating bullous keratopathy secondary to iridoschisis.Keywords: iridoschisis, bullous keratopathy, non-Descemet's stripping automated endothelial keratoplasty

  19. Definitions and outcome measures for bullous pemphigoid : Recommendations by an international panel of experts

    NARCIS (Netherlands)

    Murrell, Dedee F.; Daniel, Benjamin S.; Joly, Pascal; Borradori, Luca; Amagai, Masayuki; Hashimoto, Takashi; Caux, Frederic; Marinovic, Branka; Sinha, Animesh A.; Hertl, Michael; Bernard, Philippe; Sirois, David; Cianchini, Giuseppe; Fairley, Janet A.; Jonkman, Marcel F.; Pandya, Amit G.; Rubenstein, David; Zillikens, Detlef; Payne, Aimee S.; Woodley, David; Zambruno, Giovanna; Aoki, Valeria; Pincelli, Carlo; Diaz, Luis; Hall, Russell P.; Meurer, Michael; Mascaro, Jose M.; Schmidt, Enno; Shimizu, Hiroshi; Zone, John; Swerlick, Robert; Mimouni, Daniel; Culton, Donna; Lipozencic, Jasna; Bince, Benjamin; Grando, Sergei A.; Bystryn, Jean-Claude; Werth, Victoria P.

    Our scientific knowledge of bullous pemphigoid (BP) has dramatically progressed in recent years. However, despite the availability of various therapeutic options for the treatment of inflammatory diseases, only a few multicenter controlled trials have helped to define effective therapies in BP. A

  20. Association between bullous pemphigoid and neurologic diseases: a case-control study.

    Science.gov (United States)

    Casas-de-la-Asunción, E; Ruano-Ruiz, J; Rodríguez-Martín, A M; Vélez García-Nieto, A; Moreno-Giménez, J C

    2014-11-01

    In the past 10 years, bullous pemphigoid has been associated with other comorbidities and neurologic and psychiatric conditions in particular. Case series, small case-control studies, and large population-based studies in different Asian populations, mainland Europe, and the United Kingdom have confirmed this association. However, no data are available for the Spanish population. This was an observational, retrospective, case-control study with 1:2 matching. Fifty-four patients with bullous pemphigoid were selected. We compared the percentage of patients in each group with concurrent neurologic conditions, ischemic heart disease, diabetes, chronic obstructive pulmonary disease, and solid tumors using univariate logistic regression. An association model was constructed with conditional multiple logistic regression. The case group had a significantly higher percentage of patients with cerebrovascular accident and/or transient ischemic attack (odds ratio [OR], 3.06; 95% CI, 1.19-7.87], dementia (OR, 5.52; 95% CI, 2.19-13.93), and Parkinson disease (OR, 5; 95% CI, 1.57-15.94). A significantly higher percentage of cases had neurologic conditions (OR, 6.34; 95% CI, 2.89-13.91). Dementia and Parkinson disease were independently associated with bullous pemphigoid in the multivariate analysis. Patients with bullous pemphigoid have a higher frequency of neurologic conditions. Copyright © 2013 Elsevier España, S.L.U. and AEDV. All rights reserved.

  1. Definitions and outcome measures for bullous pemphigoid : Recommendations by an international panel of experts

    NARCIS (Netherlands)

    Murrell, Dedee F.; Daniel, Benjamin S.; Joly, Pascal; Borradori, Luca; Amagai, Masayuki; Hashimoto, Takashi; Caux, Frederic; Marinovic, Branka; Sinha, Animesh A.; Hertl, Michael; Bernard, Philippe; Sirois, David; Cianchini, Giuseppe; Fairley, Janet A.; Jonkman, Marcel F.; Pandya, Amit G.; Rubenstein, David; Zillikens, Detlef; Payne, Aimee S.; Woodley, David; Zambruno, Giovanna; Aoki, Valeria; Pincelli, Carlo; Diaz, Luis; Hall, Russell P.; Meurer, Michael; Mascaro, Jose M.; Schmidt, Enno; Shimizu, Hiroshi; Zone, John; Swerlick, Robert; Mimouni, Daniel; Culton, Donna; Lipozencic, Jasna; Bince, Benjamin; Grando, Sergei A.; Bystryn, Jean-Claude; Werth, Victoria P.

    2012-01-01

    Our scientific knowledge of bullous pemphigoid (BP) has dramatically progressed in recent years. However, despite the availability of various therapeutic options for the treatment of inflammatory diseases, only a few multicenter controlled trials have helped to define effective therapies in BP. A ma

  2. Whole body application of a potent topical corticosteroid for bullous pemphigoid

    NARCIS (Netherlands)

    Terra, J. B.; Potze, W. J. B.; Jonkman, M. F.

    BackgroundCurrent standard of treatment of bullous pemphigoid (BP) is systemic oral corticosteroids (CS). However, significant iatrogenic morbidity and mortality is reported. Studies have shown that topical potent CS is safer than oral prednisolone in BP. ObjectivesTo examine the local and systemic

  3. Sirolimus for Autoimmune Disease of Blood Cells

    Science.gov (United States)

    2017-03-16

    Autoimmune Pancytopenia; Autoimmune Lymphoproliferative Syndrome (ALPS); Evans Syndrome; Idiopathic Thrombocytopenic Purpura; Anemia, Hemolytic, Autoimmune; Autoimmune Neutropenia; Lupus Erythematosus, Systemic; Inflammatory Bowel Disease; Rheumatoid Arthritis

  4. Dermatose perfurante adquirida associada à insuficiência hepática em paciente transplantado de fígado

    OpenAIRE

    Badziak,Daniela; Lenhardt,Carolina; Barros,Michele F. de; Mandelli,Fernando Luiz; Serafini,Sérgio Zuñeda; Santamaria,Jesus Rodriguez

    2007-01-01

    A dermatose perfurante adquirida é entidade clinicopatológica caracterizada por eliminação transepitelial de material dérmico degenerado, ocorrendo em muitas condições, entre elas diabetes mellitus, insuficiência renal crônica e colangite esclerosante. Relata-se o caso de paciente de 17 anos, com dermatose perfurante adquirida associada à insuficiência hepática crônica, conseqüente à complicação hepática de transplante de fígado para tratamento de sua doença de base, a glicogenose tipo I....

  5. Dermatose perfurante adquirida associada à insuficiência hepática em paciente transplantado de fígado

    OpenAIRE

    Daniela Badziak; Carolina Lenhardt; Barros,Michele F. de; Fernando Luiz Mandelli; Sérgio Zuñeda Serafini; Jesus Rodriguez Santamaria

    2007-01-01

    A dermatose perfurante adquirida é entidade clinicopatológica caracterizada por eliminação transepitelial de material dérmico degenerado, ocorrendo em muitas condições, entre elas diabetes mellitus, insuficiência renal crônica e colangite esclerosante. Relata-se o caso de paciente de 17 anos, com dermatose perfurante adquirida associada à insuficiência hepática crônica, conseqüente à complicação hepática de transplante de fígado para tratamento de sua doença de base, a glicogenose tipo I.Acqu...

  6. Galectin-3 in autoimmunity and autoimmune diseases.

    Science.gov (United States)

    de Oliveira, Felipe L; Gatto, Mariele; Bassi, Nicola; Luisetto, Roberto; Ghirardello, Anna; Punzi, Leonardo; Doria, Andrea

    2015-08-01

    Galectin-3 (gal-3) is a β-galactoside-binding lectin, which regulates cell-cell and extracellular interactions during self/non-self-antigen recognition and cellular activation, proliferation, differentiation, migration and apoptosis. It plays a significant role in cellular and tissue pathophysiology by organizing niches that drive inflammation and immune responses. Gal-3 has some therapeutic potential in several diseases, including chronic inflammatory disorders, cancer and autoimmune diseases. Gal-3 exerts a broad spectrum of functions which differs according to its intra- or extracellular localization. Recombinant gal-3 strategy has been used to identify potential mode of action of gal-3; however, exogenous gal-3 may not reproduce the functions of the endogenous gal-3. Notably, gal-3 induces monocyte-macrophage differentiation, interferes with dendritic cell fate decision, regulates apoptosis on T lymphocytes and inhibits B-lymphocyte differentiation into immunoglobulin secreting plasma cells. Considering the influence of these cell populations in the pathogenesis of several autoimmune diseases, gal-3 seems to play a role in development of autoimmunity. Gal-3 has been suggested as a potential therapeutic agent in patients affected with some autoimmune disorders. However, the precise role of gal-3 in driving the inflammatory process in autoimmune or immune-mediated disorders remains elusive. Here, we reviewed the involvement of gal-3 in cellular and tissue events during autoimmune and immune-mediated inflammatory diseases.

  7. Mast cells are important modifiers of autoimmune disease: With so much evidence, why is there controversy?

    Directory of Open Access Journals (Sweden)

    Melissa Ann Brown

    2012-06-01

    Full Text Available There is abundant evidence that mast cells are active participants in events that mediate tissue damage in autoimmune disease. Disease-associated increases in mast cell numbers accompanied by mast cell degranulation and elaboration of numerous mast cell mediators at sites of inflammation are commonly observed in many human autoimmune diseases including multiple sclerosis, rheumatoid arthritis and bullous pemphigoid. In animal models, treatment with mast cell stabilizing drugs or mast cell ablation can result in diminished disease. A variety of receptors including those engaged by antibody, complement, pathogens and intrinsic danger signals are implicated in mast cell activation in disease. Similar to their role as first responders in infection settings, mast cells likely orchestrate early recruitment of immune cells, including neutrophils, to the sites of autoimmune destruction. This co-localization promotes cellular crosstalk and activation and results in the amplification of the local inflammatory response thereby promoting and sustaining tissue damage. Despite the evidence, there is still a debate regarding the relative role of mast cells in these processes. However, by definition, mast cells can only act as accessory cells to the self-reactive T and/or antibody driven autoimmune responses. Thus, when evaluating mast cell involvement using existing and somewhat imperfect animal models of disease, their importance is sometimes obscured. However, these potent immune cells are undoubtedly major contributors to autoimmunity and should be considered as important targets for therapeutic disease intervention.

  8. Autoimmune pancreatitis: A review

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Autoimmune pancreatitis has emerged over the last 40 years from a proposed concept to a well established and recognized entity. As an efficient mimicker of pancreatic carcinoma, its early and appropriate recognition are crucial. With mounting understanding of its pathogenesis and natural history, significant advances have been made in the diagnosis of autoimmune pancreatitis. The characteristic laboratory features and imaging seen in autoimmune pancreatitis are reviewed along with some of the proposed diagnostic criteria and treatment algorithms.

  9. Dermatoses pediátricas no Hospital das Clínicas da Universidade Federal de Pernambuco Pediatric dermatoses at the Clinicas Hospital, Federal University of Pernambuco

    Directory of Open Access Journals (Sweden)

    Josemir Belo dos Santos

    2004-05-01

    Full Text Available FUNDAMENTOS: Numerosas condições dermatológicas podem afetar a população pediátrica. Há poucos estudos sobre a dermatologia pediátrica no Brasil. OBJETIVOS: Estudar as dermatoses pediátricas no Hospital das Clínicas do Recife, Pernambuco - Brasil. MATERIAL E MÉTODOS: Durante o período de maio de 1999 a maio de 2000, foram analisados 307 prontuários de crianças atendidas no Ambulatório de Dermatologia da Universidade Federal de Pernambuco. Foram encontrados os dados referentes a caracterização individual, procedência, motivo da consulta, diagnóstico clínico laboratorial e tratamentos previamente realizados. RESULTADOS: As dermatoses alérgicas foram as mais freqüentes (17,64%, seguidas pelas desordens pigmentares (15,54% e infecções virais (13,44%. CONCLUSÃO: Os dados encontrados estão de acordo com a literatura. Quanto às infecções, representam um grande percentual dos casos analisados, devendo-se isso provavelmente ao baixo nível socioeconômico dos pacientes em estudo. A caracterização da epidemiologia dos problemas cutâneos em crianças deve ser uma prioridade de estudos futuros.BACKGROUND: Numerous skin conditions can affect the pediatric population. There are few studies on Pediatric Dermatology in Brazil. OBJECTIVES: To study the pediatric skin diseases at the Hospital das Clínicas in Recife, Pernambuco State - Brazil. MATERIAL E METHODS: From May 1999 to May 2000, 307 cases of children treated at the Dermatology Clinic of the Federal University of Pernambuco were studied. Details of the patients' features, origin, reason for consultation, clinical laboratory diagnosis and previous treatment were examined. RESULTS: Allergic dermatosis was the most frequent disease (17.64%, followed by disorders of pigmentation (15.54% and viral diseases (13.44%. CONCLUSION: This agrees with the literature. Infections represent a large percentage of cases studied and are probably due to the low socioeconomic level of the

  10. [The early detection of allergic dermatoses and mycoses of the feet in workers in contact with phenol-formaldehyde resins].

    Science.gov (United States)

    Bannikov, E A; Anton'ev, A A; Makarova, L E; Beker, V P; Kiseleva, L L

    1990-01-01

    Hypersensitivity to phenolformaldehyde resins was detected in 16.2% of workers exposed to them, allergic dermatitis and eczemas in 21.5%, mycoses of the soles in 13.5%, premorbid shifts in half of the examinees. Mycoses of the soles prevailed in subjects with a history of dermatitis and in patients suffering from allergic dermatoses.

  11. Non-Venereal Dermatoses In Male Genital Region-Prevalence And Patterns In A Referral Centre In South India

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    Karthikeyan K

    2001-01-01

    Full Text Available A series of 100 male patients with non-venereal dermatoses of external genitalia were screened amongst patients visiting Dermatology OPD of JIPMER, Pondicherry from Aug ’97 to March ’99. The overall prevalence was found to be 14.1 per 10,000. Non-venereal dermatoses were common in the 21-40 years age group. Most of the patients (74% belonged to labourer class. A total of 25 different non-venereal dermatoses were studied. Genital vitiligo was the most common disorder accounting for 16 cases. Sebaceous cyst of the scrotum was present 13 patients. Among infections and infestations, scabies was observed in 9 patients. Ariboflavinosis was seen in 9 cases. Other disorders encountered were calcinosis scrotum. Iymphangiectasia of the scrotum. Lichen simplex chronicus. Fixed drug eruption, angiokeratoma of Fordyce, lichen sclerosus et atrophicus etc. The study has been quite useful in understanding the clinical and aetiological characteristics of various types of non-veneral dermatoses in males in this subcontinen of Asia.

  12. Effect of Auricular Acupuncture with Low Power Laser on Four Chronic Allergic Dermatoses and Serum IgE Level

    Institute of Scientific and Technical Information of China (English)

    You-hong Hou; Fang Xu; Shao-xi Wu

    2005-01-01

    @@ The objective of the study was to investigate the effectiveness of low power laser irradiating auricular points on four chronic allergic dermatoses including eczema, urticaria, facial cosmetic dermatitis, and atopic dermatitis, and on the changes of serum IgE level.

  13. [Syndrome overlap: autoimmune hepatitis and autoimmune cholangitis].

    Science.gov (United States)

    Guerra Montero, Luis; Ortega Alvarez, Félix; Marquez Teves, Maguin; Asato Higa, Carmen; Sumire Umeres, Julia

    2016-01-01

    Autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis and autoimmune cholangitis are chronic autoimmune liver disease, usually present separate, the cases where characteristics of two of the above is observed liver disease is commonly referred to as Overlap Syndromes (OS). Although there is no consensus on specific criteria for the diagnosis of OS identification of this association is important for initiating appropriate treatment and prevent its progression to cirrhosis or at least the complications of cirrhosis and death. We report the case of awoman aged 22 cirrhotic which debuted are edematous ascites, severe asthenia and jaundice compliant diagnostics SS criteria and initially present any response to treatment with ursodeoxycholic acid and oral corticosteroids, but ultimately finished performing a transplant orthotopic liver.

  14. Update in endocrine autoimmunity.

    Science.gov (United States)

    Anderson, Mark S

    2008-10-01

    The endocrine system is a common target in pathogenic autoimmune responses, and there has been recent progress in our understanding, diagnosis, and treatment of autoimmune endocrine diseases. Rapid progress has recently been made in our understanding of the genetic factors involved in endocrine autoimmune diseases. Studies on monogenic autoimmune diseases that include endocrine phenotypes like autoimmune polyglandular syndrome type 1 and immune dysregulation, polyendocrinopathy, enteropathy, X-linked have helped reveal the role of key regulators in the maintenance of immune tolerance. Highly powered genetic studies have found and confirmed many new genes outside of the established role of the human leukocyte antigen locus with these diseases, and indicate an essential role of immune response pathways in these diseases. Progress has also been made in identifying new autoantigens and the development of new animal models for the study of endocrine autoimmunity. Finally, although hormone replacement therapy is still likely to be a mainstay of treatment in these disorders, there are new agents being tested for potentially treating and reversing the underlying autoimmune process. Although autoimmune endocrine disorders are complex in etiology, these recent advances should help contribute to improved outcomes for patients with, or at risk for, these disorders.

  15. THE AUTOIMMUNE ECOLOGY.

    Directory of Open Access Journals (Sweden)

    Juan-Manuel eAnaya

    2016-04-01

    Full Text Available Autoimmune diseases (ADs represent a heterogeneous group of disorders that affect specific target organs or multiple organ systems. These conditions share common immunopathogenic mechanisms (i.e., the autoimmune tautology, which explain the clinical similarities they have among them as well as their familial clustering (i.e., coaggregation. As part of the autoimmune tautology, the influence of environmental exposure on the risk of developing ADs is paramount (i.e., the autoimmune ecology. In fact, environment, more than genetics, shapes immune system. Autoimmune ecology is akin to exposome, that is all the exposures - internal and external - across the lifespan, interacting with hereditary factors (both genetics and epigenetics to favor or protect against autoimmunity and its outcomes. Herein we provide an overview of the autoimmune ecology, focusing on the immune response to environmental agents in general, and microbiota, cigarette smoking, alcohol and coffee consumption, socioeconomic status, gender and sex hormones, vitamin D, organic solvents and vaccines in particular. Inclusion of the autoimmune ecology in disease etiology and health will improve the way personalized medicine is currently conceived and applied.

  16. Bistability in autoimmune diseases

    DEFF Research Database (Denmark)

    Rapin, Nicolas; Mosekilde, Erik; Lund, Ole

    2011-01-01

    Autoimmune diseases damage host tissue, which, in turn, may trigger a stronger immune response. Systems characterized by such positive feedback loops can display co-existing stable steady states. In a mathematical model of autoimmune disease, one steady state may correspond to the healthy state...

  17. The Autoimmune Ecology.

    Science.gov (United States)

    Anaya, Juan-Manuel; Ramirez-Santana, Carolina; Alzate, Maria A; Molano-Gonzalez, Nicolas; Rojas-Villarraga, Adriana

    2016-01-01

    Autoimmune diseases (ADs) represent a heterogeneous group of disorders that affect specific target organs or multiple organ systems. These conditions share common immunopathogenic mechanisms (i.e., the autoimmune tautology), which explain the clinical similarities they have among them as well as their familial clustering (i.e., coaggregation). As part of the autoimmune tautology, the influence of environmental exposure on the risk of developing ADs is paramount (i.e., the autoimmune ecology). In fact, environment, more than genetics, shapes immune system. Autoimmune ecology is akin to exposome, that is all the exposures - internal and external - across the lifespan, interacting with hereditary factors (both genetics and epigenetics) to favor or protect against autoimmunity and its outcomes. Herein, we provide an overview of the autoimmune ecology, focusing on the immune response to environmental agents in general, and microbiota, cigarette smoking, alcohol and coffee consumption, socioeconomic status (SES), gender and sex hormones, vitamin D, organic solvents, and vaccines in particular. Inclusion of the autoimmune ecology in disease etiology and health will improve the way personalized medicine is currently conceived and applied.

  18. Frequency of apoptotic keratinocytes in the feline epidermis: a retrospective light-microscopic study of skin-biopsy specimens from 327 cats with normal skin or inflammatory dermatoses.

    Science.gov (United States)

    Vogel, Jeff W; Scott, Danny W; Erb, Hollis N

    2009-12-01

    A retrospective light-microscopic study was performed on 294 biopsy specimens of haired skin from cats with various feline inflammatory dermatoses and specimens from cats with normal skin. Conditions expected to frequently have apoptotic epidermal keratinocytes (AKs) (including erythema multiforme, systemic lupus erythematosus, thymoma-associated exfoliative dermatitis, solar dermatitis, and viral dermatopathies) were found to have significantly more AKs than other types of inflammatory dermatoses. Nevertheless, we found more than two AKs in many skin-biopsy specimens from inflammatory conditions not expected to have frequent AKs (especially those from ectoparasitic dermatoses). Only a single AK was found in 1/33 cats with normal skin.

  19. Prevalência de dermatoses em idosos residentes em instituição de longa permanência Prevalence of dermatoses in residents of institutions for the elderly

    Directory of Open Access Journals (Sweden)

    Sandra Lopes Mattos e Dinato

    2008-12-01

    Full Text Available OBJETIVOS: Determinar a prevalência das afecções dermatológicas em 75 idosos residentes em instituição de longa permanência na cidade de Santos-SP, e confrontar os achados com revisão da literatura. MÉTODOS: Setenta e cinco idosos foram submetidos a exame dermatológico. Com os dados obtidos calculou-se a prevalência, o número médio por paciente e a freqüência de dermatoses. Foi ainda analisado a distribuição nos subgrupos sexo, raça, faixa etária e nos subgrupos principais de dermatoses. RESULTADOS: O número total de dermatoses diagnosticadas foi 280, o número médio por paciente foi 3,73 e o número total de tipos de dermatoses foi 32. A prevalência das principais dermatoses encontradas foi: melanose solar (53,3%, queratose seborréica (46,6%, onicomicose (37,3%, nevo rubi (33,3%, púrpura senil (29,3%, xerose (14,6%, escabiose (12,0% e neoplasia maligna (1,3%. A distribuição das dermatoses foi 70% (IC95%: 59% a 81% maior no sexo feminino do que no masculino. CONCLUSÃO: Apesar da pequena casuística, os dados obtidos são compatíveis com os poucos relatos disponíveis na literatura. Este estudo de prevalência definiu a distribuição de dermatoses em uma fração da população de idosos. Entretanto, os dados agregados de casuísticas subseqüentes podem permitir tornar os dados mais precisos, cuja relevância é indiscutível na orientação de medidas de saúde individual e coletiva.OBJECTIVE: To determine the prevalence of dermatoses in 75 elderly residents in an institution of long permanence in the city of Santos, and to compare findings with those in literature. METHODS: Seventy five healthy elderly persons were submitted to a dermatological exam. The prevalence, the average number per elderly person and the frequency of dermatoses were calculated from the data collected. An analysis was then made of the distribution in the subgroups of, gender, race, age bracket as well as of dermatosis in the main sub

  20. Autoimmune Cholangitis: A Variant Syndrome of Autoimmune Hepatitis

    OpenAIRE

    Brij Sharma; Sujeet Raina; Rajesh Sharma

    2014-01-01

    Autoimmune cholangitis (AIC) or autoimmune cholangiopathy is a chronic inflammation of liver and a variant syndrome of autoimmune hepatitis (AIH). We present a case of an adult female who had biochemical features of cholestasis and transaminasemia but aminotransferases were not in the hepatitis range and had histological evidence of bile duct injury which was subsequently diagnosed as autoimmune cholangitis.

  1. Consequences of occupational food-related hand dermatoses with a focus on protein contact dermatitis

    DEFF Research Database (Denmark)

    Vester, Lotte; Thyssen, Jacob P; Menné, Torkil

    2012-01-01

    Background. Protein contact dermatitis is a frequent disorder among hand eczema patients who have occupational food contact. Knowledge about the consequences of having protein contact dermatitis is lacking. Objectives. To investigate the consequences of having occupational skin disease on the hands...... resulting from food handling, with a focus on protein contact dermatitis. Material and methods. One hundred and seventy-eight patients who were identified as having skin disease related to occupational food exposure and who answered a questionnaire concerning the consequences of their skin disease were......%, respectively, of the patients with other occupational food-related hand dermatoses (p = 0.02). Sixty-two per cent and 43%, respectively, had to change job because of skin problems (p = 0.02). Atopic dermatitis was equally common in the two groups. Conclusion. We found that the patients with protein contact...

  2. Autosomal recessive chronic granulomatous disease presenting with cutaneous dermatoses and ocular infection.

    Science.gov (United States)

    Low, L C M; Manson, A L; Hardman, C; Carton, J; Seneviratne, S L; Ninis, N

    2013-04-01

    Dermatoses such as eczematous dermatitis and cutaneous infection are recognized presentations of primary immunodeficiency (PID). However, atopic dermatitis affects approximately 10% of infants, and cutaneous infections are not uncommon in children, therefore the challenge for the dermatologist is to distinguish the few patients that have PID from the many that do not. We report on a 6-year-old girl who was ultimately diagnosed with autosomal recessive chronic granulomatous disease (AR-CGD) after presenting to various hospitals with dermatitis, scalp plaques recalcitrant to treatment, and recurrent infections over a 3-year period, and describe some aspects of her diagnosis and management. This report highlights the importance of considering rare disorders such as AR-CGD in the differential diagnosis of recurrent or recalcitrant dermatological infections in children.

  3. Interstitial pneumonia associated with linear immunoglobulin A/immunoglobulin G bullous dermatosis.

    Science.gov (United States)

    Kakugawa, Tomoyuki; Tomimura, Saori; Hayashi, Tomayoshi; Sakamoto, Noriho; Ishimatsu, Yuji; Mukae, Hiroshi; Kohno, Shigeru

    2013-01-01

    A 76-year-old man with interstitial lung disease was admitted to our institution after developing persistent dyspnea upon effort. He also had a relapse of bullous eruptions on the skin of the trunk and extremities, previously diagnosed as vesicular pemphigoid. Direct immunofluorescence of a skin biopsy specimen using fluorescence microscopy showed the linear deposition of immunglobulin A (IgA), IgG and C3 along the basement membrane. These findings indicated a definitive diagnosis of linear IgA/IgG bullous dermatosis. Chest computed tomography, bronchoalveolar lavage and transbronchial lung biopsy findings suggested nonspecific interstitial pneumonia. Direct immunofluorescence of the lung biopsy specimens using fluorescence microscopy also showed a deposition of IgA, IgG and C3 along the epithelial cell membranes and basement membranes of the bronchioles and alveoli. Lung disorders associated with linear IgA/IgG bullous dermatosis are extremely rare and, to our knowledge, this is the first report of such a case of interstitial pneumonia.

  4. Immunometabolism and autoimmunity.

    Science.gov (United States)

    Freitag, Jenny; Berod, Luciana; Kamradt, Thomas; Sparwasser, Tim

    2016-11-01

    A continuous increase in the prevalence of autoimmune diseases is to be expected in the aging societies worldwide. Autoimmune disorders not only cause severe disability and chronic pain, but also lead to considerable socio-economic costs. Given that the current treatment options are not curative, have substantial side effects and a high percentage of non-responders, innovative options to the existing therapeutic armament against autoimmune diseases are urgently required. Accumulating evidence suggests that changes in the metabolism of immune cells are associated with, and contribute to the pathogenesis of autoimmunity. Additionally, some autoimmune diseases share alterations in metabolic pathways, key metabolites or metabolic byproducts such as reactive oxygen species. Other examples for metabolic changes in autoimmune settings include modifications in amino acid and cholesterol levels or glucose catabolism. Thus, the emerging field of immunometabolism may hold the potential to discover new therapeutic targets. Here, we discuss recent findings describing metabolic changes in autoimmune arthritis, multiple sclerosis as well as type 1 diabetes, focusing on pathophysiological aspects.

  5. Psoriasis and autoimmunity.

    Science.gov (United States)

    Sticherling, Michael

    2016-12-01

    Psoriasis is one of the most common chronic inflammatory human skin diseases. Though clinically well characterized, the exact etiological and pathogenic mechanisms are still not known in detail. Current knowledge indicates distinct overlap to other inflammatory as well as autoimmune disorders. However, the one or more relevant autoantigens could not be characterized so-far. On the other side, several autoimmune diseases were shown to be associated with psoriasis. In addition, serological autoimmune phenomena, namely diverse circulating specific autoantibodies could be demonstrated in the past. A matter of current debate is if psoriasis is a primary autoimmune disease or secondarily evolving into autoimmunity as seen in other chronic inflammatory diseases. Related to this aspect is the concept of autoinflammation versus autoimmunity where psoriasis shares mechanisms of both entities. Though T-cells remain among the most important cellular players in the pathogenesis of psoriasis and current therapeutic strategies successfully target these cells or their products irrespective of these concepts, autoimmunity if relevant will add to the treatment armamentarium by using protective and prophylactic antigen-specific modalities.

  6. Autoimmune autonomic disorders.

    Science.gov (United States)

    Mckeon, Andrew; Benarroch, Eduardo E

    2016-01-01

    Autoimmune autonomic disorders occur because of an immune response directed against sympathetic, parasympathetic, and enteric ganglia, autonomic nerves, or central autonomic pathways. In general, peripheral autoimmune disorders manifest with either generalized or restricted autonomic failure, whereas central autoimmune disorders manifest primarily with autonomic hyperactivity. Some autonomic disorders are generalized, and others are limited in their anatomic extent, e.g., isolated gastrointestinal dysmotility. Historically, these disorders were poorly recognized, and thought to be neurodegenerative. Over the last 20 years a number of autoantibody biomarkers have been discovered that have enabled the identification of certain patients as having an autoimmune basis for either autonomic failure or hyperactivity. Peripheral autoimmune autonomic disorders include autoimmune autonomic ganglionopathy (AAG), paraneoplastic autonomic neuropathy, and acute autonomic and sensory neuropathy. AAG manifests with acute or subacute onset of generalized or selective autonomic failure. Antibody targeting the α3 subunit of the ganglionic-type nicotinic acetylcholine receptor (α3gAChR) is detected in approximately 50% of cases of AAG. Some other disorders are characterized immunologically by paraneoplastic antibodies with a high positive predictive value for cancer, such as antineuronal nuclear antibody, type 1 (ANNA-1: anti-Hu); others still are seronegative. Recognition of an autoimmune basis for autonomic disorders is important, as their manifestations are disabling, may reflect an underlying neoplasm, and have the potential to improve with a combination of symptomatic and immune therapies.

  7. Liquen escleroso extragenital ampollar y hemorrágico: a propósito de un caso Extra-genital bullous and haemorrhagic lichen sclerosus: a case report

    Directory of Open Access Journals (Sweden)

    JM López Bertrán

    2010-06-01

    Full Text Available El liquen escleroso (LE es una enfermedad inflamatoria, crónica y benigna. De etiología desconocida, se ha vinculado con diversos factores autoinmunológicos, genéticos, hormonales, infecciosos y traumáticos. Hay dos variedades clínicas de esta enfermedad: genital y extra-genital. La localización más frecuente en ambos sexos es la genital. Se presenta un caso clínico de LE extra-genital, con lesiones cutáneas poco frecuentes, ampollares y hemorrágicas, que coexistían con placas atróficas de larga evolución, no diagnosticadas previamente.Lichen sclerosus is an inflammatory disease, benign, chronic, of unknown aetiology. It has been linked with many factors such as autoimmune, genetic, hormonal and infectious diseases, as well as trauma. There are two clinical types of this disease: genital and extra-genital. The most frequent in both sexes is the genital form. We present a rare case of extra-genital localization with bullous and haemorrhagic manifestations that co-existed with late atrophic plaques, previously undiagnosed.

  8. Association study of Demodex bacteria and facial dermatoses based on DGGE technique.

    Science.gov (United States)

    Zhao, YaE; Yang, Fan; Wang, RuiLing; Niu, DongLing; Mu, Xin; Yang, Rui; Hu, Li

    2017-03-01

    The role of bacteria is unclear in the facial skin lesions caused by Demodex. To shed some light on this issue, we conducted a case-control study comparing cases with facial dermatoses with controls with healthy skin using denaturing gradient gel electrophoresis (DGGE) technique. The bacterial diversity, composition, and principal component were analyzed for Demodex bacteria and the matched facial skin bacteria. The result of mite examination showed that all 33 cases were infected with Demodex folliculorum (D. f), whereas 16 out of the 30 controls were infected with D. f, and the remaining 14 controls were infected with Demodex brevis (D. b). The diversity analysis showed that only evenness index presented statistical difference between mite bacteria and matched skin bacteria in the cases. The composition analysis showed that the DGGE bands of cases and controls were assigned to 12 taxa of 4 phyla, including Proteobacteria (39.37-52.78%), Firmicutes (2.7-26.77%), Actinobacteria (0-5.71%), and Bacteroidetes (0-2.08%). In cases, the proportion of Staphylococcus in Firmicutes was significantly higher than that in D. f controls and D. b controls, while the proportion of Sphingomonas in Proteobacteria was significantly lower than that in D. f controls. The between-group analysis (BGA) showed that all the banding patterns clustered into three groups, namely, D. f cases, D. f controls, and D. b controls. Our study suggests that the bacteria in Demodex should come from the matched facial skin bacteria. Proteobacteria and Firmicutes are the two main taxa. The increase of Staphylococcus and decrease of Sphingomonas might be associated with the development of facial dermatoses.

  9. Autoimmunity in visual loss.

    Science.gov (United States)

    Petzold, Axel; Wong, Sui; Plant, Gordon T

    2016-01-01

    There are a number of autoimmune disorders which can affect visual function. There are a very large number of mechanisms in the visual pathway which could potentially be the targets of autoimmune attack. In practice it is the retina and the anterior visual pathway (optic nerve and chiasm) that are recognised as being affected in autoimmune disorders. Multiple Sclerosis is one of the commonest causes of visual loss in young adults because of the frequency of attacks of optic neuritis in that condition, however the basis of the inflammation in Multiple Sclerosis and the confirmation of autoimmunity is lacking. The immune process is known to be highly unusual in that it is not systemic and confined to the CNS compartment. Previously an enigmatic partner to Multiple Sclerosis, Neuromyelitis Optica is now established to be autoimmune and two antibodies - to Aquaporin4 and to Myelin Oligodendrocyte Glycoprotein - have been implicated in the pathogenesis. The term Chronic Relapsing Inflammatory Optic Neuropathy is applied to those cases of optic neuritis which require long term immunosuppression and hence are presumed to be autoimmune but where no autoimmune pathogenesis has been confirmed. Optic neuritis occurring post-infection and post vaccination and conditions such as Systemic Lupus Erythematosus and various vasculitides may cause direct autoimmune attack to visual structures or indirect damage through occlusive vasculopathy. Chronic granulomatous disorders such as Sarcoidosis affect vision commonly by a variety of mechanisms, whether and how these are placed in the autoimmune panoply is unknown. As far as the retina is concerned Cancer Associated Retinopathy and Melanoma Associated Retinopathy are well characterised clinically but a candidate autoantibody (recoverin) is only described in the former disorder. Other, usually monophasic, focal retinal inflammatory disorders (Idiopathic Big Blind Spot Syndrome, Acute Zonal Occult Outer Retinopathy and Acute Macular

  10. [Non-autoimmune thyroiditis].

    Science.gov (United States)

    Rizzo, Leonardo F L; Mana, Daniela L; Bruno, Oscar D

    2014-01-01

    The term thyroiditis comprises a group of thyroid diseases characterized by the presence of inflammation, including autoimmune and non-autoimmune entities. It may manifest as an acute illness with severe thyroid pain (subacute thyroiditis and infectious thyroiditis), and conditions in which the inflammation is not clinically evident evolving without pain and presenting primarily thyroid dysfunction and/or goiter (drug-induced thyroiditis and Riedel thyroiditis). The aim of this review is to provide an updated approach on non-autoimmune thyroiditis and its clinical, diagnostic and therapeutic aspects.

  11. Autoimmunity in Immunodeficiency

    Science.gov (United States)

    Todoric, Krista; Koontz, Jessica B.; Mattox, Daniel; Tarrant, Teresa K.

    2013-01-01

    Primary immunodeficiencies (PID) comprise a diverse group of clinical disorders with varied genetic defects. Paradoxically, a substantial proportion of PID patients develop autoimmune phenomena in addition to having increased susceptibility to infections from their impaired immunity. Although much of our understanding comes from data gathered through experimental models, there are several well-characterized PID that have improved our knowledge of the pathways that drive autoimmunity. The goals of this review will be to discuss these immunodeficiencies and to review the literature with respect to the proposed mechanisms for autoimmunity within each put forth to date. PMID:23591608

  12. Leptin and autoimmune disease.

    Science.gov (United States)

    Fujita, Yoshimasa

    2017-01-01

      Leptin is secreted from adipocytes and acts mainly on the hypothalamus causing weight loss due to suppression of appetite and increased energy expenditure. On the other hand, the leptin receptor is also expressed in hematopoietic cells and its action on the immune system has become known, and the significance of leptin in autoimmune diseases has gradually become clear. It has been shown that leptin acts as an exacerbating factor in many autoimmune diseases and it is suggested that inhibition of leptin signal may be a novel therapeutic method for autoimmune diseases. In this article, we will outline the significance of leptin in the immune system based on the current reports.

  13. Autoimmune-mediated peripheral neuropathies and autoimmune pain.

    Science.gov (United States)

    Klein, Christopher J

    2016-01-01

    Peripheral neuropathies have diverse acquired and inherited causes. The autoimmune neuropathies represent an important category where treatment is often available. There are overlapping signs and symptoms between autoimmune neuropathies and other forms. Making a diagnosis can be challenging and first assisted by electrophysiologic and sometimes pathologic sampling, with autoimmune biomarkers providing increased assistance. Here we provide a review of the autoimmune and inflammatory neuropathies, their available biomarkers, and approaches to treatment. Also discussed is new evidence to support a mechanism of autoimmune pain.

  14. Human IgG1 monoclonal antibody against human collagen 17 noncollagenous 16A domain induces blisters via complement activation in experimental bullous pemphigoid model.

    Science.gov (United States)

    Li, Qiang; Ujiie, Hideyuki; Shibaki, Akihiko; Wang, Gang; Moriuchi, Reine; Qiao, Hong-jiang; Morioka, Hiroshi; Shinkuma, Satoru; Natsuga, Ken; Long, Heather A; Nishie, Wataru; Shimizu, Hiroshi

    2010-12-15

    Bullous pemphigoid (BP) is an autoimmune blistering disease caused by IgG autoantibodies targeting the noncollagenous 16A (NC16A) domain of human collagen 17 (hCOL17), which triggers blister formation via complement activation. Previous in vitro analysis demonstrated that IgG1 autoantibodies showed much stronger pathogenic activity than IgG4 autoantibodies; however, the exact pathogenic role of IgG1 autoantibodies has not been fully demonstrated in vivo. We constructed a recombinant IgG1 mAb against hCOL17 NC16A from BP patients. In COL17-humanized mice, this mAb effectively reproduced a BP phenotype that included subepidermal blisters, deposition of IgG1, C1q and C3, neutrophil infiltration, and mast cell degranulation. Subsequently, alanine substitutions at various C1q binding sites were separately introduced to the Fc region of the IgG1 mAb. Among these mutated mAbs, the one that was mutated at the P331 residue completely failed to activate the complement in vitro and drastically lost pathogenic activity in COL17-humanized mice. These findings indicate that P331 is a key residue required for complement activation and that IgG1-dependent complement activation is essential for blister formation in BP. This study is, to our knowledge, the first direct evidence that IgG1 Abs to hCOL17 NC16A can induce blister formation in vivo, and it raises the possibility that IgG1 mAbs with Fc modification may be used to block pathogenic epitopes in autoimmune diseases.

  15. Autoimmunity against laminins.

    Science.gov (United States)

    Florea, Florina; Koch, Manuel; Hashimoto, Takashi; Sitaru, Cassian

    2016-09-01

    Laminins are ubiquitous constituents of the basement membranes with major architectural and functional role as supported by the fact that absence or mutations of laminins lead to either lethal or severely impairing phenotypes. Besides genetic defects, laminins are involved in a wide range of human diseases including cancer, infections, and inflammatory diseases, as well as autoimmune disorders. A growing body of evidence implicates several laminin chains as autoantigens in blistering skin diseases, collagenoses, vasculitis, or post-infectious autoimmunity. The current paper reviews the existing knowledge on autoimmunity against laminins referring to both experimental and clinical data, and on therapeutic implications of anti-laminin antibodies. Further investigation of relevant laminin epitopes in pathogenic autoimmunity would facilitate the development of appropriate diagnostic tools for thorough characterization of patients' antibody specificities and should decisively contribute to designing more specific therapeutic interventions.

  16. Autoimmune liver diseases

    Institute of Scientific and Technical Information of China (English)

    Pietro Invernizzi; Ian R Mackay

    2008-01-01

    The liver was one of the earliest recognized sites among autoimmune diseases yet autoimmune hepatitis,primary biliary cirrhosis,primary sclerosing cholangitis,and their overlap forms,are still problematic in diagnosis and causation.The contributions herein comprise 'pairs of articles' on clinical characteristics,and concepts of etiopathogenesis,for each of the above diseases,together with childhood autoimmune liver disease,overlaps,interpretations of diagnostic serology,and liver transplantation.This issue is timely,since we are witnessing an ever increasing applicability of immunology to a wide variety of chronic diseases,hepatic and non-hepatic,in both developed and developing countries.The 11 invited expert review articles capture the changing features over recent years of the autoimmune liver diseases,the underlying immunomolecular mechanisms of development,the potent albeit still unexplained genetic influences,the expanding repertoire of immunoserological diagnostic markers,and the increasingly effective therapeutic possibilities.

  17. Vaccines, adjuvants and autoimmunity.

    Science.gov (United States)

    Guimarães, Luísa Eça; Baker, Britain; Perricone, Carlo; Shoenfeld, Yehuda

    2015-10-01

    Vaccines and autoimmunity are linked fields. Vaccine efficacy is based on whether host immune response against an antigen can elicit a memory T-cell response over time. Although the described side effects thus far have been mostly transient and acute, vaccines are able to elicit the immune system towards an autoimmune reaction. The diagnosis of a definite autoimmune disease and the occurrence of fatal outcome post-vaccination have been less frequently reported. Since vaccines are given to previously healthy hosts, who may have never developed the disease had they not been immunized, adverse events should be carefully accessed and evaluated even if they represent a limited number of occurrences. In this review of the literature, there is evidence of vaccine-induced autoimmunity and adjuvant-induced autoimmunity in both experimental models as well as human patients. Adjuvants and infectious agents may exert their immune-enhancing effects through various functional activities, encompassed by the adjuvant effect. These mechanisms are shared by different conditions triggered by adjuvants leading to the autoimmune/inflammatory syndrome induced by adjuvants (ASIA syndrome). In conclusion, there are several case reports of autoimmune diseases following vaccines, however, due to the limited number of cases, the different classifications of symptoms and the long latency period of the diseases, every attempt for an epidemiological study has so far failed to deliver a connection. Despite this, efforts to unveil the connection between the triggering of the immune system by adjuvants and the development of autoimmune conditions should be undertaken. Vaccinomics is a field that may bring to light novel customized, personalized treatment approaches in the future. Copyright © 2015 Elsevier Ltd. All rights reserved.

  18. Autoimmunity in 2014.

    Science.gov (United States)

    Selmi, Carlo

    2015-10-01

    Our PubMed search for peer-reviewed articles published in the 2014 solar year retrieved a significantly higher number of hits compared to 2013 with a net 28 % increase. Importantly, full articles related to autoimmunity constitute approximately 5 % of immunology articles. We confirm that our understanding of autoimmunity is becoming a translational paradigm with pathogenetic elements rapidly followed by new treatment options. Furthermore, numerous clinical and pathogenetic elements and features are shared among autoimmune diseases, and this is well illustrated in the recent literature. More specifically, the past year witnessed critical revisions of our understanding and management of antiphospholipid syndrome with new exciting data on the pathogenicity of the serum anti-beta2 glycoprotein autoantibody, a better understanding of the current and new treatments for rheumatoid arthritis, and new position papers on important clinical questions such as vaccinations in patients with autoimmune disease, comorbidities, or new classification criteria. Furthermore, data confirming the important connections between innate immunity and autoimmunity via toll-like receptors or the critical role of T regulatory cells in tolerance breakdown and autoimmunity perpetuation were also reported. Lastly, genetic and epigenetic data were provided to confirm that the mosaic of autoimmunity warrants a susceptible individual background which may be geographically determined and contribute to the geoepidemiology of diseases. The 2014 literature in the autoimmunity world should be cumulatively regarded as part of an annus mirabilis in which, on a different level, the 2014 Annual Meeting of the American College of Rheumatology in Boston was attended by over 16,000 participants with over selected 3000 abstracts.

  19. Lithium associated autoimmune thyroiditis.

    OpenAIRE

    Shimizu, M; Hirokawa, M.; T. Manabe; Shimozuma, K; Sonoo, H; Harada, T.

    1997-01-01

    A case of autoimmune thyroiditis after long term treatment with lithium is described in a 29 year old Japanese woman with manic depression. Positive serum antithyroglobulin and antimicrosomal antibodies, diffuse goitre, and microscopic chronic thyroiditis, as well as the clinical history of long term lithium treatment were suggestive of lithium associated autoimmune thyroiditis. Microscopically, there was a mild degree of interstitial fibrosis and a moderate degree of lymphocytic infiltration...

  20. Perfil epidemiológico de infectados pelo vírus HIV com dermatoses em Natal/RN Epidemiologic profile of HIV - positive patients with dermatoses in Natal/RN /Brazil

    Directory of Open Access Journals (Sweden)

    Fernando Cardoso

    2003-02-01

    Full Text Available FUNDAMENTOS: Não há registro de dermatoses em infectados pelo vírus HIV no Rio Grande do Norte, embora haja 899 casos acumulados de AIDS em adultos entre janeiro de 1983 e agosto de 2000 e coeficientes de mortalidade por cem mil habitantes de 3,80 e 2,47, nos anos de 1995 e 1997, respectivamente, coincidindo essa diferença de valores, com o uso de antiretrovirais. OBJETIVOS: Descrever as características epidemiológicas de infectados, a freqüência de dermatoses e suas gravidades em usuários e não usuários de antiretrovirais. MÉTODOS: Foram incluídos 172 pacientes conforme a classificação do CDC/1992; descrevendo-se o uso de antiretrovirais e dermatoses neles presentes. RESULTADOS: A amostra teve 83,72% de homens, com média de idade de 37,17 anos, contaminados por via sexual (96,5%, heterossexuais predominantes (54,7%. As doenças cutâneas mais freqüentes foram as virais, fúngicas e miscelânia. O percentual médio da pele atingida pelas dermatoses foi 12,5%. A média da carga viral foi 109.114,05 cps/ml, e a de linfócitos T CD4+ foi 383,15 céls/mm³, estando 81,4% destes, em uso de antiretrovirais. CONCLUSÕES: O perfil epidemiológico dos infectados pelo vírus HIV no RN não difere das outras regiões brasileiras. Estudos analíticos que minimizem bias de confusão são necessários para aferir o grau de interferência do uso de antiretrovirais nesses indivíduos.BACKGROUND: There is no description of dermatoses among HIV infected individuals in Rio Grande do Norte(RN / Brazil, althoug this state had an accumulated total of 899 adult Aids cases registered from january 1983 to august 2000 and mortality coefficients per 100,000 residents of 3.80 and 2.47, in the years 1995 and 1997, respectively, such that this difference in values coincided with the introduction of antiretroviral (ARV usage in this state. OBJECTIVE: To describe epidemiologic caracteristics of retrovirus infected subjects with dermatoses, including frequency

  1. The epigenetics of autoimmunity

    Science.gov (United States)

    Meda, Francesca; Folci, Marco; Baccarelli, Andrea; Selmi, Carlo

    2011-01-01

    The etiology of autoimmune diseases remains largely unknown. Concordance rates in monozygotic twins are lower than 50% while genome-wide association studies propose numerous significant associations representing only a minority of patients. These lines of evidence strongly support other complementary mechanisms involved in the regulation of genes expression ultimately causing overt autoimmunity. Alterations in the post-translational modification of histones and DNA methylation are the two major epigenetic mechanisms that may potentially cause a breakdown of immune tolerance and the perpetuation of autoimmune diseases. In recent years, several studies both in clinical settings and experimental models proposed that the epigenome may hold the key to a better understanding of autoimmunity initiation and perpetuation. More specifically, data support the impact of epigenetic changes in systemic lupus erythematosus, rheumatoid arthritis, multiple sclerosis and other autoimmune diseases, in some cases based on mechanistical observations. We herein discuss what we currently know and what we expect will come in the next future. Ultimately, epigenetic treatments already being used in oncology may soon prove beneficial also in autoimmune diseases. PMID:21278766

  2. Autoimmunity in 2015.

    Science.gov (United States)

    Selmi, Carlo

    2016-08-01

    Compared to the clear trend observed in previous years, the number of peer-reviewed articles published during 2015 and retrieved using the "autoimmunity" key word declined by 4 %, while remaining 5 % of immunology articles. On the other hand, a more detailed analysis of the published articles in leading immunology and autoimmunity journals revealed exciting scenarios, with fascinating lines of evidence being supported by convincing data and likely followed by rapid translational or clinical developments. As examples, the study of the microbiome, the development of new serum or other tissue biomarkers, and a more solid understanding of disease pathogenesis and tolerance breakdown mechanisms have been central issues in the past year. Furthermore and similar to the oncology field, progress in the understanding of single autoimmune condition is becoming most specific with psoriatic and rheumatoid arthritis being ideal paradigms with treatment options diverging after decades of common therapies, as illustrated by IL17-targeting approaches. The ultimate result of these advances is towards personalized medicine with an ideal approach being tailored on a single patient, based on a finely tuned definition of the immunogenetics, epigenetics, microbiome, and biomarkers. Finally, experimental reports suggest that cancer-associated immune mechanisms or the role of T and B cell subpopulations should be better understood in autoimmune diseases. While we hailed the 2014 literature in the autoimmunity world as part of an annus mirabilis, we should not be mistaken in the strong stimulus of research in autoimmunity represented by the 2015 articles that will be summarized in this article.

  3. Autoimmunity in 2013.

    Science.gov (United States)

    Selmi, Carlo

    2014-08-01

    The peer-reviewed publications in the field of autoimmunity published in 2013 represented a significant proportion of immunology articles and grew since the previous year to indicate that more immune-mediated phenomena may recognize an autoimmune mechanism and illustrated by osteoarthritis and atherosclerosis. As a result, our understanding of the mechanisms of autoimmunity is becoming the paradigm for translational research in which the progress in disease pathogenesis for both tolerance breakdown and inflammation perpetuation is rapidly followed by new treatment approaches and clinical management changes. The similarities across the autoimmune disease spectrum outnumber differences, particularly when treatments are compared. Indeed, the therapeutics of autoimmune diseases are based on a growing armamentarium that currently includes monoclonal antibodies and small molecules which act by targeting molecular markers or intracellular mediators with high specificity. Among the over 100 conditions considered as autoimmune, the common grounds are well illustrated by the data reported for systemic lupus erythematosus and rheumatoid arthritis or by the plethora of studies on Th17 cells and biomarkers, particularly serum autoantibodies. Further, we are particularly intrigued by studies on the genomics, epigenetics, and microRNA at different stages of disease development or on the safe and effective use of abatacept acting on the costimulation of T and B cells in rheumatoid arthritis. We are convinced that the data published in 2013 represent a promising background for future developments that will exponentially impact the work of laboratory and clinical scientists over the next years.

  4. Vaccines and autoimmunity.

    Science.gov (United States)

    De Martino, M; Chiappini, E; Galli, L

    2013-01-01

    Vaccines have eradicated or controlled many infectious diseases, saving each year millions of lives and quality of life of many other millions of people. In spite of the success of vaccines over the last two centuries, parents (and also some health care workers) gloss over the devastating consequences of diseases, which are now avoided thanks to vaccines, and direct their attention to possible negative effects of immunization. Three immunological objections are raised: vaccines cause antigenic overload, natural immunity is safer and better than vaccine-induced immunity, and vaccines induce autoimmunity. The last point is examined in this review. Theoretically, vaccines could trigger autoimmunity by means of cytokine production, anti-idiotypic network, expression of human histocompatibility leukocyte antigens, modification of surface antigens and induction of novel antigens, molecular mimicry, bystander activation, epitope spreading, and polyclonal activation of B cells. There is strong evidence that none of these mechanisms is really effective in causing autoimmune diseases. Vaccines are not a source of autoimmune diseases. By contrast, absolute evidence exists that infectious agents can trigger autoimmune mechanisms and that they do cause autoimmune diseases.

  5. Human Eosinophils Express the High Affinity IgE Receptor, FcεRI, in Bullous Pemphigoid

    Science.gov (United States)

    Messingham, Kelly N.; Holahan, Heather M.; Frydman, Alexandra S.; Fullenkamp, Colleen; Srikantha, Rupasree; Fairley, Janet A.

    2014-01-01

    Bullous pemphigoid (BP) is an autoimmune blistering disease mediated by autoantibodies targeting BP180 (type XVII collagen). Patient sera and tissues typically have IgG and IgE autoantibodies and elevated eosinophil numbers. Although the pathogenicity of the IgE autoantibodies is established in BP, their contribution to the disease process is not well understood. Our aims were two-fold: 1) To establish the clinical relationships between total and BP180-specific IgE, eosinophilia and other markers of disease activity; and 2) To determine if eosinophils from BP patients express the high affinity IgE receptor, FcεRI, as a potential mechanism of action for IgE in BP. Our analysis of 48 untreated BP patients revealed a correlation between BP180 IgG and both BP180 IgE and peripheral eosinophil count. Additionally, we established a correlation between total IgE concentration and both BP180 IgE levels and eosinophil count. When only sera from patients (n = 16) with total IgE≥400 IU/ml were analyzed, BP180 IgG levels correlated with disease severity, BP230 IgG, total circulating IgE and BP180 IgE. Finally, peripheral eosinophil count correlated more strongly with levels of BP180 IgE then with BP180 IgG. Next, eosinophil FcεRI expression was investigated in the blood and skin using several methods. Peripheral eosinophils from BP patients expressed mRNA for all three chains (α, β and γ) of the FcεRI. Surface expression of the FcεRIα was confirmed on both peripheral and tissue eosinophils from most BP patients by immunostaining. Furthermore, using a proximity ligation assay, interaction of the α- and β-chains of the FcεRI was observed in some biopsy specimens, suggesting tissue expression of the trimeric receptor form in some patients. These studies provide clinical support for the relevance of IgE in BP disease and provide one mechanism of action of these antibodies, via binding to the FcεRI on eosinophils. PMID:25255430

  6. Human eosinophils express the high affinity IgE receptor, FcεRI, in bullous pemphigoid.

    Directory of Open Access Journals (Sweden)

    Kelly N Messingham

    Full Text Available Bullous pemphigoid (BP is an autoimmune blistering disease mediated by autoantibodies targeting BP180 (type XVII collagen. Patient sera and tissues typically have IgG and IgE autoantibodies and elevated eosinophil numbers. Although the pathogenicity of the IgE autoantibodies is established in BP, their contribution to the disease process is not well understood. Our aims were two-fold: 1 To establish the clinical relationships between total and BP180-specific IgE, eosinophilia and other markers of disease activity; and 2 To determine if eosinophils from BP patients express the high affinity IgE receptor, FcεRI, as a potential mechanism of action for IgE in BP. Our analysis of 48 untreated BP patients revealed a correlation between BP180 IgG and both BP180 IgE and peripheral eosinophil count. Additionally, we established a correlation between total IgE concentration and both BP180 IgE levels and eosinophil count. When only sera from patients (n = 16 with total IgE ≥ 400 IU/ml were analyzed, BP180 IgG levels correlated with disease severity, BP230 IgG, total circulating IgE and BP180 IgE. Finally, peripheral eosinophil count correlated more strongly with levels of BP180 IgE then with BP180 IgG. Next, eosinophil FcεRI expression was investigated in the blood and skin using several methods. Peripheral eosinophils from BP patients expressed mRNA for all three chains (α, β and γ of the FcεRI. Surface expression of the FcεRIα was confirmed on both peripheral and tissue eosinophils from most BP patients by immunostaining. Furthermore, using a proximity ligation assay, interaction of the α- and β-chains of the FcεRI was observed in some biopsy specimens, suggesting tissue expression of the trimeric receptor form in some patients. These studies provide clinical support for the relevance of IgE in BP disease and provide one mechanism of action of these antibodies, via binding to the FcεRI on eosinophils.

  7. Mast Cells are Important Modifiers of Autoimmune Disease: With so Much Evidence, Why is There Still Controversy?

    Science.gov (United States)

    Brown, Melissa A; Hatfield, Julianne K

    2012-01-01

    There is abundant evidence that mast cells are active participants in events that mediate tissue damage in autoimmune disease. Disease-associated increases in mast cell numbers accompanied by mast cell degranulation and elaboration of numerous mast cell mediators at sites of inflammation are commonly observed in many human autoimmune diseases including multiple sclerosis, rheumatoid arthritis, and bullous pemphigoid. In animal models, treatment with mast cell stabilizing drugs or mast cell ablation can result in diminished disease. A variety of receptors including those engaged by antibody, complement, pathogens, and intrinsic danger signals are implicated in mast cell activation in disease. Similar to their role as first responders in infection settings, mast cells likely orchestrate early recruitment of immune cells, including neutrophils, to the sites of autoimmune destruction. This co-localization promotes cellular crosstalk and activation and results in the amplification of the local inflammatory response thereby promoting and sustaining tissue damage. Despite the evidence, there is still a debate regarding the relative role of mast cells in these processes. However, by definition, mast cells can only act as accessory cells to the self-reactive T and/or antibody driven autoimmune responses. Thus, when evaluating mast cell involvement using existing and somewhat imperfect animal models of disease, their importance is sometimes obscured. However, these potent immune cells are undoubtedly major contributors to autoimmunity and should be considered as important targets for therapeutic disease intervention.

  8. A retrospective analysis of dermatoses in the perimenopausal population attending a tertiary care centre in South India

    Directory of Open Access Journals (Sweden)

    Shamma Aboobacker

    2015-01-01

    Conclusion: The leading dermatoses being eczema and urticaria in the perimenopausal population probably accounts for a tendency of exaggerated response to external factors. The population studied in the current study might be of significance due to complete lack of treatment in the form of hormone replacement therapy (HRT, while routine sun exposure and cultural practices predominate. However, evaluation with respect to individual factors is beyond the scope of the current study and may be necessary to define a causal relationship.

  9. Autoimmune thyroid disease and other non-endocrine autoimmune diseases

    Directory of Open Access Journals (Sweden)

    Todorović-Đilas Ljiljana

    2011-01-01

    Full Text Available Introduction, Autoimmune diseases are chronic conditions initiated by the loss of immunological tolerance to self-antigens. They constitute heterogeneous group of disorders, in which multiple alterations in the immune system result in a spectrum of syndromes that either target specific organs or affect the body systematically. Recent epidemiological studies have shown a possible shift of one autoimmune disease to another or the fact that more than one autoimmune disease may coexist in a single patient or in the same family. Numerous autoimmune diseases have been shown to coexist frequently with thyroid autoimmune diseases. Autoimmune thyroid disease and other organ specific non-endocrine autoimmune diseases. This part of the study reviews the prevalence of autoimmune thyroid disease coexisting with: pernicious anaemia, vitiligo, celiac disease, autoimmune liver disease, miastenia gravis, alopecia areata and sclerosis multiplex, and several recommendations for screening have been given. Autoimmune thyroid disease and other organ non-specific non-endocrine autoimmune diseases. Special attention is given to the correlation between autoimmune thyroid disease and rheumatoid arthritis, systemic lupus erythematosus, syndrome Sjögren, systemic sclerosis and mixed connective tissue disease. Conclusions. Screening for autoimmune thyroid diseases should be recommended in everyday clinical practice, in patients with primary organ-specific or organ non-specific autoimmune disease. Other­wise, in patients with primary thyroid autoimmune disease, there is no good reason of seeking for all other autoimmune diseases, although these patients have a greater risk of developing other autoimmune disease. Economic aspects of medicine require further analyzing of these data, from cost/benefit point of view to justified either mandatory screening or medical practitioner judgment.

  10. The mechanisms of action of phototherapy in the treatment of the most common dermatoses.

    Science.gov (United States)

    Bulat, Vedrana; Situm, Mirna; Dediol, Iva; Ljubicić, Ivana; Bradić, Lada

    2011-09-01

    Phototherapy denotes the use of ultraviolet (UV) light in the management of several dermatoses. Most phototherapy regimens utilize ultraviolet radiation of different wavelenghts. Currently, irradiations with broadband UVB (290-320 nm), narrowband UVB (311-313 nm), 308 nm excimer laser, UVA 1 (340-400 nm), UVA with psoralen (PUVA), and extracorporeal photochemotherapy (photopheresis) are being used. The interplay of the various photobiologic pathways is far from being completely understood. Disordes that may benefit from such approach are numerous, with psoriasis, atopic dermatitis, cutaneous T-cell lymphomas, morphea, and vitiligo as main indications. The immunomodulatory effects of UVB radiation primarily affect the epidermis and superficial dermis, while UVA radiation affects mid and deep dermal components, especially blood vessels. UVB radiation is absorbed by endogenous chromophores, such as nuclear DNA, which initiates a cascade of events. Absorption of UV light by nucleotides causes the formation of DNA photoproducts and suppresses DNA synthesis. In addition UV light stimulates synthesis of prostaglandins and cytokines that play important roles in immune suppression. It may reduce the number of Langerhans cells, cutaneous T lymphocytes and mast cells in the dermis. UV radiation can also affect extranuclear molecular targets located in the cytoplasm and cell membrane. Immune suppression, alteration in cytokine expression, and cell cycle arrest may all contribute to the suppression of disease activity. PUVA is a form of chemophototherapy which uses UVA light to activate chemicals known as psoralens, hence psoralen ultraviolet A. The conjunction of psoralens with epidermal DNA inhibits DNA replication and causes cell cycle arrest. Psoralen photosensitization also causes an alteration in the expression of cytokines and cytokine receptors. Psoralens interact with RNA, proteins and other cellular components and indirectly modify proteins and lipids via singlet

  11. Autoimmune gastritis: Pathologist's viewpoint.

    Science.gov (United States)

    Coati, Irene; Fassan, Matteo; Farinati, Fabio; Graham, David Y; Genta, Robert M; Rugge, Massimo

    2015-11-14

    Western countries are seeing a constant decline in the incidence of Helicobacter pylori-associated gastritis, coupled with a rising epidemiological and clinical impact of autoimmune gastritis. This latter gastropathy is due to autoimmune aggression targeting parietal cells through a complex interaction of auto-antibodies against the parietal cell proton pump and intrinsic factor, and sensitized T cells. Given the specific target of this aggression, autoimmune gastritis is typically restricted to the gastric corpus-fundus mucosa. In advanced cases, the oxyntic epithelia are replaced by atrophic (and metaplastic) mucosa, creating the phenotypic background in which both gastric neuroendocrine tumors and (intestinal-type) adenocarcinomas may develop. Despite improvements in our understanding of the phenotypic changes or cascades occurring in this autoimmune setting, no reliable biomarkers are available for identifying patients at higher risk of developing a gastric neoplasm. The standardization of autoimmune gastritis histology reports and classifications in diagnostic practice is a prerequisite for implementing definitive secondary prevention strategies based on multidisciplinary diagnostic approaches integrating endoscopy, serology, histology and molecular profiling.

  12. Vaccines and autoimmunity.

    Science.gov (United States)

    Agmon-Levin, Nancy; Paz, Ziv; Israeli, Eitan; Shoenfeld, Yehuda

    2009-11-01

    Vaccines have been used for over 200 years and are the most effective way of preventing the morbidity and mortality associated with infections. Like other drugs, vaccines can cause adverse events, but unlike conventional medicines, which are prescribed to people who are ill, vaccines are administered to healthy individuals, thus increasing the concern over adverse reactions. Most side effects attributed to vaccines are mild, acute and transient; however, rare reactions such as hypersensitivity, induction of infection, and autoimmunity do occur and can be severe and even fatal. The rarity and subacute presentation of post-vaccination autoimmune phenomena means that ascertaining causality between these events can be difficult. Moreover, the latency period between vaccination and autoimmunity ranges from days to years. In this article, on the basis of published evidence and our own experience, we discuss the various aspects of the causal and temporal interactions between vaccines and autoimmune phenomena, as well as the possible mechanisms by which different components of vaccines might induce autoimmunity.

  13. Complement and autoimmunity.

    Science.gov (United States)

    Ballanti, Eleonora; Perricone, Carlo; Greco, Elisabetta; Ballanti, Marta; Di Muzio, Gioia; Chimenti, Maria Sole; Perricone, Roberto

    2013-07-01

    The complement system is a component of the innate immune system. Its main function was initially believed to be limited to the recognition and elimination of pathogens through direct killing or stimulation of phagocytosis. However, in recent years, the immunoregulatory functions of the complement system were demonstrated and it was determined that the complement proteins play an important role in modulating adaptive immunity and in bridging innate and adaptive responses. When the delicate mechanisms that regulate this sophisticated enzymatic system are unbalanced, the complement system may cause damage, mediating tissue inflammation. Dysregulation of the complement system has been involved in the pathogenesis and clinical manifestations of several autoimmune diseases, such as systemic lupus erythematosus, vasculitides, Sjögren's syndrome, antiphospholipid syndrome, systemic sclerosis, dermatomyositis, and rheumatoid arthritis. Complement deficiencies have been associated with an increased risk to develop autoimmune disorders. Because of its functions, the complement system is an attractive therapeutic target for a wide range of diseases. Up to date, several compounds interfering with the complement cascade have been studied in experimental models for autoimmune diseases. The main therapeutic strategies are inhibition of complement activation components, inhibition of complement receptors, and inhibition of membrane attack complex. At present, none of the available agents was proven to be both safe and effective for treatment of autoimmune diseases in humans. Nonetheless, data from preclinical studies and initial clinical trials suggest that the modulation of the complement system could constitute a viable strategy for the treatment of autoimmune conditions in the decades to come.

  14. Autoimmunity and Asbestos Exposure

    Directory of Open Access Journals (Sweden)

    Jean C. Pfau

    2014-01-01

    Full Text Available Despite a body of evidence supporting an association between asbestos exposure and autoantibodies indicative of systemic autoimmunity, such as antinuclear antibodies (ANA, a strong epidemiological link has never been made to specific autoimmune diseases. This is in contrast with another silicate dust, crystalline silica, for which there is considerable evidence linking exposure to diseases such as systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis. Instead, the asbestos literature is heavily focused on cancer, including mesothelioma and pulmonary carcinoma. Possible contributing factors to the absence of a stronger epidemiological association between asbestos and autoimmune disease include (a a lack of statistical power due to relatively small or diffuse exposure cohorts, (b exposure misclassification, (c latency of clinical disease, (d mild or subclinical entities that remain undetected or masked by other pathologies, or (e effects that are specific to certain fiber types, so that analyses on mixed exposures do not reach statistical significance. This review summarizes epidemiological, animal model, and in vitro data related to asbestos exposures and autoimmunity. These combined data help build toward a better understanding of the fiber-associated factors contributing to immune dysfunction that may raise the risk of autoimmunity and the possible contribution to asbestos-related pulmonary disease.

  15. Pregnancy with autoimmune hepatitis

    Science.gov (United States)

    Braga, António Costa; Vasconcelos, Carlos; Braga, Jorge

    2016-01-01

    Aim: The aim of this study was to review our experience with gestations in autoimmune hepatitis patients. Background: There are only limited data describing pregnancy in patients with autoimmune hepatitis. Patients and methods: Retrospective analysis of pregnancies with autoimmune hepatitis followed in Centro Hospitalar do Porto, Portugal in the last ten years. Results: We reported nine pregnancies in seven patients with autoimmune hepatitis. Two patients had documented liver cirrhosis prior to the pregnancy. In this study, 66.7% of patients were treated with azathioprine and 88.9% with prednisolone. Clinical improvements were observed in 11.1% of pregnancies and 22.2% exacerbations were diagnosed. There were six live births and two preterm deliveries (preterm delivery rate of 33%). We also report three first trimester miscarriages (early gestation miscarriage rate of 33%). There were no neonatal or maternal deaths. Conclusion: The favorable obstetric outcome is a realistic expectation in patients with autoimmune hepatitis. Tight monitoring and control of asymptomatic and unpredictable exacerbations, which are unrelated to the severity of the underlying disease, are essential to the prognosis of the current pregnancy. PMID:27458515

  16. Autoimmune movement disorders.

    Science.gov (United States)

    Mckeon, Andrew; Vincent, Angela

    2016-01-01

    Autoimmune movement disorders encapsulate a large and diverse group of neurologic disorders occurring either in isolation or accompanying more diffuse autoimmune encephalitic illnesses. The full range of movement phenomena has been described and, as they often occur in adults, many of the presentations can mimic neurodegenerative disorders, such as Huntington disease. Disorders may be ataxic, hypokinetic (parkinsonism), or hyperkinetic (myoclonus, chorea, tics, and other dyskinetic disorders). The autoantibody targets are diverse and include neuronal surface proteins such as leucine-rich, glioma-inactivated 1 (LGI1) and glycine receptors, as well as antibodies (such as intracellular antigens) that are markers of a central nervous system process mediated by CD8+ cytotoxic T cells. However, there are two conditions, stiff-person syndrome (also known as stiff-man syndrome) and progressive encephalomyelitis with rigidity and myoclonus (PERM), that are always autoimmune movement disorders. In some instances (such as Purkinje cell cytoplasmic antibody-1 (PCA-1) autoimmunity), antibodies detected in serum and cerebrospinal fluid can be indicative of a paraneoplastic cause, and may direct the cancer search. In other instances (such as 65kDa isoform of glutamic acid decarboxylase (GAD65) autoimmunity), a paraneoplastic cause is very unlikely, and early treatment with immunotherapy may promote improvement or recovery. Here we describe the different types of movement disorder and the clinical features and antibodies associated with them, and discuss treatment.

  17. Bullous Pemphigoid With a Dual Pattern of Glomerular Immune Complex Disease.

    Science.gov (United States)

    Hoorn, Ewout J; Taams, Noor E; Hurskainen, Tiina; Salih, Mahdi; Weening, Jan J; Jonkman, Marcel F; Pas, Hendri H; Schreurs, Marco W J

    2016-02-01

    A 75-year-old man presented with a blistering skin disease and nephrotic syndrome. Bullous pemphigoid was diagnosed by linear immunoglobulin G (IgG) and C3 staining along the basement membrane zone of a skin biopsy specimen and by the presence of circulating IgG recognizing the 180-kDa bullous pemphigoid antigen (BP180; type XVII collagen). A kidney biopsy specimen showed endocapillary inflammation without crescents. Direct immunofluorescence showed strong IgG and C3 staining in a combined granular and linear pattern along the glomerular basement membrane. Electron microscopy showed subepithelial deposits. In serum, no antibodies against the Goodpasture antigen (type IV collagen) or phospholipase A2 receptor were detected. Indirect immunofluorescence studies using the patient's serum showed a strikingly linear but not granular IgG pattern along the epithelial basement membranes of monkey esophagus and kidney. Although type XVII collagen was recently identified in the glomerulus, the patient's serum did not produce a 180-kDa band on immunoblot of kidney tissue and still stained glomeruli of BP180 knockout mice by indirect immunofluorescence. The patient was treated with prednisone and azathioprine, which resulted in complete remission of skin and kidney manifestations. Although bullous pemphigoid has been reported previously in association with anti-glomerular basement membrane disease or membranous nephropathy, this case demonstrates both elements in 1 patient. This concurrence and the linear pattern on indirect immunofluorescence support the possibility of cross-reactive or parallel autoantibodies to basement membranes with a secondary membranous component. Copyright © 2016 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

  18. [Autoimmune thyroid disease and other non-endocrine autoimmune diseases].

    Science.gov (United States)

    Dilas, Ljiljana Todorović; Icin, Tijana; Paro, Jovanka Novaković; Bajkin, Ivana

    2011-01-01

    Autoimmune diseases are chronic conditions initiated by the loss of immunological tolerance to self-antigens. They constitute heterogeneous group of disorders, in which multiple alterations in the immune system result in a spectrum of syndromes that either target specific organs or affect the body systematically. Recent epidemiological studies have shown a possible shift of one autoimmune disease to another or the fact that more than one autoimmune disease may coexist in a single patient or in the same family. Numerous autoimmune diseases have been shown to coexist frequently with thyroid autoimmune diseases. AUTOIMMNUNE THYROID DISEASE AND OTHER ORGAN SPECIFIC NON-ENDOCRINE AUTOIMMUNE DISEASES: This part of the study reviews the prevalence of autoimmune thyroid disease coexisting with: pernicious anaemia, vitiligo, celiac disease, autoimmune liver disease, miastenia gravis, alopecia areata and sclerosis multiplex, and several recommendations for screening have been given. AUTOIMMUNE THYROID DISEASE AND OTHER ORGAN NON-SPECIFIC NON-ENDOCRINE AUTOIMMUNE DISEASES: Special attention is given to the correlation between autoimmune thyroid disease and rheumatoid arthritis, systemic lupus erythematosus, syndrome Sjögren, systemic sclerosis and mixed connective tissue disease. Screening for autoimmune thyroid diseases should be recommended in everyday clinical practice, in patients with primary organ-specific or organ non-specific autoimmune disease. Otherwise, in patients with primary thyroid autoimmune disease, there is no good reason of seeking for all other autoimmune diseases, although these patients have a greater risk of developing other autoimmune disease. Economic aspects of medicine require further analyzing of these data, from cost/benefit point of view to justified either mandatory screening or medical practitioner judgment.

  19. Microvesicles in Autoimmune Diseases.

    Science.gov (United States)

    Liu, M-L; Williams, K J; Werth, V P

    During apoptosis or activation, cells can release a subcellular structure, called a membrane microvesicle (also known as microparticle) into the extracellular environment. Microvesicles bud-off as a portion of cell membrane with its associated proteins and lipids surrounding a cytosolic core that contains intracellular proteins, lipids, and nucleic acids (DNA, RNA, siRNA, microRNA, lncRNA). Biologically active molecules on the microvesicle surface and encapsulated within can act on recipient cells as a novel mode of intercellular communication. Apoptosis has long been known to be involved in the development of diseases of autoimmunity. Abnormally persistent microvesicles, particularly apoptotic microvesicles, can accelerate autoimmune responses locally in specific organs and tissues as well as systemically. In this review, we focus on studies implicating microvesicles in the pathogenesis of autoimmune diseases and their complications. © 2016 Elsevier Inc. All rights reserved.

  20. Headache in autoimmune diseases.

    Science.gov (United States)

    John, Seby; Hajj-Ali, Rula A

    2014-03-01

    Autoimmune diseases are a group of heterogeneous inflammatory disorders characterized by systemic or localized inflammation, leading to ischemia and tissue destruction. These include disorders like systemic lupus erythematosus and related diseases, systemic vasculitides, and central nervous system (CNS) vasculitis (primary or secondary). Headache is a very common manifestation of CNS involvement of these diseases. Although headache characteristics can be unspecific and often non-diagnostic, it is important to recognize because headache can be the first manifestation of CNS involvement. Prompt recognition and treatment is necessary not only to treat the headache, but also to help prevent serious neurological sequelae that frequently accompany autoimmune diseases. In this review, we discuss headache associated with autoimmune diseases along with important mimics.

  1. Bullous Variant of Sweet’s Syndrome after Herpes Zoster Virus Infection

    OpenAIRE

    2011-01-01

    Aim: Cutaneous manifestations of Sweet’s syndrome (SS) are typically painful plaque-forming erythematous papules, while bullae are quite uncommon. We present a case of bullous variant of SS in acute myeloid leukaemia. In this case, herpes infection of the left mandible had preceded the development of SS. Case Report: A 75-year-old male with myelodysplastic syndrome first presented with herpes zoster virus infection-like bullae and erosive plaques on the left side of the face and neck. Treatme...

  2. Irritant bullous contact dermatitis caused by a rove beetle: an illustrated clinical course.

    Science.gov (United States)

    McGrath, Lindsay; Piliouras, Peter; Robertson, Ivan

    2013-05-01

    A 26-year-old Australian female traveller in Sierra Leone presented with an irritant bullous contact dermatitis consistent with paederus dermatitis. The lesions were treated with a potent topical corticosteroid with good effect. The affected area resolved in 6 weeks and hyperpigmention persisted for months until complete resolution. This dermatitis occurs when beetles of the genus Paederus (rove beetles) are crushed on the skin, releasing pederin. The same dermatitis ensues with Australian Paederus species. Serial clinical photographs are presented which will aid Australian dermatologists in the diagnosis of this dermatitis, which presents in regional Australian patients and returned overseas travellers.

  3. Bullous mycosis fungoides associated with an extensive ulcer and a severe leukemoid reaction

    Directory of Open Access Journals (Sweden)

    Shuei Sato

    2011-12-01

    Full Text Available This report presents a case of bullous mycosis fungoides associated with an extensive ulcer and a severe leukemoid reaction. The rash began as indurated erythema which was always followed by ulceration. The rashes initially responded to radiation therapy, but multiple recurrences appeared. Several bullae appeared on the trunk during the course of the illness, without any evidence of paraneoplastic pemphigus. Finally, the ulcer covered a large part of the trunk, and the patient died of sepsis with an extreme leukocyte count of 118,000/μL. A bone marrow analysis revealed a leukemoid reaction and an autopsy revealed pseudomembranous colitis.

  4. The results descemet-stripping automated endothelial keratoplasty at bullous keratopathy

    Directory of Open Access Journals (Sweden)

    S. V. Trufanov

    2012-01-01

    Full Text Available Purpose: to evaluate outcomes of the descemet-stripping automated endothelial keratoplasty using eye drops Kombinil-Duo dur- ing postoperative period.Methods: 26 patients suffered from bullous keratopathy underwent descemet-stripping automated endothelial keratoplasty using our own technique. We applied eye drops Kombinil-Duo during postoperative period.Results: 25 patients had clear corneas whole period of observation. Graft rejection occurred in 1 eye. Mean BSCVA was 0.47±0.21.Conclusion: Kombinil-Duo has been allowed to avoid infectious complication and to increase efficiency of the operative interven-tion during postoperative period.

  5. Amiodarone-induced exudative bullous lesion and hepatotoxicity in a patient with ventricular tachycardia

    Directory of Open Access Journals (Sweden)

    Ahmet Karakurt

    2015-09-01

    Full Text Available Amiodarone is a potent, iodine rich, highly lipophilic class III antiarrhythmic drug widely used for the management of both supraventricular and ventricular arrhythmias. It tends to concentrate in tissues including fat, lung, liver cornea and skin. Several side effects have been reported in patients taking amiodarone. The mechanisms of amiodarone-induced side effects are poorly understood. Accumulation of amiodarone in tissues and organs has been suggested as a possible mechanism. The most frequent dermatologic side effects are photosensitivity, skin discoloration and erythema. This article presents the case of a patient who developed amiodarone-induced bullous skin lesions and hepatotoxicity.

  6. Atypical response to treatment in linear IgA bullous dermatosis of childhood: Revision of literature.

    Science.gov (United States)

    Moleiro, Susana; Santos, Vera; Calha, Manuela; Pessoa, Graça

    2011-06-15

    A three-year-old boy presented with 2 months of worsening skin lesions characterized by multiple clear vesicles and bullae. The histopathological and immunohistochemical examinations revealed changes consistent with linear IgA bullous dermatosis of childhood. Treatment with dapsone and prednisolone resulted in gradual clinical improvement. However, within a week of therapy he presented with diabetic ketoacidosis, the onset of type I diabetes mellitus. Since then, keeping this child asymptomatic has been a challenge. This case emphasizes the importance of close monitoring of patients taking systemic corticosteroids; the coexistence of other immune mediated conditions may influence the success of treatment.

  7. Common mechanisms of autoimmune diseases (the autoimmune tautology).

    Science.gov (United States)

    Anaya, Juan-Manuel

    2012-09-01

    The fact that autoimmune diseases share subphenotypes, physiopathological mechanisms and genetic factors has been called autoimmune tautology, and indicates that they have a common origin. The autoimmune phenotypes vary depending on the target cell and the affected organ, gender, ancestry, trigger factors and age at onset. Ten shared characteristics supporting this logical theory are herein reviewed.

  8. Epigenomics of autoimmune diseases.

    Science.gov (United States)

    Gupta, Bhawna; Hawkins, R David

    2015-03-01

    Autoimmune diseases are complex disorders of largely unknown etiology. Genetic studies have identified a limited number of causal genes from a marginal number of individuals, and demonstrated a high degree of discordance in monozygotic twins. Studies have begun to reveal epigenetic contributions to these diseases, primarily through the study of DNA methylation, but chromatin and non-coding RNA changes are also emerging. Moving forward an integrative analysis of genomic, transcriptomic and epigenomic data, with the latter two coming from specific cell types, will provide an understanding that has been missed from genetics alone. We provide an overview of the current state of the field and vision for deriving the epigenomics of autoimmunity.

  9. A pilot trial of dermoscopy as a rapid assessment tool in pediatric dermatoses.

    Science.gov (United States)

    Silverberg, Nanette B

    2011-03-01

    Dermoscopy is a noninvasive technique to assess skin architecture. A pilot study was conducted using polarized dermoscopy as a tool to monitor the pediatric skin barrier. Ten pediatric patients (age range, 1-14 years) with mild to moderate atopic dermatitis (AD), ichthyosis vulgaris (IV), and/or keratosis pilaris (KP) participated in a 4-week clinical trial. After a week of emollient usage alone, a mid-potency topical corticosteroid cream was added twice daily if necessary to treat erythema, dermatitis, or pruritus. The participants were assessed at weeks 0, 1, and 4 using the eczema area and severity index (EASI) for atopic dermatitis, investigator global assessment for atopic dermatitis, children dermatology life quality index (CDLQI), and clinical and dermoscopic photography. Dermoscopic appearance demonstrated dermal vascular ectasia in AD and KP, hyperkeratosis and prominence of the interkeratinocyte space in AD and IV and widening of the follicular orifice in KP. Improvements in these dermoscopic abnormalities were noted after emollient usage, mirroring improvements in clinical appearance, EASI, and CDLQI. Dermoscopy is a promising tool to assess localized improvement in skin architecture in pediatric dermatoses. Further studies and development of scoring systems will be needed to apply this technology to clinical practice.

  10. A PROSPECTIVE STUDY ON DERMATOSES IN PREGNANCY IN A TERTIARY CARE HOSPITAL OF SOUTHERN ASSAM

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    Vaswatee

    2015-09-01

    Full Text Available Various skin diseases are seen in pregnancy. Some of these diseases occur specifically in pregnancy. Maternal and fetal morbidity and mortality can be decreased by early diagnosis and appropriate treatment of these conditions. A total of 53 pregnant females were found to have dermatological disorders or sexually transmitted diseases during the study period. The commonest pregnancy specific dermatoses in the study was polymorphic eruption of pregnancy (22.64% followed by prurigo of pregnancy (9.43%, pruritic folliculitis of pregnancy (5.66%, pruritus gravidarum (0.01%, impetigo herpetiformis (0.01% and pemphigoid gestationis (0.01%. The commonest dermatological disorder was scabies (13.20% followed by candidiasis (11.32%, dermatophytosis (9.43%, melasma (5.66% and equal number of cases of urticaria (3.7% and acne (3.7%. Among sexually transmitted diseases, commonest disease was genital warts (3.7% followed by single case (0.01% each of syphilis, molluscum contagiosum and one patient was seropositive for HIV.

  11. THE ‘HOLI’ DERMATOSES: ANNUAL SPATE OF SKIN DISEASES FOLLOWING THE SPRING FESTIVAL IN INDIA

    Science.gov (United States)

    Ghosh, Sudip Kumar; Bandyopadhyay, Debabrata; Chatterjee, Gobinda; Saha, Debabrata

    2009-01-01

    Background: ‘Holi’ is an annual spring festival celebrated all over India. The central ritual of Holi involves throwing of colors on one another. Playing with toxic industrial dyes often results in various dermatological complaints in a significant number of people immediately following the celebration. Aims: To describe patterns of various skin manifestations directly or indirectly related to the use of different colors in the celebration of Holi. Methods: Observational clinical study on consecutive patients presenting to a teaching hospital in Kolkata, India. Results: Forty-two patients with a mean age of 24.2 years were studied. Itching was the commonest symptom (25, 59.5%), followed by burning sensation, pain, oozing, and scaling. Eleven patients’ symptoms were attributed to activities related to preparation of colors and the removal of colors from the skin surface. Eczematous lesions were the most common pattern (24, 57.1%) followed by erosions, xerosis and scaling, erythema, urticaria, acute nail-fold inflammation, and abrasions. Thirteen (30.9%) patients reported aggravation of preexisting dermatoses (acne, eczema, and paronychia). Secondary pyoderma occurred in 3 (7.1%). Face was the commonest site affected (24, 57.1%), followed by dorsum of the hands, scalp, forearm, palms, arms, and trunk. Ocular complaints in the form of redness, watering, and grittiness occurred in 7 (16.7%) patients. Conclusion: Various forms of cutaneous manifestations, often associated with ocular complaints, occur commonly due to Holi colors. Public awareness and regulatory actions are needed to avoid these preventable conditions. PMID:20161854

  12. The use of oral cyclosporin to treat feline dermatoses: a retrospective analysis of 23 cases.

    Science.gov (United States)

    Vercelli, A; Raviri, G; Cornegliani, L

    2006-06-01

    Limited information is available regarding the use of cyclosporin A (CsA) for the treatment of feline dermatoses. The aim of this retrospective study was therefore to describe the efficacy of CsA for the therapy of eosinophilic granuloma (EG), eosinophilic plaque, indolent ulcer, linear granulomas, idiopathic pruritus and stomatitis. A computer search for feline dermatological cases treated with CsA between 1999 and 2004 was performed. Based on history, clinical signs and laboratory diagnostic tests, it was then possible to divide cases into three groups and to select 23 cats. Seven cats had one or more of the following conditions: EG, eosinophilic plaque, indolent ulcer and/or linear granuloma (group A); eight cats had idiopathic pruritus (group B) and eight cats had plasmacytic stomatitis (group C). Doses ranged from 5.8 to 13.3 mg kg(-1) oral CsA. All cats were monitored, with complete serum blood analysis and physical examination, monthly for a minimum of 6 months. Response to therapy was scored (severity of lesions and pruritus) with a 0-10 visual analogue scale at each visit (day 0, day, 30, day 60, day 90). All cats in groups A and B were cured and were maintained on alternate day therapy. In group C, 4/8 patients went into remission, while remaining cats have a fair to good improvement. Routine haematological and biochemical examination failed to reveal abnormalities related to CsA administration.

  13. [Autoimmune hemolytic anemia in children].

    Science.gov (United States)

    Becheur, M; Bouslama, B; Slama, H; Toumi, N E H

    2015-01-01

    Autoimmune hemolytic anemia is a rare condition in children which differs from the adult form. It is defined by immune-mediated destruction of red blood cells caused by autoantibodies. Characteristics of the autoantibodies are responsible for the various clinical entities. Classifications of autoimmune hemolytic anemia include warm autoimmune hemolytic anemia, cold autoimmune hemolytic anemia, and paroxysmal cold hemoglobinuria. For each classification, this review discusses the epidemiology, etiology, clinical presentation, laboratory evaluation, and treatment options.

  14. Autoimmune muscular pathologies.

    Science.gov (United States)

    Dalakas, M C

    2005-05-01

    The T cell-mediated mechanism responsible for Polymyositis and inclusion Body Myositis and the complement-mediated microangiopathy associated with Dermatomyositis are reviewed. The management of autoimmune myopathies with the presently available immunotherapeutic agents as well as new therapies and ongoing trials are discussed.

  15. Autoimmune pancreatitis and cholangitis

    Institute of Scientific and Technical Information of China (English)

    Niraj; Jani; James; Buxbaum

    2015-01-01

    Autoimmune pancreatitis(AIP) is part of a systemic fibrosclerotic process characterized by lymphoplasmacytic infiltrate with immunoglobulin G subtype-4(Ig G4) positive cells. It characteristically presents with biliary obstruction due to mass-like swelling of the pancreas. Frequently AIP is accompanied by extra-pancreaticmanifestations including retroperitoneal fibrosis, thyroid disease, and salivary gland involvement. Auto-antibodies, hypergammaglobulemia, and prompt resolution of pancreatic and extrapancreatic findings with steroids signify its autoimmune nature. Refractory cases are responsive to immunomodulators and rituximab. Involvement of the biliary tree, termed IgG 4 associated cholangiopathy, mimics primary sclerosing cholangitis and is challenging to manage. High IgG 4 levels and swelling of the pancreas with a diminutive pancreatic duct are suggestive of autoimmune pancreatitis. Given similarities in presentation but radical differences in management and outcome, differentiation from pancreatic malignancy is of paramount importance. There is controversy regarding the optimal diagnostic criterion and steroid trials to make the diagnosis. Additionally, the retroperitoneal location of the pancreas and requirement for histologic sampling, makes tissue acquisition challenging. Recently, a second type of autoimmune pancreatitis has been recognized with similar clinical presentation and steroid response though different histology, serologic, and extrapancreatic findings.

  16. Autoimmune paediatric liver disease

    Institute of Scientific and Technical Information of China (English)

    Giorgina Mieli-Vergani; Diego Vergani

    2008-01-01

    Liver disorders with a likely autoimmune pathogenesis in childhood include autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC),and de novo AIH after liver transplantation.AIH is divided into two subtypes according to seropositivity for smooth muscle and/or antinuclear antibody (SMA/ANA,type 1) or liver kidney microsomal antibody (LKM1,type 2).There is a female predominance in both.LKM1 positive patients tend to present more acutely,at a younger age,and commonly have partial IgA deficiency,while duration of symptoms before diagnosis,clinical signs,family history of autoimmunity, presence of associated autoimmune disorders,response to treatment,and long-term prognosis are similar in both groups. The most common type of paediatric sclerosing cholangitis is ASC.The clinical,biochemical, immunological,and histological presentation of ASC is often indistinguishable from that of AIH type 1.In both,there are high IgG,non-organ specific autoantibodies,and interface hepatitis.Diagnosis is made by cholangiography.Children with ASC respond to immunosuppression satisfactorily and similarly to AIH in respect to remission and relapse rates,times to normalization of biochemical parameters, and decreased inflammatory activity on follow up liver biopsies. However,the cholangiopathy can progress.There may be evolution from AIH to ASC over the years,despite treatment.De novo AIH after liver transplantation affects patients not transplanted for autoimmune disorders and is strikingly reminiscent of classical AIH,including elevated titres of serum antibodies, hypergammaglobulinaemia,and histological findings of interface hepatitis,bridging fibrosis,and collapse.Like classical AIH,it responds to treatment with prednisolone and azathioprine.De novo AIH post liver transplantation may derive from interference by calcineurin inhibitors with the intrathymic physiological mechanisms of T-cell maturation and selection.Whether this condition is a distinct entity or a form of

  17. Descemet-stripping automated endothelial keratoplasty for vitrectomized cases with traumatic aniridia and aphakic bullous keratopathy

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    Eguchi H

    2012-09-01

    Full Text Available Hiroshi Eguchi, Tatsuro Miyamoto, Fumika Hotta, Machiko Tomida, Masayuki Inoue, Yoshinori MitamuraDepartment of Ophthalmology, Institute of Health Biosciences, The University of Tokushima Graduate School, Tokushima, JapanAbstract: The surgical indication for Descemet-stripping automated endothelial keratoplasty (DSAEK is largely limited to phakic or pseudophakic cases of endothelial dysfunction with normal pupils, because the endothelial lenticule is generally attached to the recipient cornea by use of gas tamponade into the anterior chamber. Although it may be desirable for vitrectomized cases with aniridia and aphakic bullous keratopathy without capsule support to undergo DSAEK, one of the major problems is lenticule detachment during surgery or in the postoperative period. To perform DSAEK in such cases, special surgical techniques are needed in order to facilitate adhesion of the lenticule. Herein, we describe a suture technique for attaching the endothelial lenticule in DSAEK for aniridic and aphakic cases that have undergone vitrectomy for traumatic vitreoretinal disease.Keywords: traumatic aniridia, aphakic bullous keratopathy, Descemet-stripping automated endothelial keratoplasty, vitrectomy

  18. Beauveria bassiana keratitis in bullous keratopathy: antifungal sensitivity testing and management.

    Science.gov (United States)

    Figueira, Luís; Pinheiro, Dolores; Moreira, Raul; Pinto, Eugénia; Simões, Joana; Camisa, Elisa; Torrão, Luís; Palmares, Jorge; Falcão-Reis, Fernando

    2012-01-01

    Beauveria bassiana is a ubiquitous fungus available as an insecticide. In humans, it has limited virulence; to our knowledge, only 3 cases of invasive disease and 10 cases of keratitis have been documented. We report the first case of B bassiana keratitis in a patient with aphakic bullous keratopathy. The fungal keratitis proved to be highly resistant to topical clotrimazole. Molecular identification was based on DNA sequence analysis. The minimal inhibitory concentrations (MIC) obtained were 2 µg/mL for voriconazole, 0.250 µg/mL for posaconazole, and >128 µg/mL for fluconazole; amphotericin B MIC was >16 µg/mL. In the absence of clinical improvement, a penetrating keratoplasty (PK) was performed. The patient was discharged on topical and systemic voriconazole and prednisolone 40 mg PO/day. The eye remained calm with a transparent cornea and clear anterior chamber. B bassiana keratitis is extremely rare, with only a few cases reported. Its risk factors are unknown. We report the first case in a patient with aphakic bullous keratopathy, which proved highly resistant to antifungal therapy (antifungal susceptibility results are presented). A PK was necessary for clinical improvement. A review of the literature is performed in an effort to define therapeutic strategies.

  19. 大疱性硬斑病1例%A case of bullous morphea

    Institute of Scientific and Technical Information of China (English)

    贾虹; 赵春霞; 石继海; 曹元华; 曾学思

    2001-01-01

    A rare case of bullous morphea(BM) was reported. A 25-year-oldman was seen in our hospital with a 3-year history of cutaneous lesions, and the movement of his joints was limited. Bullous lesions appeared one year later. The lesions were markedly atrophic and sclerotic. Some joints were stiffened. Some blisters were present over the extensor surface of the left arm. Nikolsky' s sign was negative. No Raynaud's phenomenon and internal organ involvement were observed. Biopsy of the skin lesion showed bundles of collagen fibers extending throughout the dermis. The blister was subepidermal in position.%报告1例少见的大疱性硬斑病。患者男性,25岁。四肢皮肤明显硬化萎缩,部分关节强直固定。左前臂伸侧大小不等的水疱,尼氏征阴性。无雷诺现象等系统损害。病理检查为典型的真皮均-化胶原纤维变性等硬皮病表现及表皮下水疱。

  20. Linear IgA bullous dermatosis: an unusual cause of upper eyelid cicatricial entropion.

    Science.gov (United States)

    Carruth, Bryant P; Meyer, Dale R

    2013-01-01

    Chronic cicatrizing conjunctivitis is a relatively uncommon condition resulting in significant ophthalmic morbidity, including keratoconjunctivitis sicca, cicatricial entropion, trichiasis, corneal scarring, significant discomfort, and visual loss. The potential causes of cicatrizing conjunctivitis are varied and include commonly encountered entities such as ocular cicatricial pemphigoid, Stevens-Johnson syndrome, and trachoma and many more rare causes which are particularly difficult to diagnose and treat and may not be familiar to the ophthalmologist. The authors herein present a case of chronic cicatrizing conjunctivitis, cicatricial entropion, and trichiasis caused by a rare entity called linear IgA bullous dermatosis. The case presentation conforms to the tenets of the Declaration of Helsinki and is Health Insurance Portability and Accountability Act compliant. This chronic dermatosis has a varied presentation, and the ophthalmic manifestations in particular have been infrequently described. This case demonstrates the benefits of immunohistochemistry in diagnosis and the difficulties in medical and surgical management of linear IgA bullous dermatosis while underscoring the lifelong difficulties in managing chronic inflammatory conditions causing ocular cicatrization.

  1. Nonsegmental Vitiligo and Autoimmune Mechanism

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    Naoki Oiso

    2011-01-01

    Full Text Available Nonsegmental vitiligo is a depigmented skin disorder showing acquired, progressive, and depigmented lesions of the skin, mucosa, and hair. It is believed to be caused mainly by the autoimmune loss of melanocytes from the involved areas. It is frequently associated with other autoimmune diseases, particularly autoimmune thyroid diseases including Hashimoto's thyroiditis and Graves' disease, rheumatoid arthritis, type 1 diabetes, psoriasis, pernicious anemia, systemic lupus erythematosus, Addison's disease, and alopecia areata. This indicates the presence of genetically determined susceptibility to not only vitiligo but also to other autoimmune disorders. Here, we summarize current understanding of autoimmune pathogenesis in non-segmental vitiligo.

  2. [Polyglandular autoimmune syndromes : An overview].

    Science.gov (United States)

    Komminoth, P

    2016-05-01

    Polyglandular autoimmune syndromes (PGAS), also known as autoimmune polyendocrinopathy syndromes (APS), are a heterogeneous group of rare, genetically caused diseases of the immune system which lead to inflammatory damage of various endocrine glands resulting in malfunctions. In addition, autoimmune diseases of non-endocrine organs may also be found. Early diagnosis of PGAS is often overlooked because of heterogeneous symptoms and the progressive occurrence of the individual diseases. The two most important forms of PGAS are the juvenile and adult types. The juvenile type (PGAS type 1) is caused by mutations in the autoimmune regulator (AIRE) gene on chromosome 21, exhibits geographic variations in incidence and is defined by the combination of mucocutaneous candidiasis, Addison's disease and hypoparathyroidism. In addition, autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) syndrome and other autoimmune diseases can also occur. The adult form of PGAS (PGAS type 2) is a multigenetic disorder associated with some HLA haplotypes, is more common than the juvenile type, shows female predominance and exhibits the combination of type 1 diabetes, autoimmune thyroid disease, Addison's disease and other autoimmune disorders. The histological alterations in affected organs of PGAS patients are similar to findings in sporadically occurring autoimmune diseases of these organs but there are no pathognomic fine tissue findings. If patients exhibit autoimmune changes in two different endocrine glands or if there are indications of several autoimmune disorders from the patient history, it is important to consider PGAS and inform the clinicians of this suspicion.

  3. Dermatoses em pacientes com diabetes mellitus Skin lesions in diabetic patients

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    N T Foss

    2005-08-01

    Full Text Available OBJETIVO: Ainda é desconhecida a relação do diabetes com fatores determinantes ou precipitantes de lesões dermatológicas em pacientes diabéticos. Assim, o objetivo do estudo foi investigar a presença de lesões cutâneas, não referidas pelo paciente diabético e sua relação com o controle metabólico da doença. MÉTODOS: Foram examinados 403 pacientes, dos quais 31% eram diabéticos do tipo 1 e 69% do tipo 2. Em ambulatório de um hospital universitário, os pacientes foram atendidos por endocrinologista para a avaliação endócrino-metabólica e por dermatologista para a avaliação dermatológica. O grau de controle metabólico foi documentado em 136 pacientes por meio da dosagem de hemoglobina glicada. RESULTADOS: Houve predomínio de dermatofitoses (82,6%, seguido de grupo de dermatoses como acne e degeneração actínica (66,7%, piodermites (5%, tumores cutâneos (3% e necrobiose lipoídica (1%. Entre as dermatoses mais comuns em diabéticos, foram confirmados com exame histológico: dois diagnósticos de necrobiose (0,4%, cinco de dermopatia diabética (1,2% e três casos de mal perfurante plantar (0,7%. Os valores da hemoglobina glicada foram: 7,2% em pacientes com controle metabólico adequado nos dois tipos de diabetes e de 11,9% e 12,7% nos tipos 1 e 2, respectivamente, com controle inadequado. Nos pacientes com controle metabólico inadequado foi observada freqüência maior de dermatofitoses, em ambos os tipos de diabetes. CONCLUSÕES: Os dados revelaram freqüência elevada de lesão dermatológica nos pacientes diabéticos, especialmente dermatofitoses. Dessa forma, o descontrole metabólico do diabético propicia maior suscetibilidade a infecções cutâneas.OBJECTIVE: It is yet unknown the relationship between diabetes and determinants or triggering factors of skin lesions in diabetic patients. The purpose of the present study was to investigate the presence of unreported skin lesions in diabetic patients and their

  4. IgG/IgE bullous pemphigoid with CD45 lymphocytic reactivity to dermal blood vessels, nerves and eccrine sweat glands

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    Ana Maria Abreu-Velez

    2010-01-01

    Full Text Available Context: Bullous pemphigoid (BP, the most common autoimmune blistering disease, is mediated by autoantibodies. BP primarily affects the elderly and is characterized by the development of urticarial plaques surmounted by subepidermal blisters, and the deposition of immunoglobulins and complement at the basement membrane zone (BMZ of the skin. BP is immunologically characterized by the development of autoantibodies targeting two structural proteins of the hemidesmosomes, BP180 (collagen XVII and BP230 (BPAG1. Case Report: A 63 -year-old Caucasian female patient was evaluated for a 4 day history of several itching, erythematous blisters on her extremities. Biopsies for hematoxylin and eosin (H&E examination, as well as Periodic acid-Schiff (PAS, immunohistochemistry (IHC and direct immunofluorescence (DIF analysis were performed. Results: H&E demonstrated a subepidermal blister, with partial re-epithelialization of the blister floor. Within the blister lumen, numerous neutrophils were present, with occasional eosinophils and lymphocytes also noted. Within the dermis, a mild, superficial, perivascular and periadnexal infiltrate of lymphocytes, histiocytes and occasional eosinophils was identified, with mild perivascular leukocytoclastic debris. The PAS stain was positive at the BMZ, and around selected blood vessels, nerves and sweat glands. DIF revealed linear deposits of IgG and Complement/C3 and fibrinogen at the BMZ, and around selected dermal nerves, blood vessels and sweat glands. Strong granular deposits of IgE were also observed, colocalizing with monoclonal antibodies to Collagen IV (CIV. By IHC, positive CD45 staining of lymphocytes was seen surrounding selected dermal blood vessels, eccrine sweat glands, and nerves. Conclusion : The patient displayed IgG, IgE, and fibrinogen autoantibodies against the BMZ, as well as around some dermal nerves and sweat glands; their binding in the skin could trigger complement activation. In addition, the

  5. Eosinophil localization to the basement membrane zone is autoantibody- and complement-dependent in a human cryosection model of bullous pemphigoid.

    Science.gov (United States)

    Messingham, Kelly N; Wang, Jeffrey W; Holahan, Heather M; Srikantha, Rupasree; Aust, Samantha C; Fairley, Janet A

    2016-01-01

    Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by antibodies (IgG and IgE) targeting cell-substrate adhesion proteins. A variety of BP models suggest that autoantibody-dependent neutrophil degranulation is essential for blister formation. However, lesional biopsies reveal a predominance of eosinophils and few neutrophils. Our goal was to evaluate the role of antibodies and complement in eosinophil localization, degranulation and split formation at the dermo-epidermal junction (DEJ) utilizing a human skin cryosection model of BP paired with a human eosinophilic cell line, 15HL-60. Expression of receptors for IgG (FcγRII), IgE (FcεRI) and complement (CR1 and CR3) was confirmed on 15HL-60 cells using flow cytometry. 15HL-60 expression of granule protein [eosinophil derived neurotoxin (EDN) and eosinophil peroxidase (EPO)] mRNA and their degranulation in vitro was confirmed using RT-PCR and ELISA, respectively. For cryosection experiments, BP or control sera or IgG and IgE antibodies purified from BP sera were utilized in combination with 15HL-60 cells ± fresh complement. Both BP serum and fresh complement were required for localization of 15-HL60 cells to the DEJ. Interestingly, eosinophil localization to the DEJ was dependent on IgG, but not IgE, and complement. However, no subepidermal split was observed. Additionally, the 15HL-60 cells did not degranulate under any experimental conditions and direct application of cell lysate to cryosections did not result in a split. Our observation that eosinophil localization to the DEJ is dependent on IgG mediated complement fixation provides additional insight into the sequence of events during the development of BP lesions.

  6. The ′Holi′ dermatoses : Annual spate of skin diseases following the spring festival in India

    Directory of Open Access Journals (Sweden)

    Ghosh Sudip

    2009-01-01

    Full Text Available Background : ′Holi′ is an annual spring festival celebrated all over India. The central ritual of Holi involves throwing of colors on one another. Playing with toxic industrial dyes often results in various dermatological complaints in a significant number of people immediately following the celebration. Aims : To describe patterns of various skin manifestations directly or indirectly related to the use of different colors in the celebration of Holi. Methods : Observational clinical study on consecutive patients presenting to a teaching hospital in Kolkata, India. Results : Forty-two patients with a mean age of 24.2 years were studied. Itching was the commonest symptom (25, 59.5%, followed by burning sensation, pain, oozing, and scaling. Eleven patients′ symptoms were attributed to activities related to preparation of colors and the removal of colors from the skin surface. Eczematous lesions were the most common pattern (24, 57.1% followed by erosions, xerosis and scaling, erythema, urticaria, acute nail-fold inflammation, and abrasions. Thirteen (30.9% patients reported aggravation of preexisting dermatoses (acne, eczema, and paronychia. Secondary pyoderma occurred in 3 (7.1%. Face was the commonest site affected (24, 57.1%, followed by dorsum of the hands, scalp, forearm, palms, arms, and trunk. Ocular complaints in the form of redness, watering, and grittiness occurred in 7 (16.7% patients. Conclusion : Various forms of cutaneous manifestations, often associated with ocular complaints, occur commonly due to Holi colors. Public awareness and regulatory actions are needed to avoid these preventable conditions.

  7. A clinico-etiological study of dermatoses in pediatric age group in tertiary health care center in South Gujarat region

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    Sugat A Jawade

    2015-01-01

    Full Text Available Background: Dermatologic conditions have different presentation and management in pediatric age group from that in adult; this to be studied separately for statistical and population based analysis. Objective: To study the pattern of various dermatoses in infants and children in tertiary health care center in South Gujarat region. Materials and Methods: This is a prospective study; various dermatoses were studied in pediatric patients up to 14 years of age attending the Dermatology OPD of New Civil Hospital, Surat, Gujarat over a period of 12 months from June 2009 to June 2010. All patients were divided into four different study groups: 1 to 6 years and 7 to 14 years. Results: There were 596 boys and 425 girls in total 1021 study populations. Majority of the skin conditions in neonates were erythema toxicum neonatorum (12.97%, scabies (9.92%, mongolian spot (9.16%, and seborrheic dermatitis (7.63%. In > 1 month to 14 years age group of children among infectious disorder, children were found to be affected most by scabies (24.49%, impetigo (5.96%, pyoderma (5.62%, molluscum contagiosum (5.39%, tinea capitis (4.49%, leprosy (2.02%, and viral warts (1.35% while among non-infectious disorders, they were affected by atopic dermatitis (4.27%, pityriasis alba (4.16%, seborrheic dermatitis (3.60%, pityriasis rosea (3.15%, others (3.01%, phrynoderma (2.70%, lichen planus (2.58%, contact dermatitis (1.57% and ichthyosis (1.45%. Conclusion: There is a need to emphasize on training the management of common pediatric dermatoses to dermatologists, general practitioners and pediatricians for early treatment.

  8. Sarcoidosis and Thyroid Autoimmunity

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    Piera Fazzi

    2017-08-01

    Full Text Available Most of the studies have shown a higher risk for subclinical and clinical hypothyroidism, antithyroid autoantibodies [overall antithyroid peroxidase antibodies (TPOAb], and in general, thyroid autoimmunity, overall in the female gender in patients with sarcoidosis (S. A significantly higher prevalence of clinical hypothyroidism and Graves’ disease was also described in female S patients with respect to controls. Gallium-67 (Ga-67 scyntigraphy in S patients, in the case of thyroid uptake, suggests the presence of aggressive autoimmune thyroiditis and hypothyroidism. For this reason, ultrasonography and thyroid function should be done in the case of Ga-67 thyroid uptake. In conclusion, thyroid function, TPOAb measurement, and ultrasonography should be done to assess the clinical profile in female S patients, and the ones at high risk (female individuals, with TPOAb positivity, and hypoechoic and small thyroid should have periodically thyroid function evaluations and suitable treatments.

  9. Update on autoimmune hepatitis

    Institute of Scientific and Technical Information of China (English)

    Andreas Teufel; Peter R Galle; Stephan Kanzler

    2009-01-01

    Autoimmune hepatitis (AIH) is a necroinflammatory liver disease of unknown etiology that occurs in children and adults of all ages. Characteristics are its autoimmune features, hyperglobulinemia (IgG), and the presence of circulating autoantibodies, as well as a response to immunosuppressant drugs. Current treatment consists of prednisone and azathioprine and in most patients this disease has become very treatable. Over the past 2 years, a couple of new insights into the genetic aspects, clinical course and treatment of AIH have been reported, which will be the focus of this review. In particular, we concentrate on genome-wide microsatellite analysis, a novel mouse model of AIH, the evaluation of a large AIH cohort for overlap syndromes,suggested novel criteria for the diagnosis of AIH, and the latest studies on treatment of AIH with budenoside and mycophenolate mofetil.

  10. Inheritance of autoimmune neuroinflammation

    OpenAIRE

    Stridh, Pernilla

    2010-01-01

    Multiple sclerosis (MS) is a chronic neuro-inflammatory disease with anticipated complex etiology. Susceptibility to MS is conferred by numerous genes, with very low odds ratios that explain minute fractions of disease. This indicates that unknown factors are responsible for the remaining genetic contribution, termed the missing heritability . Due to the similarities to MS pathogenesis, we studied myelin oligodendrocyte glycoprotein (MOG)-induced experimental autoimmune ...

  11. Autoimmune Addison's disease.

    Science.gov (United States)

    Napier, Catherine; Pearce, Simon H S

    2012-12-01

    Addison's disease is a rare autoimmune disorder. In the developed world, autoimmune adrenalitis is the commonest cause of primary adrenal insufficiency, where the majority of patients have circulating antibodies against the key steroidogenic enzyme 21-hydroxylase. A complex interplay of genetic, immunological and environmental factors culminates in symptomatic adrenocortical insufficiency, with symptoms typically developing over months to years. Biochemical evaluation and further targeted investigations must confirm primary adrenal failure and establish the underlying aetiology. The diagnosis of adrenocortical insufficiency will necessitate lifelong glucocorticoid and mineralocorticoid replacement therapy, aiming to emulate physiological patterns of hormone secretion to achieve well-being and good quality of life. Education of patients and healthcare professionals is essential to minimise the risk of a life-threatening adrenal crisis, which must be promptly recognised and aggressively managed when it does occur. This article provides an overview of our current understanding of the natural history and underlying genetic and immunological basis of this condition. Future research may reveal novel therapeutic strategies for patient management. Until then, optimisation of pharmacological intervention and continued emphasis on education and empowerment of patients should underpin the management of individuals with autoimmune Addison's disease. Copyright © 2012. Published by Elsevier Masson SAS.

  12. Autoantibodies in Autoimmune Pancreatitis

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    Daniel S. Smyk

    2012-01-01

    Full Text Available Autoimmune pancreatitis (AIP was first used to describe cases of pancreatitis with narrowing of the pancreatic duct, enlargement of the pancreas, hyper-γ-globulinaemia, and antinuclear antibody (ANA positivity serologically. The main differential diagnosis, is pancreatic cancer, which can be ruled out through radiological, serological, and histological investigations. The targets of ANA in patients with autoimmune pancreatitis do not appear to be similar to those found in other rheumatological diseases, as dsDNA, SS-A, and SS-B are not frequently recognized by AIP-related ANA. Other disease-specific autoantibodies, such as, antimitochondrial, antineutrophil cytoplasmic antibodies or diabetes-specific autoantibodies are virtually absent. Further studies have focused on the identification of pancreas-specific autoantigens and reported significant reactivity to lactoferrin, carbonic anhydrase, pancreas secretory trypsin inhibitor, amylase-alpha, heat-shock protein, and plasminogen-binding protein. This paper discusses the findings of these investigations and their relevance to the diagnosis, management, and pathogenesis of autoimmune pancreatitis.

  13. Complement in autoimmune diseases.

    Science.gov (United States)

    Vignesh, Pandiarajan; Rawat, Amit; Sharma, Madhubala; Singh, Surjit

    2017-02-01

    The complement system is an ancient and evolutionary conserved element of the innate immune mechanism. It comprises of more than 20 serum proteins most of which are synthesized in the liver. These proteins are synthesized as inactive precursor proteins which are activated by appropriate stimuli. The activated forms of these proteins act as proteases and cleave other components successively in amplification pathways leading to exponential generation of final effectors. Three major pathways of complement pathways have been described, namely the classical, alternative and lectin pathways which are activated by different stimuli. However, all the 3 pathways converge on Complement C3. Cleavage of C3 and C5 successively leads to the production of the membrane attack complex which is final common effector. Excessive and uncontrolled activation of the complement has been implicated in the host of autoimmune diseases. But the complement has also been bemusedly described as the proverbial "double edged sword". On one hand, complement is the final effector of tissue injury in autoimmune diseases and on the other, deficiencies of some components of the complement can result in autoimmune diseases. Currently available tools such as enzyme based immunoassays for functional assessment of complement pathways, flow cytometry, next generation sequencing and proteomics-based approaches provide an exciting opportunity to study this ancient yet mysterious element of innate immunity.

  14. Etiopathogenesis of Insulin Autoimmunity

    Directory of Open Access Journals (Sweden)

    Norio Kanatsuna

    2012-01-01

    Full Text Available Autoimmunity against pancreatic islet beta cells is strongly associated with proinsulin, insulin, or both. The insulin autoreactivity is particularly pronounced in children with young age at onset of type 1 diabetes. Possible mechanisms for (proinsulin autoimmunity may involve beta-cell destruction resulting in proinsulin peptide presentation on HLA-DR-DQ Class II molecules in pancreatic draining lymphnodes. Recent data on proinsulin peptide binding to type 1 diabetes-associated HLA-DQ2 and -DQ8 is reviewed and illustrated by molecular modeling. The importance of the cellular immune reaction involving cytotoxic CD8-positive T cells to kill beta cells through Class I MHC is discussed along with speculations of the possible role of B lymphocytes in presenting the proinsulin autoantigen over and over again through insulin-carrying insulin autoantibodies. In contrast to autoantibodies against other islet autoantigens such as GAD65, IA-2, and ZnT8 transporters, it has not been possible yet to standardize the insulin autoantibody test. As islet autoantibodies predict type 1 diabetes, it is imperative to clarify the mechanisms of insulin autoimmunity.

  15. Autoimmune Progesterone Anaphylaxis

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    Mohammad Hassan Bemanian

    2007-06-01

    Full Text Available Progesterone induced dermatitis is a rare disorder. It typically occurs in females due to anautoimmune phenomenon to endogenous progesterone production, but can also be caused byexogenous intake of a synthetic progestin. Here in, we present a case of autoimmune progesterone anaphylaxis (AIPA observed in an adolescent female.The patient is an 18-year-old Caucasian female with no significant past medical history and noprior exogenous hormone use, who presented to her primary care physician complaining of cyclic skin eruptions with dyspnea, cough and respiratory distress. She noted that her symptoms occurred monthly, just prior to her menses. An intradermal skin test using 0.1 cml of progesterone was performed. The patient developed a 15mm wheal after 15 minutes, confirming the diagnosis of AIPA.The patient was started on a continuous regimen of an oral conjugated estrogen (0.625mg. The skin eruptions and respiratory symptoms have not returned since the initiation of this therapy.Autoimmune progesterone dermatitis manifests via the occurrence of cyclic skin eruptions.Women with the disorder commonly present with dermatologic lesions in the luteal phase of themenstrual cycle, if there are any other organ involvement in addition to skin (e.g. lung, GI thereaction should be called as autoimmune progesterone anaphylaxis. Diagnosis of AIPA is confirmed by performing a skin allergen test using progesterone.

  16. A Retrospective and Comparative Study of Familial and Non-familial Bullous Lichen Planus

    Institute of Scientific and Technical Information of China (English)

    HUANG Changzheng; YAN Xiaofeng; YANG Lingyun; ZHANG Jing; TIAN Jin; LI Jiawen; WANG Chunsen; TU Yating

    2007-01-01

    In order to compare the clinical characteristics of familial and non-familial bullous lichen planus (BLP), the archival data of 36 BLP patients with positive family history and 21 BLP patients with negative family history diagnosed according to the clinical features and histopathology were collected in our department from 1956 to 2003. The clinical features were analyzed and compared. There were significant differences between familial and non-familial BLP in age of onset, duration of disease and extension of eruption (P<0.01). It was concluded that familial BLP appeared to differ from the non-familial form in its earlier age of onset, longer duration of the disease, more extensive eruption and more tendency to involve nails. Hereditary factors may play a role in the pathogenesis of familial BLP.

  17. Bullous Dermatosis in an End-Stage Renal Disease Patient: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Zeenat Yousuf Bhat

    2016-01-01

    Full Text Available Patients with advanced chronic kidney disease including ESRD patients may present with a wide spectrum of cutaneous abnormalities, ranging from xerosis to hyperpigmentation to severe deforming necrotizing lesions. Skin problems are not uncommon in this population of patients, with a clinical presentation that can be quite bizarre, mandating a long list of differential diagnostic possibilities, and subsequent rise of a puzzling diagnostic challenge. We describe an ESRD patient who presented with blistering, nonhealing ulcerative lesions with a diagnostic skin biopsy revealing a mixed pattern of linear IgA bullous dermatosis and dermatitis herpetiformis. A clinical remission could be achieved with pulse intravenous steroids followed by oral maintenance in combination with dapsone, with no evidence of recurrence.

  18. Bullous Dermatosis in an End-Stage Renal Disease Patient: A Case Report and Literature Review

    Science.gov (United States)

    Abu Minshar, Marwan; Thompson, Andrew; Malik, Yahya Osman

    2016-01-01

    Patients with advanced chronic kidney disease including ESRD patients may present with a wide spectrum of cutaneous abnormalities, ranging from xerosis to hyperpigmentation to severe deforming necrotizing lesions. Skin problems are not uncommon in this population of patients, with a clinical presentation that can be quite bizarre, mandating a long list of differential diagnostic possibilities, and subsequent rise of a puzzling diagnostic challenge. We describe an ESRD patient who presented with blistering, nonhealing ulcerative lesions with a diagnostic skin biopsy revealing a mixed pattern of linear IgA bullous dermatosis and dermatitis herpetiformis. A clinical remission could be achieved with pulse intravenous steroids followed by oral maintenance in combination with dapsone, with no evidence of recurrence. PMID:28003921

  19. An atypical case of herpes simplex virus endotheliitis presented as bullous keratopathy.

    Science.gov (United States)

    Papaioannou, Lamprini; Tsolkas, Georgios; Theodossiadis, Panagiotis; Papathanassiou, Miltiadis

    2013-12-01

    To present an atypical case of herpes simplex virus (HSV) endotheliitis. The authors report the case of a 62-year-old female patient who presented with unilateral diffuse corneal edema with Descemet's membrane folds and bullae, without keratic precipitates, iritis, significant anterior chamber reaction, or intraocular pressure (IOP) elevation. The patient had no documented positive history of ocular surgery and no abnormal findings were present in the fellow eye. Endotheliitis of viral origin was suspected and Goldmann-Witmer coefficient for HSV, cytomegalovirus, and varicela zoster virus was calculated. Goldmann-Witmer coefficient was positive for HSV. Treatment with oral valacyclovir and topical dexamethasone resulted in complete resolution of corneal edema within 1 week. HSV endotheliitis can present with bullous keratopathy as the only clinical manifestation, without typical findings such as keratic precipitates, iritis, and IOP elevation.

  20. Herpes Simplex Virus Infection Mimicking Bullous Disease in an Immunocompromised Patient

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    Anne L.Y. Lecluse

    2010-06-01

    Full Text Available Immunodeficient patients are at risk of developing extended or atypical herpes simplex virus infections, which can be easily misdiagnosed. We present the case of a 79-year-old, treatment-induced (oral corticosteroid, immunocompromised female with an extensive atypical herpes simplex virus infection. This patient presented with multiple erosions and vesicles on the trunk with a subacute onset. The clinical differential diagnosis was herpes simplex infection, herpes zoster infection, pemphigus vulgaris or bullous pemphigoid. Due to the atypical clinical presentation and negative Tzanck test, suspicion of viral infection was low. High-dose steroid treatment was initiated. Subsequent histopathology, however, showed a herpes simplex virus infection. After discontinuing steroid treatment and initiating antiviral treatment, the patient recovered within a week. Emphasis must be placed on the importance of clinical awareness of extended and clinically atypical herpes simplex infections in immunocompromised patients. A negative Tzanck test does not rule out the possibility of a herpes infection.

  1. Autoantibodies in autoimmune liver diseases.

    Science.gov (United States)

    Sener, Asli Gamze

    2015-11-01

    Autoimmune hepatitis is a chronic hepatitis of unknown etiology characterized by clinical, histological, and immunological features, generally including circulating autoantibodies and a high total serum and/or gamma globulin. Liver-related autoantibodies are very significant for the correct diagnosis and classification of autoimmune liver diseases (AILD), namely autoimmune hepatitis types 1 and 2 (AIH-1 and 2), primary biliary cirrhosis (PBC), and the sclerosing cholangitis types in adults and children. This article intends to review recent studies that investigate autoantibodies in autoimmune liver diseases from a microbiological perspective.

  2. Bullous Keratopathy

    Science.gov (United States)

    ... cloudy cornea with blisters on the surface. Treatment Salty eye drops Drugs to lower pressure in the ... and treatment—surgical and nonsurgical—of eye disorders). Salty eye drops (hypertonic saline) and salty ointments are ...

  3. Neuropathology of autoimmune encephalitides.

    Science.gov (United States)

    Bauer, Jan; Bien, Christian G

    2016-01-01

    In recent years a large number of antibody-associated or antibody-defined encephalitides have been discovered. These conditions are often referred to as autoimmune encephalitides. The clinical features include prominent epileptic seizures, cognitive and psychiatric disturbance. These encephalitides can be divided in those with antibodies against intracellular antigens and those with antibodies against surface antigens. The discovery of new antibodies against targets on the surface of neurons is especially interesting since patients with such antibodies can be successfully treated immunologically. This chapter focuses on the pathology and the pathogenetic mechanisms involved in these encephalitides and discusses some of the questions that are raised in this exciting new field. It is important to realise, however, that because of the use of antibodies to diagnose the patients, and their improvement with treatment, there are relatively few biopsy or postmortem reports, limiting the neuropathological data and conclusions that can be drawn. For this reason we especially focus on the most frequent autoimmune encephalitides, those with antibodies to the NMDA receptor and with antibodies to the known protein components of the VGKC complex. Analysis of these encephalitides show completely different pathogenic mechanisms. In VGKC complex encephalitis, antibodies seem to bind to their target and activate complement, leading to destruction and loss of neurons. On the other hand, in NMDAR encephalitis, complement activation and neuronal degeneration seems to be largely absent. Instead, binding of antibodies leads to a decrease of NMDA receptors resulting in a hypofunction. This hypofunction offers an explanation for some of the clinical features such as psychosis and episodic memory impairment, but not for the frequent seizures. Thus, additional analysis of the few human brain specimens present and the use of specific animal models are needed to further understand the effects

  4. Warm autoimmune hemolytic anemia.

    Science.gov (United States)

    Naik, Rakhi

    2015-06-01

    Warm autoimmune hemolytic anemia (AIHA) is defined as the destruction of circulating red blood cells (RBCs) in the setting of anti-RBC autoantibodies that optimally react at 37°C. The pathophysiology of disease involves phagocytosis of autoantibody-coated RBCs in the spleen and complement-mediated hemolysis. Thus far, treatment is aimed at decreasing autoantibody production with immunosuppression or reducing phagocytosis of affected cells in the spleen. The role of complement inhibitors in warm AIHA has not been explored. This article addresses the diagnosis, etiology, and treatment of warm AIHA and highlights the role of complement in disease pathology.

  5. Autoimmune diseases and myelodysplastic syndromes.

    Science.gov (United States)

    Komrokji, Rami S; Kulasekararaj, Austin; Al Ali, Najla H; Kordasti, Shahram; Bart-Smith, Emily; Craig, Benjamin M; Padron, Eric; Zhang, Ling; Lancet, Jeffrey E; Pinilla-Ibarz, Javier; List, Alan F; Mufti, Ghulam J; Epling-Burnette, Pearlie K

    2016-05-01

    Immune dysregulation and altered T-cell hemostasis play important roles in the pathogenesis of myelodysplastic syndromes (MDS). Recent studies suggest an increased risk of MDS among patients with autoimmune diseases. Here, we investigated the prevalence of autoimmune diseases among MDS patients, comparing characteristics and outcomes in those with and without autoimmune diseases. From our study group of 1408 MDS patients, 391 (28%) had autoimmune disease, with hypothyroidism being the most common type, accounting for 44% (n = 171) of patients (12% among all MDS patients analyzed). Other autoimmune diseases with ≥5% prevalence included idiopathic thrombocytopenic purpura in 12% (n = 46), rheumatoid arthritis in 10% (n = 41), and psoriasis in 7% (n = 28) of patients. Autoimmune diseases were more common in female MDS patients, those with RA or RCMD WHO subtype, and those who were less dependent on red blood cell transfusion. Median overall survival (OS) was 60 months (95% CI, 50-70) for patients with autoimmune diseases versus 45 months (95% CI, 40-49) for those without (log-rank test, P = 0.006). By multivariate analysis adjusting for revised IPSS and age >60 years, autoimmune diseases were a statistically significant independent factor for OS (HR 0.78; 95% CI, 0.66-0.92; P = 0.004). The rate of acute myeloid leukemia (AML) transformation was 23% (n = 89) in MDS patients with autoimmune disease versus 30% (n = 301) in those without (P = 0.011). Patient groups did not differ in response to azacitidine or lenalidomide treatment. Autoimmune diseases are prevalent among MDS patients. MDS patients with autoimmune diseases have better OS and less AML transformation.

  6. Prevalence of collagen VII-specific autoantibodies in patients with autoimmune and inflammatory diseases

    Directory of Open Access Journals (Sweden)

    Licarete Emilia

    2012-04-01

    Full Text Available Abstract Background Autoimmunity to collagen VII is typically associated with the skin blistering disease epidermolysis bullosa acquisita (EBA, but also occurs occasionally in patients with systemic lupus erythematosus or inflammatory bowel disease. The aim of our present study was to develop an accurate immunoassay for assessing the presence of autoantibodies against collagen VII in large cohorts of patients and healthy donors. Methods Based on in silico antigenic analysis and previous wetlab epitope mapping data, we designed a chimeric collagen VII construct containing all collagen VII epitopes with higher antigenicity. ELISA was performed with sera from patients with EBA (n = 50, Crohn's disease (CD, n = 50, ulcerative colitis (UC, n = 50, bullous pemphigoid (BP, n = 76, and pemphigus vulgaris (PV, n = 42 and healthy donors (n = 245. Results By ELISA, the receiver operating characteristics analysis yielded an area under the curve of 0.98 (95% CI: 0.9638-1.005, allowing to set the cut-off at 0.32 OD at a calculated specificity of 98% and a sensitivity of 94%. Running the optimized test showed that serum IgG autoantibodies from 47 EBA (94%; 95% CI: 87.41%-100%, 2 CD (4%; 95% CI: 0%-9.43%, 8 UC (16%; 95% CI: 5.8%-26%, 2 BP (2.63%; 95% CI: 0%-6.23%, and 4 PV (9.52%; 95% CI: 0%-18.4% patients as well as from 4 (1.63%; 95% CI: 0%-3.21% healthy donors reacted with the chimeric protein. Further analysis revealed that in 34%, 37%, 16% and 100% of sera autoantibodies of IgG1, IgG2, IgG3, and IgG4 isotype, respectively, recognized the recombinant autoantigen. Conclusions Using a chimeric protein, we developed a new sensitive and specific ELISA to detect collagen specific antibodies. Our results show a low prevalence of collagen VII-specific autoantibodies in inflammatory bowel disease, pemphigus and bullous pemphigoid. Furthermore, we show that the autoimmune response against collagen VII is dominated by IgG4 autoantibodies. The new immunoassay should

  7. The lesional skin of linear IgA bullous dermatosis expresses growth-regulated peptide (GRO)-alpha.

    Science.gov (United States)

    Tsunemi, Yuichiro; Ihn, Hironobu; Saeki, Hidehisa; Tamaki, Kunihiko

    2004-07-01

    The patient was a 62-year-old man with erythema with tense vesiculobullae and erosions on the bilateral elbows, right knee, and one buttock. A skin biopsy specimen revealed subepidermal blister formation with a predominant infiltration of neutrophils and papillary neutrophilic microabscesses. Direct immunofluorescence study showed linear deposition of IgA and weak deposition of IgG at the basement membrane zone of the lesional skin, and indirect immunofluorescence study showed linear deposition of IgA at the epidermal side of the 1M NaCl-separated normal skin. He was diagnosed with linear IgA bullous dermatosis. Immunohistochemical study revealed that the lesional and perilesional keratinocytes expressed growth-regulated peptide (GRO) -alpha, a potent chemoattractant for neutrophils. This suggests that GRO-alpha plays a role in the infiltration of neutrophils into the lesional skin and in bulla formation in linear IgA bullous dermatosis.

  8. 水疱型毛母质瘤1例%Pilomatricoma with Bullous Appearance:A Case Report

    Institute of Scientific and Technical Information of China (English)

    孔玉龙; 王克玉

    2014-01-01

    患者,男,12岁。右侧颈部及右上臂囊性肿物2年。皮损组织病理示:表皮正常,真皮内见镜影细胞及嗜碱性粒细胞浸润,可见多处钙化区,其周围被纤维组织包绕。诊断:水疱型毛母质瘤。%A 12-year-old male patient presented with purplish red bullous on right neck and right upper arm. The histopathology of the skin lession showed a normal epidermis. Shadow cells and basophilic cell infil-tration with calcifying areas, surrounded by fibrous tissue. He was diagnosed as pilomatricoma with bullous appearance.

  9. Electrosmog and autoimmune disease.

    Science.gov (United States)

    Marshall, Trevor G; Heil, Trudy J Rumann

    2016-07-13

    Studies in mice have shown that environmental electromagnetic waves tend to suppress the murine immune system with a potency similar to NSAIDs, yet the nature of any Electrosmog effects upon humans remains controversial. Previously, we reported how the human Vitamin-D receptor (VDR) and its ligand, 1,25-dihydroxyvitamin-D (1,25-D), are associated with many chronic inflammatory and autoimmune diseases. We have shown how olmesartan, a drug marketed for mild hypertension, acts as a high-affinity partial agonist for the VDR, and that it seems to reverse disease activity resulting from VDR dysfunction. We here report that structural instability of the activated VDR becomes apparent when observing hydrogen bond behavior with molecular dynamics, revealing that the VDR pathway exhibits a susceptibility to Electrosmog. Further, we note that characteristic modes of instability lie in the microwave frequency range, which is currently populated by cellphone and WiFi communication signals, and that the susceptibility is ligand dependent. A case series of 64 patient-reported outcomes subsequent to use of a silver-threaded cap designed to protect the brain and brain stem from microwave Electrosmog resulted in 90 % reporting "definite" or "strong" changes in their disease symptoms. This is much higher than the 3-5 % rate reported for electromagnetic hypersensitivity in a healthy population and suggests that effective control of environmental Electrosmog immunomodulation may soon become necessary for successful therapy of autoimmune disease.

  10. [Hydroxychloroquine for autoimmune diseases].

    Science.gov (United States)

    Danza, Álvaro; Graña, Diego; Goñi, Mabel; Vargas, Andrea; Ruiz-Irastorza, Guillermo

    2016-02-01

    Hydroxychloroquine (HCQ) is by far the most frequently used antimalarial for the management of Systemic Autoimmune Diseases. It has immunomodulatory, hypolipidemic, hypoglycemic and antithrombotic properties and it diminishes the risk of malignancies. The most important mechanisms to explain the immunomodulatory actions are its ability to reduce inflammatory pathways and Toll-like receptors activation. The safety profile is favorable. In spite of its low frequency, retinal toxicity is potentially severe. In systemic lupus erythematous HCQ therapy reduces activity, the accrual of organ damage, risk of infections and thrombosis and improves the cardiometabolic profile. It contributes to induce lupus nephritis remission, spares steroid use and increases survival rates. In rheumatoid arthritis, it improves cardiometabolic risk and has a favorable effect in joint inflammation. In Sjögren's syndrome, an increased lacrimal quality as well as an improvement in objective and subjective inflammatory markers has been demonstrated with HCQ. In Antiphospholipid Syndrome, HCQ is effective in primary and secondary thrombosis prevention. The effectiveness of the drug in other systemic autoimmune diseases is less established. HCQ therapy may improve dermatological manifestations in Dermatomyositis and may have a positive effects in the treatment of Sarcoidosis and Still disease.

  11. Is Tolerance Broken in Autoimmunity?

    Directory of Open Access Journals (Sweden)

    Dama Laxminarayana

    2017-11-01

    Full Text Available Autoimmune diseases are classified into about 80 different types based on their specificity related to system, organ and/or tissue. About 5% of the western population is affected by this anomaly, but its worldwide incidence is unknown. Autoimmune diseases are heterogeneous in nature and clinical manifestations range from benign disorders to life-threatening conditions. Autoimmunity strikes at any stage of life, but age and/or gender also play role in onset of some of these anomalies. The autoimmune pathogenesis is initiated by the origination of autoantigens, which leads to the development of autoantibodies followed by auto-immunogenicity and the ultimate onset of autoimmunity. There is a lack of suitable therapies to treat autoimmune diseases, because mechanisms involved in the onset of these anomalies were poorly understood. Present therapies are limited to symptomatic treatment and come with severe side effects. Here, I described the molecular mechanisms and cellular events involved in the initiation of autoimmunity and proposed better strategies to modulate such molecular and cellular anomalies, which will help in preventing and/or controlling autoimmune pathogenesis and ultimately aid in enhancing the quality of life.

  12. A CONTROVERSIAL ON THE DIAGNOSIS OF CHRONIC BULLOUS TYPE MUCOCUTANEOUS DISEASE INVOLVING ORAL MUCOSA (A CASE REPORT)

    OpenAIRE

    Isadora Gracia; Harum Sasanti

    2006-01-01

    A case of chronic bullous type mucocutaneous disease involving oral mucosa was reported from a 56 years old man with never healing oral ulcers and wound on the perianal skin for three years. There were also red and black spots on the limb and back skin and a lesion on nail. Painful oral lesion consisted of mucous erosion, desquamative gingivitis, and sloughing area on palate and tongue. The patient is diabetic. The first perianal skin diagnosis was granulomatous candidasis with differential d...

  13. Aetiopathogenesis of autoimmune hepatitis

    Institute of Scientific and Technical Information of China (English)

    Diego Vergani; Giorgina Mieli-Vergani

    2008-01-01

    The histological hallmark of autoimmune hepatitis (AIH) is a dense portal mononuclear cell infiltrate that invades the surrounding parenchyma and comprises T and B lymphocytes,macrophages,and plasma cells.An unknown but powerful stimulus must be promoting the formation of this massive inflammatory cellular reaction that is likely to initiate and perpetuate liver damage.An autoimmune attack can follow different pathways to inflict damage on hepatocytes.Liver damage is likely to be orchestrated by CD4+T lymphocytes recognizing an autoantigenic liver peptide.To trigger an autoimmune response,the peptide must be embraced by an HLA class Ⅱ molecule and presented to naive CD4+T helper (Th0) cells by professional antigen presenting cells,with the co-stimulation of ligand-ligand fostering interaction between the two cells.Th0 cells become activated,differentiate into functional phenotypes according to the cytokines prevailing in the microenvironment and the nature of the antigen,and initiate a cascade of immune reactions determined by the cytokines produced by the activated T cells.Th1 cells,arising in the presence of the macrophage-derived interleukin (IL)-12,secrete mainly IL-2 and interferon-gamma (IFN-γ),which activate macrophages,enhance expression of HLA class Ⅰ (increasing liver cell vulnerability to a CD8+T cell cytotoxic attack),and induce expression of HLA class Ⅱ molecules on hepatocytes.Th2 cells,which differentiate from Th0 if the microenvironment is rich in IL-4,produce mainly IL-4,IL-10,and IL-13 which favour autoantibody production by B lymphocytes.Physiologically,Th1 and Th2 antagonize each other.Th17 cells,a recently described population,arise in the presence of transforming growth factor beta (TGF-β) and IL-6 and appear to have an important effector role in inflammation and autoimmunity.The process of autoantigen recognition is strictly controlled by regulatory mechanisms,such as those exerted by CD4+CD25+regulatory T cells,which derive from Th0

  14. Mucormycosis in systemic autoimmune diseases.

    Science.gov (United States)

    Royer, Mathieu; Puéchal, Xavier

    2014-07-01

    Mucormycosis is an emerging infection in systemic autoimmune diseases. All published cases of systemic autoimmune diseases complicated by mucormycosis were reviewed. The clinical features, diagnostic procedures and the main principles of treatment were analyzed. Twenty-four cases of mucormycosis have been reported in systemic auto-immune diseases, of which 83% in systemic lupus erythematosus, all occurring during immunosuppressants. In most cases, the infection was disseminated or rhinocerebral and it had mimicked a flare of the underlying connective tissue disease. A fatal outcome was reported in 58.3% of these patients. In conclusion, mucormycosis often mimics a flare of the underlying systemic disease and is associated with a high mortality rate. Systemic lupus erythematosus is by far the most common associated systemic autoimmune disease. A high degree of awareness is warranted to rapidly rule out infection, of which mucormycosis, in immunocompromised patients with systemic autoimmune disease before a disease flare is conclusively diagnosed.

  15. Spontaneous germinal centers and autoimmunity.

    Science.gov (United States)

    Domeier, Phillip P; Schell, Stephanie L; Rahman, Ziaur S M

    2017-02-01

    Germinal centers (GCs) are dynamic microenvironments that form in the secondary lymphoid organs and generate somatically mutated high-affinity antibodies necessary to establish an effective humoral immune response. Tight regulation of GC responses is critical for maintaining self-tolerance. GCs can arise in the absence of purposeful immunization or overt infection (called spontaneous GCs, Spt-GCs). In autoimmune-prone mice and patients with autoimmune disease, aberrant regulation of Spt-GCs is thought to promote the development of somatically mutated pathogenic autoantibodies and the subsequent development of autoimmunity. The mechanisms that control the formation of Spt-GCs and promote systemic autoimmune diseases remain an open question and the focus of ongoing studies. Here, we discuss the most current studies on the role of Spt-GCs in autoimmunity.

  16. Study on dermatoses and their prevalence in groups of confirmed alcoholic individuals in comparison to a non-alcoholic group of individuals Estudo das dermatoses e sua prevalencia em individuos comprovadamente alcoolistas comparativamente a um grupo de individuos não alcoolistas

    Directory of Open Access Journals (Sweden)

    Maria Cecilia Teixeira de Carvalho Bruno

    2013-06-01

    Full Text Available BACKGROUND: The direct relationship between alcoholism and dermatoses has been evaluated in recent researches. However, there are few objective surveys that demonstrate and prove a direct relationship between alcohol and a specific dermatosis. OBJECTIVES: to verify the prevalence of dermatoses in alcoholics, analize the dermatological changes found in these patients and their evolution during alcoholic abstinence. Also, to compare the results obtained with a non-alcoholic control group and with the data found in medical literature. METHODS: the dermatologic conditions of 278 alcoholic patients (250 men, 28 women were studied over a period of 4 years, and compared to those of a control group of 271 non-alcoholic individuals (249 men, 22 women, members of the Military Police Force. The individuals in both groups were between 20 and 60 years old. RESULTS: Pellagra, nummular eczema, purpura pigmentosa chronica (also known as pigmented purpuric dermatosis and psoriasis were more frequent in the group of alcoholics and, apparently, occurred in parallel with alcoholism that seems to play a role in the evolution of these dermatoses. The dermatopathies were more frequent before the age of forty, regardless of factors such as profession, race or gender. CONCLUSION: the association of dermatoses and alcoholism was extremely significant according to the statistical data. Alcoholism can be considered a risk factor for pellagra, psoriasis, nummular eczema and purpura pigmentosa chronica dermatoses, which can, as well, be considered alcoholism indicators. FUNDAMENTOS: A relação direta entre o alcoolismo e as dermatoses vem sendo avaliada em recentes pesquisas. No entanto são poucos os estudos objetivos que demonstram e comprovam uma relação direta entre o álcool e uma determinada dermatose. OBJETIVOS: Verificar a prevalência de dermatoses em alcoolistas, avaliar as alterações dermatológicas encontradas nesses doentes e sua evolução frente abstin

  17. Endocrine autoimmunity in Turner syndrome

    Science.gov (United States)

    2013-01-01

    Background Turner syndrome is caused by numeric and structural abnormalities of the X chromosome. An increased frequency of autoimmunity as well as an elevated incidence of autoantibodies was observed in Turner patients. The aim of this study was to conduct a retrospective analysis of the incidence of autoimmunity in 66 Italian patients affected by Turner syndrome. Methods Sixty-six unselected and consecutive Italian Turner patients were recruited. The association between age, karyotype and the presence of clinical/pre-clinical autoimmune disorders and of autoantibodies was examined. Results Out of the 66 Turner patients, 26 had thyroid autoimmune disorders (39.4%), 14 patients had Hashimoto’s thyroiditis with clinical or subclinical hypothyroidism (21.2%) and 12 patients had circulating anti-thyroid antibodies, echographic pattern of diffuse hypoechogenicity and normal thyroid hormone levels (18.2%). None were affected by Graves’ disease. We analyzed the overall incidence of thyroid autoimmunity within the 3 different age groups 0–9.9, 10–19.9 and 20–29.9 years. No statistically significant difference was observed in the incidence of thyroid autoimmunity within the age-groups (χ2-test p > 0.05). Out of the 66 patients, 31 patients had the 45,X karyotype; within this first group 14 out of 31 patients were affected by autoimmune thyroid disease. A second group of 29 patients included 19 patients with mosaicism, 5 patients with deletions and 5 patients with ring chromosome; out of these 29 patients 7 were affected by autoimmune thyroid disease. A third group included 6 patients with X isochromosome; 5 out of 6 were affected by autoimmune thyroid disease. A statistically significant difference in the frequency of thyroid autoimmunity within the different karyotype groups was observed (χ2-test p = 0.0173). When comparing the X isochromosome group with the pooled group of other karyotypes, of note, the frequency of thyroid autoimmunity was

  18. Adult autoimmune enteropathy

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    Recent reports have suggested that autoimmune enteropathy involving the small bowel may occur in adults as well as in children. Apparently, the endoscopic and histological changes are similar to celiac disease before treatment, but these are not altered by any form of dietary restriction, including a gluten-free diet. As in celiac disease, histologic changes in gastric and colonic biopsies have also been recorded. Anti enterocyte antibodies detected with immunofluorescent methods have been reported by a few laboratories, but these antibodies appear not to be specific and may simply represent epiphenomena. A widely available, reproducible and quantitative anti-enterocyte antibody assay is needed that could be applied in small bowel disorders that have the histological appearance of celiac disease, but fail to respond to a gluten-free diet.

  19. SOCS, inflammation and autoimmunity

    Directory of Open Access Journals (Sweden)

    Akihiko eYoshimura

    2012-03-01

    Full Text Available Cytokines play essential roles in innate and adaptive immunity. However, excess cytokines or dysregulation of cytokine signaling can cause a variety of diseases, including allergies, autoimmune diseases, inflammation, and cancer. Most cytokines utilize the so-called Janus kinase-signal transducers and activators of transcription (JAK-STAT pathway. This pathway is negatively regulated by various mechanisms including suppressors of cytokine signaling (SOCS proteins. SOCS proteins bind to JAK or cytokine receptors, thereby suppressing further signaling events. Especially, SOCS1 and SOCS3 are strong inhibitors of JAK, because these two contain kinase inhibitory region (KIR at the N-terminus. Studies using conditional knockout mice have shown that SOCS proteins are key physiological as well as pathological regulators of immune homeostasis. Recent studies have also demonstrated that SOCS1 and SOCS3 are important regulators of helper T cell differentiation and functions.

  20. Psychoneuroimmunology - psyche and autoimmunity.

    Science.gov (United States)

    Ziemssen, Tjalf

    2012-01-01

    Psychoneuroimmunology is a relatively young field of research that investigates interactions between central nervous and immune system. The brain modulates the immune system by the endocrine and autonomic nervous system. Vice versa, the immune system modulates brain activity including sleep and body temperature. Based on a close functional and anatomical link, the immune and nervous systems act in a highly reciprocal manner. From fever to stress, the influence of one system on the other has evolved in an intricate manner to help sense danger and to mount an appropriate adaptive response. Over recent decades, reasonable evidence has emerged that these brain-to-immune interactions are highly modulated by psychological factors which influence immunity and autoimmune disease. For several diseases, the relevance of psychoneuroimmunological findings has already been demonstrated.

  1. Update on Autoimmune Hepatitis.

    Science.gov (United States)

    Liberal, Rodrigo; Vergani, Diego; Mieli-Vergani, Giorgina

    2015-03-01

    Autoimmune hepatitis (AIH), a liver disorder affecting both children and adults, is characterized by inflammatory liver histology, elevated transaminase levels, circulating nonorganspecific autoantibodies, and increased levels of immunoglobulin G, in the absence of a known etiology. Two types of AIH are recognized according to seropositivity: smooth muscle antibody and/or antinuclear antibody define AIH type 1 and antibodies to liver-kidney microsome type 1 and/or liver cytosol type 1 define AIH type 2. AIH type 1 affects both adults and children, while AIH type 2 is mainly a paediatric disease, though it does occasionally affects young adults. AIH should be considered during the diagnostic workup of any patient with increased liver enzyme levels. AIH is exquisitely responsive to immunosuppressive treatment with prednisolone with or without azathioprine, with symptom free long-term survival for the majority of patients. For those who do not respond to standard treatment, or who are difficult-to-treat, mycophenolate mofetil and, in the absence of a response, calcineurin inhibitors should be tried in addition to steroids. The pathogenesis of AIH is not fully understood, although there is mounting evidence that genetic susceptibility, molecular mimicry and impaired immunoregulatory networks contribute to the initiation and perpetuation of the autoimmune attack. Liver damage is thought to be mediated primarily by CD4 T-cells, although recent studies support the involvement of diverse populations, including Th17 cells. A deeper understanding of the pathogenesis of AIH is likely to contribute to the development of novel treatments, such as the adoptive transfer of autologous expanded antigenspecific regulatory T-cells, which ultimately aim at restoring tolerance to liver-derived antigens.

  2. Pathophysiology of autoimmune polyneuropathies.

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    Dalakas, Marinos C

    2013-06-01

    The most common autoimmune neuropathies include the acute inflammatory polyneuropathy [the Guillain-Barré Syndrome(s)]; chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN) and IgM anti-MAG-antibody mediated paraproteinemic neuropathy. These neuropathies occur when immunologic tolerance to peripheral nerve components (myelin, Schwann cell, axon, and motor or ganglionic neurons) is lost. Based on the immunopathologic similarities with experimental allergic neuritis induced after immunization with nerve proteins, disease transfer experiments with the patients' serum or with intraneural injections, and immunocytochemical studies on the patients' nerves, it appears that both cellular and humoral factors, either independently or in concert with each other, play a role in the cause of these neuropathies. Although in some of them there is direct evidence for autoimmune reactivity mediated by specific antibodies or autoreactive T lymphocytes, in others the underlying immune-mediated mechanisms have not been fully elucidated, in spite of good response to immunotherapies. The review highlights the factors associated with breaking the T-cell tolerance, the T-cell activation and costimulatory molecules, the immunoregulatory T-cells and relevant cytokines and the antibodies against peripheral nerve glycolipids or glycoproteins that seem to be of pathogenic relevance. Antigens in the nodal, paranodal and juxtaparanodal regions are discussed as potentially critical targets in explaining conduction failure and rapid recovery. Based on the immunopathologic network believed to play a fundamental role in the pathogenesis of these neuropathies, future therapeutic directions are highlighted using new biological agents against T-cells, cytokines, B-cells, transmigration and transduction molecules.

  3. Selfie: Autoimmunity, boon or bane.

    Science.gov (United States)

    Ahsan, Haseeb

    2017-01-01

    The immune system provides protection to tissues damaged by infectious microrganisms or physical damage. In autoimmune diseases, the immune system recognizes and attacks its own tissues, i.e., self-destruction. Various agents such as genetic factors and environmental triggers are thought to play a major role in the development of autoimmune diseases. A common feature of all autoimmune diseases is the presence of autoantibodies and inflammation, including mononuclear phagocytes, autoreactive T lymphocytes, and autoantibody producing B cells (plasma cells). It has long been known that B cells produce autoantibodies and, thereby, contribute to the pathogenesis of many autoimmune diseases. Autoimmune diseases can be classified as organ-specific or non-organ specific depending on whether the autoimmune response is directed against a particular tissue or against widespread antigens as in chronic inflammatory autoimmune diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). Both SLE and RA are characterized by the presence of autoantibodies which play a major role in their etiopathogenesis. SLE is characterized by circulating antibodies and immune complex deposition that can trigger an inflammatory damage in organs. RA is a progressive inflammatory disease in which T cells, B cells, and pro-inflammatory cytokines play a key role in its pathophysiology.

  4. Type 1 diabetes associated autoimmunity.

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    Kahaly, George J; Hansen, Martin P

    2016-07-01

    Diabetes mellitus is increasing in prevalence worldwide. The economic costs are considerable given the cardiovascular complications and co-morbidities that it may entail. Type 1 diabetes (T1D) is a chronic autoimmune disease characterized by the loss of insulin-producing pancreatic β-cells. The pathogenesis of T1D is complex and multifactorial and involves a genetic susceptibility that predisposes to abnormal immune responses in the presence of ill-defined environmental insults to the pancreatic islets. Genetic background may affect the risk for autoimmune disease and patients with T1D exhibit an increased risk of other autoimmune disorders such as autoimmune thyroid disease, Addison's disease, autoimmune gastritis, coeliac disease and vitiligo. Approximately 20%-25% of patients with T1D have thyroid antibodies, and up to 50% of such patients progress to clinical autoimmune thyroid disease. Approximately 0.5% of diabetic patients have concomitant Addison's disease and 4% have coeliac disease. The prevalence of autoimmune gastritis and pernicious anemia is 5% to 10% and 2.6% to 4%, respectively. Early detection of antibodies and latent organ-specific dysfunction is advocated to alert physicians to take appropriate action in order to prevent full-blown disease. Patients and family members should be educated to be able to recognize signs and symptoms of underlying disease. Copyright © 2016 Elsevier B.V. All rights reserved.

  5. Development of autoimmunity in lymphoma.

    Science.gov (United States)

    Jardin, Fabrice

    2008-03-01

    Development of lymphoproliferative diseases during the course of autoimmune and chronic inflammatory conditions is well established. Conversely, development of clinical or biological signs of autoimmunity at the time of the diagnosis of lymphoma or during its course indicates that lymphoma and autoimmune manifestations may constitute two faces of the same process. The aim of this review is to describe autoimmune manifestations related to non-Hodgkin's lymphoma and Hodgkin's lymphoma, their specificity according to the lymphoma subtype and their physiopathological signification. Lymphoma-related autoimmune manifestations include mainly skin diseases, hematological manifestations, rheumatic diseases and renal lesions. Despite the lack of studies providing a systematic prospective assessment, autoimmune manifestations are observed in all lymphoma subtypes and seem particularly prevalent in marginal-zone lymphoma and T-cell lymphoma. Autoimmune manifestation's physiopathology may implicate production of autoantibodies by CD5-positive autoreactive B cells, a loss of immune tolerance, an alteration of the Fas/Fas-ligand pathway and/or a chronic antigenic stimulation. Monoclonal antibodies (including rituximab, Campath-1H or epratuzumab) constitute the most promising approach to treat lymphoma-related immune disorders.

  6. Recurrent rates and risk factors associated with recurrent painful bullous keratopathy after primary phototherapeutic keratectomy

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    Kasetsuwan N

    2015-09-01

    Full Text Available Ngamjit Kasetsuwan, Kanokorn Sakpisuttivanit, Usanee Reinprayoon, Vilavun Puangsricharern Department of Ophthalmology, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Bangkok, Thailand Objective: To assess the recurrent rate, mean survival time, and risk factors associated with recurrent painful bullous keratopathy (BK after primary treatment with phototherapeutic keratectomy.Methods: Medical records from 72 patients (72 eyes who had phototherapeutic keratectomy for painful BK were evaluated. Data for sex, age, duration of BK, associated ocular and systemic diseases (hypertension, diabetes mellitus, ischemic heart disease, asthma, dyslipidemia, and rheumatoid arthritis, frequency and degree of pain (grade 1–3, visual acuity, corneal thickness, intraocular pressure, and laser setting were extracted and analyzed.Results: The mean age of the patients was 64.2±11.4 years. The mean preoperative duration of BK was 15.0±11.0 months. Most patients had pseudophakic BK (69.40%. Majority of the cases had grade 3 degree of pain (48.60%. Glaucoma and hypertension were markedly found among these patients (51.40% and 19.40%, respectively. Preoperative mean intraocular pressure and corneal thickness were 13.70±4.95 mmHg and 734.1±83.80 µm, respectively. The mean laser diameter and depth were 8.36±1.22 mm and 38.89±8.81 µm, respectively. Systemic disease was significantly associated with the risk for developing recurrent painful BK (P=0.022, hazard ratio [HR] 1.673, 95% confidence interval [CI] 1.08–2.58. The overall recurrent rate was 51%. The average duration time of recurrent painful BK was 17.3±12.9 months (range 1–50 months. The median survival time before recurrence was 29.0±6.6 months.Conclusion: Systemic disease was found to be the only risk factor significantly associated with the development of recurrent painful BK. Low recurrent rate and long mean survival time showed that phototherapeutic

  7. Questions and Answers on Autoimmunity and Autoimmune Diseases

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    ... Autoimmune Coalition Your Privacy Get Involved Donate Grassroots Fundraising ? Advocate for Change Take our Survey Information List ... Common Thread Coping Tools InFocus Newsletter Questions & Answers Fundraising Grassroots Fundraising Workplace Giving Special Events AARDA on ...

  8. [Autoimmune pancreatitis as an element of autoimmune polyglandular syndrome].

    Science.gov (United States)

    Dyrla, Przemysław; Nowak, Tomasz; Gil, Jerzy; Adamiec, Cezary; Bobula, Mariusz; Saracyn, Marek

    2016-05-01

    Autoimmune pancreatitis constantly belongs to diseases which often causes significant diagnostic problem and often runs out with surgical intervention as considered to be a pancreatic cancer. Important although usually underestimated problems are polyglandular syndromes, which may consist of autoimmune pancreatitis (AIP) problem as well. This case report is an example of autoimmune polyglandular syndrome (APS), which was connected with the surgical treatment with biliary bypass anastomosis because of the unresectable lesion in the head of pancreas. The definite remission of the pancreatic lesion finally came after a steroid therapy. Differentiation between neoplastic and inflammatory pancreatic tumors very often remains a serious clinical problem. On grounds of imaging and cytopathology exams it is often difficult to decide about the nature of a lesion. The negative result of cytopathological biopsy examination does not finally settle straightforward diagnosis. Diagnostic problems affect also autoimmune pancreatitis. It is worth to undertake attempts to differentiate pancreatic lesions especially in cases of concomitance with other autoimmune polyglandular syndromes. That is because it is connected with completely different treatment and outcome. We should remember about diagnostic criteria of autoimmune pancreatitis. Appropriate diagnosis for patients with AIP gives them a chance to avoid serious surgical resection and possible complications.

  9. One Case Report of Linear IgA Bullous Dermatosis and Literature Review%线状 IgA 大疱性皮肤病1例报道并文献复习

    Institute of Scientific and Technical Information of China (English)

    乔丽; 杨志勇; 田蓉

    2015-01-01

    目的:探讨线状 IgA 大疱性皮肤病(LABD)的病因、诊断和鉴别诊断、治疗及预后,以提高对该病的认识。方法对1例 LABD 患者的临床资料、组织病理、免疫荧光进行分析,并对相关文献进行分析。结果患者表现在红斑基础上的水疱,或外观正常皮肤上出现的水疱,病理组织活检和免疫荧光确诊为 LABD。结论 LABD 是一种少见的自身免疫性表皮下大疱病,确切的发病机制还不清楚。常见的靶抗原有 LAD97,LAD -1,BP180,BP230和 LAD285等,其中 BP180抗原可能在发病中起作用。特征性的基底膜带线状 IgA 抗体沉积为确诊本病的主要依据。氨苯砜是治疗 LABD 的首选药物。%Objective To investigate the etiology, diagnosis and differential diagnosis, treatment and prognosis of linear IgA bullous der-matosis(LABD) for improving the cognition on this disease. Methods The clinical, histopathological and immunofluorescence data in one case of LABD were analyzed and the relevant literature was analyzed. Results The patient with LABD showed blisters on the basis of erythema or blisters appeared on the skin with normal appearance. The histopathological and immunofluorescence biopsy diagnosed as LABD. Conclusion LABD is a rare autoimmune subepidermal bullous disease. The common target antigens are LAD97, LAD - 1, BP180, BP230, LAD285, etc.,in which BP180 antigen may play a role in the pathogenesis. The characteristic basement membrane linear IgA an-tibody deposition is the main basis for diagnosing LABD. Dapsone is the drug of first choice for treating LABD.

  10. Linear IgA/IgG bullous dermatosis reacts with multiple laminins and integrins.

    Science.gov (United States)

    Li, Xiaoguang; Tsuchisaka, Atsunari; Qian, Hua; Teye, Kwesi; Ishii, Norito; Sogame, Ryosuke; Harada, Kazutoshi; Nakagomi, Daiki; Shimada, Shinji; Tateishi, Chiharu; Hirako, Yoshiaki; Hashimoto, Takashi

    2015-01-01

    Since the original description by Zone et al in 1994, the disease entity and target antigens in linear IgA/IgG bullous dermatosis (LAGBD) have not been clarified in 20 years. To determine autoantibodies and autoantigens in a new LAGBD case which showed atypical clinical and histopathological findings without apparent mucosal involvement. We performed various indirect immunofluorescence and immunoblotting studies. Indirect immunofluorescence of 1M NaCl-split skin showed IgG and IgA reactivity with both epidermal and dermal sides. Immunoblotting studies using various antigen sources revealed circulating IgG and IgA antibodies reactive with laminin-332, laminin-γ1 and integrin α6β4 in various patterns. Absorption study using recombinant proteins of laminin-γ1 indicated that the patient serum reacted with different epitopes between laminin-γ1 and laminin-γ2. This study presented for the first time a LAGBD patient with IgG and IgA antibodies to various laminins and integrins.

  11. Unilateral bullous pemphigoid without erythema and eosinophil infiltration in a hemiplegic patient.

    Science.gov (United States)

    Tsuruta, Daisuke; Nishikawa, Takeji; Yamagami, Jun; Hashimoto, Takashi

    2012-09-01

    In this report, we describe an 88-year-old male stroke patient with unilateral bullous pemphigoid limited to the hemiplegic side. Physical examinations revealed multiple tense bullae with clear and/or bloody contents without apparent erythema on the right thigh and lower leg, accompanied by erosions on the right chest. Histopathologically, no eosinophils were infiltrated into and around the subepidermal bullae. Immunofluorescence revealed deposited and circulating immunoglobulin (Ig)G anti-basement membrane zone antibodies. Immunoblot assays using various antigen sources and enzyme-linked immunosorbent assay revealed that IgG antibodies in this case reacted with unique epitopes between NC16a and C-terminal domains on the 120-kDa LAD-1, the extracellular truncated form of BP180. Three observations were unique in our case. First, the distribution of bullae in our patient was limited to the hemiplegic side. Second, there was no apparent erythema clinically and no eosinophilic infiltration histopathologically. Third, the patient achieved remission without the use of oral corticosteroids. The unusual epitopes in this case may contribute to these phenomena.

  12. Sex differences in autoimmune diseases

    Directory of Open Access Journals (Sweden)

    Voskuhl Rhonda

    2011-01-01

    Full Text Available Abstract Women are more susceptible to a variety of autoimmune diseases including systemic lupus erythematosus (SLE, multiple sclerosis (MS, primary biliary cirrhosis, rheumatoid arthritis and Hashimoto's thyroiditis. This increased susceptibility in females compared to males is also present in animal models of autoimmune diseases such as spontaneous SLE in (NZBxNZWF1 and NZM.2328 mice, experimental autoimmune encephalomyelitis (EAE in SJL mice, thyroiditis, Sjogren's syndrome in MRL/Mp-lpr/lpr mice and diabetes in non-obese diabetic mice. Indeed, being female confers a greater risk of developing these diseases than any single genetic or environmental risk factor discovered to date. Understanding how the state of being female so profoundly affects autoimmune disease susceptibility would accomplish two major goals. First, it would lead to an insight into the major pathways of disease pathogenesis and, secondly, it would likely lead to novel treatments which would disrupt such pathways.

  13. Epigenetic alterations underlying autoimmune diseases.

    Science.gov (United States)

    Aslani, Saeed; Mahmoudi, Mahdi; Karami, Jafar; Jamshidi, Ahmad Reza; Malekshahi, Zahra; Nicknam, Mohammad Hossein

    2016-01-01

    Recent breakthroughs in genetic explorations have extended our understanding through discovery of genetic patterns subjected to autoimmune diseases (AID). Genetics, on the contrary, has not answered all the conundrums to describe a comprehensive explanation of causal mechanisms of disease etiopathology with regard to the function of environment, sex, or aging. The other side of the coin, epigenetics which is defined by gene manifestation modification without DNA sequence alteration, reportedly has come in to provide new insights towards disease apprehension through bridging the genetics and environmental factors. New investigations in genetic and environmental contributing factors for autoimmunity provide new explanation whereby the interactions between genetic elements and epigenetic modifications signed by environmental agents may be responsible for autoimmune disease initiation and perpetuation. It is aimed through this article to review recent progress attempting to reveal how epigenetics associates with the pathogenesis of autoimmune diseases.

  14. Autoimmune Hepatitis and PSC Connection.

    Science.gov (United States)

    Vergani, Diego; Mieli-Vergani, Giorgina

    2008-02-01

    This article describes the connection between autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC). The two conditions have chronicity, liver inflammation, and a positive autoimmune serology in common; they differ in terms of gender distribution and bile duct damage. There is evidence suggesting that AIH and PSC are immune-mediated diseases. PSC and AIH could lie within the spectrum of the same disease process. Future studies should determine how frequently AIH evolves to PSC.

  15. [Sexuality and auto-immunity].

    Science.gov (United States)

    Abraham, Georges; Vlatkovic, Dejan

    2010-03-24

    The idea that it might be a link between auto-immune affections and sexual disturbances could appear a vain purpose at a first glance. Nevertheless, as we start from a new point of view, it is understandable that we focus on a possible common tendency to develop self-aggression and self-destruction. Similarities which could play a role in the development of an auto-immune disease and of a sexual dixturbance as well.

  16. Xenobiotic Exposure and Autoimmune Hepatitis

    Directory of Open Access Journals (Sweden)

    Kathleen M. Gilbert

    2010-01-01

    Full Text Available Although genetics contributes to the development of autoimmune diseases, it is clear that “environmental” factors are also required. These factors are thought to encompass exposure to certain drugs and environmental pollutants. This paper examines the mechanisms that normally maintain immune unresponsiveness in the liver and discusses how exposure to certain xenobiotics such as trichloroethylene may disrupt those mechanisms and promote autoimmune hepatitis.

  17. Hashimoto's Thyroiditis and Autoimmune Gastritis.

    Science.gov (United States)

    Cellini, Miriam; Santaguida, Maria Giulia; Virili, Camilla; Capriello, Silvia; Brusca, Nunzia; Gargano, Lucilla; Centanni, Marco

    2017-01-01

    The term "thyrogastric syndrome" defines the association between autoimmune thyroid disease and chronic autoimmune gastritis (CAG), and it was first described in the early 1960s. More recently, this association has been included in polyglandular autoimmune syndrome type IIIb, in which autoimmune thyroiditis represents the pivotal disorder. Hashimoto's thyroiditis (HT) is the most frequent autoimmune disease, and it has been reported to be associated with gastric disorders in 10-40% of patients while about 40% of patients with autoimmune gastritis also present HT. Some intriguing similarities have been described about the pathogenic mechanism of these two disorders, involving a complex interaction among genetic, embryological, immunologic, and environmental factors. CAG is characterized by a partial or total disappearance of parietal cells implying the impairment of both hydrochloric acid and intrinsic factor production. The clinical outcome of this gastric damage is the occurrence of a hypochlorhydric-dependent iron-deficient anemia, followed by pernicious anemia concomitant with the progression to a severe gastric atrophy. Malabsorption of levothyroxine may occur as well. We have briefly summarized in this minireview the most recent achievements on this peculiar association of diseases that, in the last years, have been increasingly diagnosed.

  18. Autoimmune NMDA receptor encephalitis.

    Science.gov (United States)

    Lazar-Molnar, Eszter; Tebo, Anne E

    2015-01-01

    Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is a treatable autoimmune disease of the central nervous system (CNS) with prominent neurologic and psychiatric features at disease onset. The disease is associated with the production of autoantibodies to NMDAR, a protein involved in memory function and synaptic plasticity. Affected patients develop a multistage progressive illness with symptoms ranging from memory deficits, seizures and psychosis, to potentially lethal catatonia, and autonomic and breathing instability. The outcome can be much improved with accurate diagnosis and early treatment using adequate immunosuppressive therapy. However, since the neurological and psychiatric symptoms as well as the clinical examination results can be non-specific, the disease is probably under-recognized. Reliable and accurate clinical testing for the identification of NMDAR autoantibodies is crucial for diagnosis, timely treatment selection, and monitoring. Recently, a cell-based indirect immunofluorescent antibody test for the detection of IgG antibodies to NMDAR has become available for diagnostic use. This review highlights the progress and challenges of laboratory testing in the evaluation and management anti-NMDAR encephalitis, and perspectives for the future.

  19. CD8+ T-Cell Deficiency, Epstein-Barr Virus Infection, Vitamin D Deficiency, and Steps to Autoimmunity: A Unifying Hypothesis

    Directory of Open Access Journals (Sweden)

    Michael P. Pender

    2012-01-01

    Full Text Available CD8+ T-cell deficiency is a feature of many chronic autoimmune diseases, including multiple sclerosis, rheumatoid arthritis, systemic lupus erythematosus, Sjögren's syndrome, systemic sclerosis, dermatomyositis, primary biliary cirrhosis, primary sclerosing cholangitis, ulcerative colitis, Crohn's disease, psoriasis, vitiligo, bullous pemphigoid, alopecia areata, idiopathic dilated cardiomyopathy, type 1 diabetes mellitus, Graves' disease, Hashimoto's thyroiditis, myasthenia gravis, IgA nephropathy, membranous nephropathy, and pernicious anaemia. It also occurs in healthy blood relatives of patients with autoimmune diseases, suggesting it is genetically determined. Here it is proposed that this CD8+ T-cell deficiency underlies the development of chronic autoimmune diseases by impairing CD8+ T-cell control of Epstein-Barr virus (EBV infection, with the result that EBV-infected autoreactive B cells accumulate in the target organ where they produce pathogenic autoantibodies and provide costimulatory survival signals to autoreactive T cells which would otherwise die in the target organ by activation-induced apoptosis. Autoimmunity is postulated to evolve in the following steps: (1 CD8+ T-cell deficiency, (2 primary EBV infection, (3 decreased CD8+ T-cell control of EBV, (4 increased EBV load and increased anti-EBV antibodies, (5 EBV infection in the target organ, (6 clonal expansion of EBV-infected autoreactive B cells in the target organ, (7 infiltration of autoreactive T cells into the target organ, and (8 development of ectopic lymphoid follicles in the target organ. It is also proposed that deprivation of sunlight and vitamin D at higher latitudes facilitates the development of autoimmune diseases by aggravating the CD8+ T-cell deficiency and thereby further impairing control of EBV. The hypothesis makes predictions which can be tested, including the prevention and successful treatment of chronic autoimmune diseases by controlling EBV infection.

  20. Dermatoses among children from celebration of “Holi,” the spring festival, in India: A cross-sectional observational study

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    Sudip Kumar Ghosh

    2016-01-01

    Full Text Available Background: “Holi” is a spring festival celebrated primarily in the Indian subcontinent and also abroad by expatriate Indians. It is a festival of colors, traditionally celebrated by mutual application of colors in different forms on a particular day of the year. These colors frequently comprise a range of synthetic dyes which have harmful effects on the skin and mucosae. Children take part in this colorful festival with much enthusiasm and vigor, making them prone to develop different “Holi”-related dermatoses. Our objective was to find out the different patterns of “Holi”-related dermatoses in a group of pediatric patients. Methodology: This was a cross-sectional descriptive study carried out over a period of 6 years (2010–2015. Consecutive patients of pediatric age group who attended dermatology outpatient department (OPD with different dermatoses following application of “Holi” color were included in this study. Results: A total of 63 patients (mean age 11 years; range 1–16 years were evaluated with a female to male ratio of 1.3:1. Itching is the predominant presenting symptom followed by burning sensation, dryness, scaling, oozing, and loss of hair. Examination revealed that eczematous lesion was the most common (69.8% reaction pattern followed by xerosis, desquamation, excoriation, erythema, morbilliform eruption, erosion, alopecia, ulceration, acute paronychia, and hyperpigmentation. The face was the most common (76.4% site of affection. Conclusion: A sizable number of patients of pediatric age group may be affected by “Holi”-related dermatoses necessitating precautionary measures.

  1. Dermatoses among Children from Celebration of “Holi,” the Spring Festival, in India: A Cross-sectional Observational Study

    Science.gov (United States)

    Ghosh, Sudip Kumar; Bandyopadhyay, Debabrata; Agarwal, Megha; Rudra, Olympia

    2016-01-01

    Background: “Holi” is a spring festival celebrated primarily in the Indian subcontinent and also abroad by expatriate Indians. It is a festival of colors, traditionally celebrated by mutual application of colors in different forms on a particular day of the year. These colors frequently comprise a range of synthetic dyes which have harmful effects on the skin and mucosae. Children take part in this colorful festival with much enthusiasm and vigor, making them prone to develop different “Holi”-related dermatoses. Our objective was to find out the different patterns of “Holi”-related dermatoses in a group of pediatric patients. Methodology: This was a cross-sectional descriptive study carried out over a period of 6 years (2010–2015). Consecutive patients of pediatric age group who attended dermatology outpatient department (OPD) with different dermatoses following application of “Holi” color were included in this study. Results: A total of 63 patients (mean age 11 years; range 1–16 years) were evaluated with a female to male ratio of 1.3:1. Itching is the predominant presenting symptom followed by burning sensation, dryness, scaling, oozing, and loss of hair. Examination revealed that eczematous lesion was the most common (69.8%) reaction pattern followed by xerosis, desquamation, excoriation, erythema, morbilliform eruption, erosion, alopecia, ulceration, acute paronychia, and hyperpigmentation. The face was the most common (76.4%) site of affection. Conclusion: A sizable number of patients of pediatric age group may be affected by “Holi”-related dermatoses necessitating precautionary measures. PMID:27688442

  2. Prevalence study of dermatoses referred to the phototherapy unit at the Dermatology Service of the Clinics Hospital of Porto Alegre, RS, Brazil*

    Science.gov (United States)

    Casara, Carolina; Eidt, Leticia; Cunha, Vivian

    2013-01-01

    BACKGROUND: Phototherapy consists of exposure to ultraviolet radiation for therapeutic reasons. Radiation is already used in dermatological practice, and many studies have already proved the beneficial effect of UV light treatment for chronic inflammatory or lymphoproliferative skin diseases. The Dermatology Service of the Clinics Hospital of Porto Alegre (Hospital de Clínicas de Porto Alegre) has been using phototherapy for a long time, and no official data have been described so far. OBJECTIVES: To study the prevalence of dermatoses referred to the phototherapy unit at the Clinics Hospital of Porto Alegre and describe the total number of patients who have already been referred to this sector and their phototype. METHODS: This is a descriptive cross-sectional study. Data were collected through a review of the phototherapy patients' records (secondary data), which are available on a database of the Dermatology Service of the Clinics Hospital of Porto Alegre, from August 1997 to July 2011. RESULTS: A total of 653 records were analyzed. Phototype 3 was the most prevalent (n=313). Distribution of the prevalence of dermatoses referred to the phototherapy unit was as follows: vitiligo (279), psoriasis (255), cutaneous T-cell lymphoma/mycosis fungoides (29), graftversus-host disease (15), scleroderma (11), atopic dermatitis (10), alopecia areata (6), parapsoriasis (5), eczema (4), granuloma annulare (4), and others (35). As vitiligo and psoriasis were the two most prevalent dermatoses, they were analyzed separately, with no statistical difference in prevalence between them (P=0,177). CONCLUSIONS: Our findings are in accordance with the literature, showing that although phototherapy is still mostly indicated to treat psoriasis, it has been used to treat other dermatoses, since the results are promising. PMID:23739698

  3. The multiple autoimmune syndromes. A clue for the autoimmune tautology.

    Science.gov (United States)

    Anaya, Juan-Manuel; Castiblanco, John; Rojas-Villarraga, Adriana; Pineda-Tamayo, Ricardo; Levy, Roger A; Gómez-Puerta, José; Dias, Carlos; Mantilla, Ruben D; Gallo, Juan Esteban; Cervera, Ricard; Shoenfeld, Yehuda; Arcos-Burgos, Mauricio

    2012-12-01

    The multiple autoimmune syndromes (MAS) consist on the presence of three or more well-defined autoimmune diseases (ADs) in a single patient. The aim of this study was to analyze the clinical and genetic characteristics of a large series of patients with MAS. A cluster analysis and familial aggregation analysis of ADs was performed in 84 patients. A genome-wide microsatellite screen was performed in MAS families, and associated loci were investigated through the pedigree disequilibrium test. Systemic lupus erythematosus (SLE), autoimmune thyroid disease (AITD), and Sjögren's syndrome together were the most frequent ADs encountered. Three main clusters were established. Aggregation for type 1 diabetes, AITD, SLE, and all ADs as a trait was found. Eight loci associated with MAS were observed harboring autoimmunity genes. The MAS represent the best example of polyautoimmunity as well as the effect of a single genotype on diverse phenotypes. Its study provides important clues to elucidate the common mechanisms of ADs (i.e., autoimmune tautology).

  4. Autoimmune/Inflammatory Syndrome Induced by Adjuvants and Thyroid Autoimmunity

    Science.gov (United States)

    Watad, Abdulla; David, Paula; Brown, Stav; Shoenfeld, Yehuda

    2017-01-01

    The autoimmune/inflammatory syndrome induced by adjuvants (ASIA), presented by Shoenfeld and Agmon-Levin in 2011, is an entity that incorporates diverse autoimmune conditions induced by the exposure to various adjuvants. Adjuvants are agents that entail the capability to induce immune reactions. Adjuvants are found in many vaccines and used mainly to increase the response to vaccination in the general population. Silicone has also been reported to be able to induce diverse immune reactions. Clinical cases and series of heterogeneous autoimmune conditions including systemic sclerosis, systemic lupus erythematosus, and rheumatoid arthritis have been reported to be induced by several adjuvants. However, only a small number of cases of autoimmune thyroid disorder have been included under the umbrella of ASIA syndrome. Indeed, clinical cases of Hashimoto’s thyroiditis and/or subacute thyroiditis were observed after the exposure to vaccines as well as silicone implantation. In our review, we aimed to summarize the current knowledge on ASIA syndrome presented as endocrinopathies, focusing on autoimmune thyroid disorders associated with the various adjuvants. PMID:28167927

  5. Bullous Pemphigoid Associated with the Dipeptidyl Peptidase-4 Inhibitor Sitagliptin in a Patient with Liver Cirrhosis Complicated with Rapidly Progressive Hepatocellular Carcinoma.

    Science.gov (United States)

    Harada, Masaru; Yoneda, Akitoshi; Haruyama, Sanehito; Yabuki, Kei; Honma, Yuichi; Hiura, Masaaki; Shibata, Michihiko; Matsuoka, Hidehiko; Uchiwa, Yasuhiro

    2017-09-15

    A 78-year-old man presented with cutaneous blisters of the limbs and abdominal distension. He had been treated for various diseases, including liver cirrhosis. He had begun receiving sitagliptin, a dipeptidyl peptidase-4 (DPP-4) inhibitor, for diabetes mellitus three years before the hospitalization. A skin biopsy demonstrated bullous pemphigoid. Ultrasonography (US) revealed multiple liver tumors, although he had been receiving regular US studies. We stopped sitagliptin and started insulin and corticosteroids. However, his renal dysfunction progressed, and he died 14 days after the hospitalization. We should therefore be careful of various complications, including bullous pemphigoid and progression of tumors, when using DPP-4 inhibitors.

  6. Diagnostic value of eccrine glands and hair follicles in direct immunofluorescent analysis of pemphigus vulgaris and bullous pemphigoid.

    Science.gov (United States)

    Zhou, Cheng; Yu, Yan; Elston, Dirk M

    2016-04-01

    The immunofluorescence pattern in adnexal structures may be of value, especially when the epidermis is not well represented in diagnostic sections. We studied a total of 88 cases of pemphigus vulgaris (PV) and bullous pemphigoid (BP) accessioned between 2010 and 2015 (40 cases of PV and 48 cases of BP). Immunofluorescence patterns and sensitivity in adnexal structures were similar to those observed in the epidermis. One case of PV and three cases of BP showed weak or absent fluorescence in the epidermis, while the eccrine glands were strongly positive, suggesting that careful examination of adnexal structures can be of value.

  7. Sustained clinical response to rituximab in a case of life-threatening overlap subepidermal autoimmune blistering disease.

    Science.gov (United States)

    Li, Yaohan; Foshee, J B; Sontheimer, Richard D

    2011-04-01

    The conventional treatment for the autoimmune bullous skin diseases is broad-spectrum immunosuppressive regimen typically combining systemic corticosteroids with adjuvant immunosuppressive therapeutic agents. Orphan diseases in the pemphigus, pemphigoid, and epidermolysis bullosa acquisita groups of clinical disorders are often clinically severe, requiring long-term treatment with such drugs or drug combinations. Rituximab, a chimeric recombinant monoclonal antibody targeting CD20(+) B cells, has recently been suggested to be effective in the treatment of pemphigus with relatively few adverse effects. The clinical value of rituximab in other immune-mediated blistering diseases has been less thoroughly examined. We report a case of a woman who presented initially with the Brunsting-Perry phenotype of cicatricial pemphigoid who subsequently developed severe generalized subepidermal blisters healing with scarring and milia formation thought to be clinically compatible with epidermolysis bullosa acquisita, although type VII collagen autoantibodies were never identified. Treatment with a number of conventional systemic agents was unsuccessful and complicated by methicillin-resistant Staphylococcus aureus-induced cutaneous ulcers and near-fatal gram-negative sepsis. This woman has enjoyed an 18-month complete clinical remission after a single inductive 4-week cycle of intravenous rituximab. This outcome supports the idea that systemic memory B-cell depletion with drugs such as rituximab should be considered for therapeutically refractory subepidermal autoimmune blistering diseases in addition to intraepidermal autoimmune blistering diseases. A potential role for the immunologic phenomenon of epitope spreading in the generation of overlapping features of autoimmune blistering diseases, and its contribution to therapeutic refractoriness ("hardening"), is discussed.

  8. Autoimmune hepatitis in association with lymphocytic colitis.

    LENUS (Irish Health Repository)

    Cronin, Edmond M

    2012-02-03

    Autoimmune hepatitis is a rare, chronic inflammatory disorder which has been associated with a number of other auto-immune conditions. However, there are no reports in the medical literature of an association with microscopic (lymphocytic) colitis. We report the case of a 53-year-old woman with several autoimmune conditions, including lymphocytic colitis, who presented with an acute hepatitis. On the basis of the clinical features, serology, and histopathology, we diagnosed autoimmune hepatitis. To our knowledge, this is the first report of autoimmune hepatitis in association with lymphocytic colitis, and lends support to the theory of an autoimmune etiology for lymphocytic colitis.

  9. An immunohistochemical study of the distribution of plasminogen and plasminogen activators in bullous pemphigoid.

    Science.gov (United States)

    Venning, V A; Wojnarowska, F; Cederholm-Williams, S

    1993-03-01

    Abnormalities of the cutaneous plasminogen/plasminogen activator system have been associated with acantholytic disorders, psoriasis, keratinocytes in culture, and epidermis in healing wounds. The present study was undertaken to investigate the possible role of the plasmin/plasminogen protease system in lesion development in bullous pemphigoid (BP). Using polyclonal antibodies and a fluorescent technique, the immunohistochemical distribution of plasmin/plasminogen, fibrinogen and the plasminogen activators, urokinase (uPA) and tissue plasminogen activator (tPA), were studied in lesional and non-lesional skin from nine BP patients, one with linear IgA disease (LAD) and one with pemphigoid gestationis (PG). The distribution of the proteases was compared with that in normal skin (n = 4) and in suction blisters (n = 2). In normal skin, fibrinogen, tPA and uPA were absent from the epidermis and plasminogen was confined to the basal layer. Uninvolved BP skin was identical to controls. Focal areas of suprabasal plasminogen expression in the region of a blister was seen in 3/9 BP lesions and in 1/2 suction blisters. In 6/9 BP lesions and both uninvolved and lesional LAD and PG skin were identical to controls, and no suprabasal expression of plasminogen was present. These findings suggest that suprabasal plasminogen expression is unlikely to play a fundamental role in the pathogenesis of blister formation in BP as enhanced expression was not present in every case and the finding was not specific to BP, also occurring in a suction blister. Enhanced plasminogen expression rather may be a reflection of the processes of tissue repair.

  10. Therapeutic apheresis in autoimmune diseases

    Directory of Open Access Journals (Sweden)

    Bambauer R

    2013-11-01

    Full Text Available Rolf Bambauer,1 Reinhard Latza,2 Carolin Bambauer,3 Daniel Burgard,4 Ralf Schiel5 1Institute for Blood Purification, Homburg, 2Laboratorium of Medicine, St Ingbert, 3Main Hospital Darmstadt, Darmstadt, 4Herz Zentrum, Cardiology, Völklingen, 5Inselklinik Heringsdorf GmbH, Seeheilbad Heringsdorf, Germany Abstract: Systemic autoimmune diseases based on an immune pathogenesis produce autoantibodies and circulating immune complexes, which cause inflammation in the tissues of various organs. In most cases, these diseases have a bad prognosis without treatment. Therapeutic apheresis in combination with immunosuppressive therapies has led to a steady increase in survival rates over the last 35 years. Here we provide an overview of the most important pathogenic aspects indicating that therapeutic apheresis can be a supportive therapy in some systemic autoimmune diseases, such as systemic lupus erythematosus, antiphospholipid syndrome, rheumatoid arthritis, and inflammatory eye disease. With the introduction of novel and effective biologic agents, therapeutic apheresis is indicated only in severe cases, such as in rapid progression despite immunosuppressive therapy and/or biologic agents, and in patients with renal involvement, acute generalized vasculitis, thrombocytopenia, leucopenia, pulmonary, cardiac, or cerebral involvement. In mild forms of autoimmune disease, treatment with immunosuppressive therapies and/or biologic agents seems to be sufficient. The prognosis of autoimmune diseases with varying organ manifestations has improved considerably in recent years, due in part to very aggressive therapy schemes. Keywords: therapeutic apheresis, autoimmune diseases, systemic lupus erythematosus, antiphospholipid syndrome, rheumatoid arthritis, inflammatory eye disease

  11. The immunogenetics of autoimmune diabetes and autoimmune thyroid disease.

    Science.gov (United States)

    Tomer, Y; Barbesino, G; Greenberg, D; Davies, T F

    1997-03-01

    Although medical genetics is a well-developed area of interest, relatively little is known about the diseases caused by the combination of many genes. These multiinfluenced diseases include the autoimmune endocrine diseases. Recent advances in the techniques for whole-genome screening have shown a variety of loci that are linked to the development of insulin-dependent diabetes mellitus, and similar data are likely to be soon generated in autoimmune thyroid disease. Here, the authors survey the current state of genetic knowledge in these two areas and describe the investigative and analytical techniques that are now available. (Trends Endocrinol Metab 1997;8:63-70). (c) 1997, Elsevier Science Inc.

  12. Localized bullous pemphigoid: report of a case with an immunofluorescence and electron microscopical studies on the lesional distribution of 180-KD bullous pemphigoid antigen, beta 4 integrin, and type VII collagen.

    Science.gov (United States)

    Kitajima, Y; Suzuki, M; Johkura, Y; Yaoita, H

    1993-07-01

    A 67-year-old woman with a left-sided hemiplegia had localized bullous pemphigoid demonstrating typical clinical lesions on the left pretibial skin and the radial-side skin of the right forearm. The histology showed a subepidermal blister with extensive hyperkeratosis, hypergranulosis, and acanthosis. Direct immunofluorescence revealed distinct linear deposits of IgG and C3 at the dermo-epidermal junction in the perilesional skin and in the roof of the blisters, but few deposits in nonlesional skin. Electron microscopy revealed separation in the lamina lucida. Indirect immunofluorescence of type VII collagen showed its localization in the blister floor. The distribution of the 180-KD bullous pemphigoid antigen (BPA) and beta 4 integrin, hemidesmosomal transmembrane proteins, were studied in the lesional skin by indirect immunofluorescence. Both 180-KD BPA and beta 4 integrin were localized in the blister roof. By immunoelectron microscopy, beta 4 integrin was detected in small groups on the cell surface facing the blister cavity. Since the epitope of the monoclonal antibody to 180-KD BPA used here is known to be localized at a distance of 20 to 50 nm from the membrane surface and this epitope retained in the blister roof, it appears that the blister was produced in the deep lamina lucida. The lesions were cleared with topical 0.05% clobetasole propionate ointment.

  13. Type 1 autoimmune pancreatitis

    Directory of Open Access Journals (Sweden)

    Zen Yoh

    2011-12-01

    Full Text Available Abstract Before the concept of autoimmune pancreatitis (AIP was established, this form of pancreatitis had been recognized as lymphoplasmacytic sclerosing pancreatitis or non-alcoholic duct destructive chronic pancreatitis based on unique histological features. With the discovery in 2001 that serum IgG4 concentrations are specifically elevated in AIP patients, this emerging entity has been more widely accepted. Classical cases of AIP are now called type 1 as another distinct subtype (type 2 AIP has been identified. Type 1 AIP, which accounts for 2% of chronic pancreatitis cases, predominantly affects adult males. Patients usually present with obstructive jaundice due to enlargement of the pancreatic head or thickening of the lower bile duct wall. Pancreatic cancer is the leading differential diagnosis for which serological, imaging, and histological examinations need to be considered. Serologically, an elevated level of IgG4 is the most sensitive and specific finding. Imaging features include irregular narrowing of the pancreatic duct, diffuse or focal enlargement of the pancreas, a peri-pancreatic capsule-like rim, and enhancement at the late phase of contrast-enhanced images. Biopsy or surgical specimens show diffuse lymphoplasmacytic infiltration containing many IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis. A dramatic response to steroid therapy is another characteristic, and serological or radiological effects are normally identified within the first 2 or 3 weeks. Type 1 AIP is estimated as a pancreatic manifestation of systemic IgG4-related disease based on the fact that synchronous or metachronous lesions can develop in multiple organs (e.g. bile duct, salivary/lacrimal glands, retroperitoneum, artery, lung, and kidney and those lesions are histologically identical irrespective of the organ of origin. Several potential autoantigens have been identified so far. A Th2-dominant immune reaction and the activation of

  14. Autoimmunity and type I diabetes.

    Science.gov (United States)

    Bach, J F

    1997-03-01

    Insulin-dependent diabetes mellitus (IDDM) is a T-cell-mediated autoimmune disease. The effector mechanisms essentially involve cytokine-mediated inflammation ultimately leading to beta-cell destruction. Several candidate autoantigens have been delineated for both the pathogenic T-cell response and the nonpathogenic antibody response used for disease prediction. Because of antigen spreading, it is not yet clear which of these antigens are involved in the triggering of the autoimmune response. In any case, this TH1 autoimmune response is amplified and perpetuated by an immune dysregulation involving TH2 cells. Both effector and regulatory mechanisms are placed under the tight control of major histocompatibility complex (MHC) and non-MHC genes. (Trends Endocrinol Metab 1997; 8:71-74). (c) 1997, Elsevier Science Inc.

  15. PD-1, gender, and autoimmunity

    Science.gov (United States)

    Dinesh, Ravi K.; Hahn, Bevra H.; Singh, Ram Pyare

    2010-01-01

    Programmed death 1 (PD-1) and its ligands (PD-L1 and PD-L2) are responsible for inhibitory T cell signaling that helps mediate the mechanisms of tolerance and immune homeostasis. The PD-1:PD-L signaling pathway has been shown to play an important role in a variety of diseases, including autoimmune conditions, chronic infection, and cancer. Recently, investigators have explored the role of sex hormones in modulating the pathway in autoimmune conditions. Exploring the effects of sex hormones on the PD-1:PD-L pathway could shed light on the gender biased nature of many autoimmune conditions as well as aide in the development of therapeutics targeting the immune system. PMID:20433954

  16. Improvement of inflammatory dermatoses severity and quality of life in patients treated with a betamethasone valerate plaster (LIBERE study)

    Science.gov (United States)

    Maccari, Francois

    2016-01-01

    Abstract Background: A ready to use betamethasone valerate 0.1% (BMV) dressing was effective and well-tolerated by patients receiving chronic plaque psoriasis treatment. Objective: Collect data related to BMV dressing used in the context of market authorization. Methods: An observational, prospective study, including 258 patients with a maximum 4-weeks-treatment of inflammatory dermatosis with BMV 2.25 mg plaster was performed. The prescription pattern was described and the disease severity assessed using a Physician Global Assessment (PGA). Patient satisfaction as well as their quality of life (DLQI) were evaluated. Clinical evaluation was performed before and after the treatment. Results: The DLQI scores improved from 10.0 ± 5.4 to 3.5 ± 3.5 points (p < 0.0001) and PGA decreased from 12.5 ± 3.1 to 4.2 ± 3.0 points (p < 0.0001). The highest DLQI and PGA improvement was reported for the eczema group. Subjects reported the test dressing was better than prior therapies with 93.5% very satisfied and 90.4% satisfied. Conclusions: The BMV dressing is well-tolerated and effective in the treatment of inflammatory dermatoses, improving both the objective signs of the disease as well as patients’ quality of life. PMID:25898980

  17. Phenotypic characterization of lymphocyte subsets in mycosis fungoides. Comparison with large plaque parapsoriasis and benign chronic dermatoses.

    Science.gov (United States)

    Ralfkiaer, E; Wantzin, G L; Mason, D Y; Hou-Jensen, K; Stein, H; Thomsen, K

    1985-11-01

    Altogether, 209 skin biopsies from 103 patients with mycosis fungoides (MF), large plaque parapsoriasis (LPP), and benign chronic dermatoses (BCD) have been examined immunohistologically with the use of a panel of 21 monoclonal antibodies against lymphoid cells and their subsets. All the infiltrates contained a mixture of T-lymphocytes, Langerhans cells, and other types of HLA-DR-positive dermal macrophages. The neoplastic T-cells in MF lesions expressed proliferation-(transferrin receptor) and activation-(the OKT10 antigen) associated markers more frequently than the T-cells in LPP and BCD. In other respects, the neoplastic T-cells in plaque lesions of MF resembled those seen in LPP and BCD; and most of these cases demonstrated a clear predominance of T-cells of helper/inducer type. The neoplastic T-cells in tumor lesions of MF were much more heterogeneous in phenotype. Only eight of these cases could be classified as T-helper neoplasms. In the remaining ten tumor cases, the neoplastic cells expressed either suppressor/cytotoxic or aberrant T-cell phenotypes. There were no phenotypic differences between the "classical" tumor stages and MF d'emblee cases. The data indicate that the early lesions of MF show an immunohistologic reaction pattern common to many immune responses of the skin and that the neoplastic cells in the advanced stages are more heterogeneous in phenotype than previously recognized.

  18. Multiple autoimmune syndrome with celiac disease.

    Science.gov (United States)

    Harpreet, Singh; Deepak, Jain; Kiran, B

    2016-01-01

    Multiple autoimmune syndrome (MAS) is a condition characterised by three or more autoimmune disorders in a same individual. Familial, immunologic and infectious factors are implicated in the development of MAS. Here we report a case of a 32-year-old woman with co-existence of four auto-immune diseases, namely autoimmune hypothyroidism, Sjögren's syndrome, systemic lupus erythematosus (SLE) and celiac disease which leads to the final diagnosis of multiple autoimmune syndrome type 3 with celiac disease. Patients with single autoimmune disorder are at 25% risk of developing other autoimmune disorders. The present case emphasises to clinicians that there is a need for continued surveillance for the development of new autoimmune disease in predisposed patients.

  19. An autosomal locus causing autoimmune disease: Autoimmune polyglandular disease type I assigned to chromosome 21

    NARCIS (Netherlands)

    J. Aaltonen (Johanna); P. Björses (Petra); L.A. Sandkuijl (Lodewijk); J. Perheentupa (Jaakko); L. Peltonen (Leena Johanna)

    1994-01-01

    textabstractAutoimmune polyglandular disease type I (APECED) is an autosomal recessive autoimmune disease characterized by a variable combination of the failure of the endocrine glands. The pathogenesis of this unique autoimmune disease is unknown; unlike many other autoimmune diseases, APECED does

  20. Bullous pemphigoid and linear IgA dermatosis sera recognize a similar 120-kDa keratinocyte collagenous glycoprotein with antigenic cross-reactivity to BP180

    NARCIS (Netherlands)

    Pas, HH; Kloosterhuis, GJ; Heeres, K; vanderMeer, JB; Jonkman, MF

    1997-01-01

    Circulating IgG from a large subset of bullous pein; phigoid (SP) patients reacted on immunoblot with a 120-kDa protein in conditioned keratinocyte culture medium and in keratinocyte cell extracts, A protein with a similar molecular weight was recognized by circulating IgA from a subset of patients

  1. Multiplex autoantibody detection for autoimmune liver diseases and autoimmune gastritis.

    Science.gov (United States)

    Vanderlocht, Joris; van der Cruys, Mart; Stals, Frans; Bakker-Jonges, Liesbeth; Damoiseaux, Jan

    2017-09-01

    Autoantibody detection for autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and autoimmune gastritis (AIG) is traditionally performed by IIF on a combination of tissues. Multiplex line/dot blots (LIA/DIA) offer multiple advantages, i.e. automation, objective reading, no interfering reactivities, no coincidental findings. In the current study we evaluated automated DIA (D-Tek) for detecting autoantibodies related to autoimmune diseases of the gastrointestinal tract. We tested samples of the Dutch EQC program and compared the results with the consensus of the participating labs. For the autoimmune liver diseases and AIG, respectively, 64 and 36 samples were tested. For anti-mitochondrial and anti-smooth muscle antibodies a concordance rate of 97% and 88% was observed, respectively. The concordance rate for anti-parietal cell antibodies was 92% when samples without EQC consensus (n=15) were excluded. For antibodies against intrinsic factor a concordance of 96% was observed. For all these antibodies discrepancies were identified that relate to the different test characteristics and the preponderance of IIF utilizing labs in the EQC program. In conclusion, we observed good agreement of the tested DIA blots with the consensus results of the Dutch EQC program. Taken together with the logistic advantages these blots are a good alternative for autoantibody detection in the respective diseases. A large prospective multicenter study is warranted to position these novel tests further in the whole spectrum of assays for the detection of these antibodies in a routine autoimmune laboratory. Copyright © 2017 Elsevier B.V. All rights reserved.

  2. TISSUE INHIBITOR OF METALLOPROTEINASE 1, MATRIX METALLOPROTEINASE 9, ALPHA-1 ANTITRYPSIN, METALLOTHIONEIN AND UROKINASE TYPE PLASMINOGEN ACTIVATOR RECEPTOR IN SKIN BIOPSIES FROM PATIENTS AFFECTED BY AUTOIMMUNE BLISTERING DISEASES

    Directory of Open Access Journals (Sweden)

    Ana Maria Abreu Velez

    2013-07-01

    Full Text Available Introduction: Proteinases and proteinase inhibitors have been described to play a role in autoimmune skin blistering diseases. We studied skin lesional biopsies from patients affected by several autoimmune skin blistering diseases for proteinases and proteinase inhibitors. Methods: We utilized immunohistochemistry to evaluate biopsies for alpha-1-antitrypsin, human matrix metalloproteinase 9 (MMP9, human tissue inhibitor of metalloproteinases 1 (TIMP-1, metallothionein and urokinase type plasminogen activator receptor (uPAR. We tested 30 patients affected by endemic pemphigus, 30 controls from the endemic area, and 15 normal controls. We also tested 30 biopsies from patients with bullous pemphigoid (BP, 20 with pemphigus vulgaris (PV, 8 with pemphigus foliaceus, and 14 with dermatitis herpetiformis (DH. Results: Contrary to findings in the current literature, most autoimmune skin blistering disease biopsies were negative for uPAR and MMP9. Only some chronic patients with El Bagre-EPF were positive to MMP9 in the dermis, in proximity to telocytes. TIMP-1 and metallothionein were positive in half of the biopsies from BP patients at the basement membrane of the skin, within several skin appendices, in areas of dermal blood vessel inflammation and within dermal mesenchymal-epithelial cell junctions.

  3. Autoimmune Skin Diseases in the Dog

    OpenAIRE

    Parker, W. M.

    1981-01-01

    Diagnoses of autoimmune skin diseases require very careful observation of the skin lesions, and selection of an intact vesicle for histopathological examination. If available, immunofluorescent studies can be very useful in confirming the diagnosis of autoimmune skin disease. Seven autoimmune skin diseases are briefly reviewed. Therapy must be aggressive and owner warned of the guarded prognosis.

  4. [Biermer's disease and autoimmune hemolytic anemia].

    Science.gov (United States)

    Nafil, Hatim; Tazi, Illias; Mahmal, Lahoucine

    2012-01-01

    Biermer's disease is an autoimmune atrophic gastritis of the fundus predominantly responsible for a malabsorption of vitamin B12. Despite its association with several autoimmune disorders, few observations have reported an association with autoimmune hemolytic anemia (AIHA). We report a case of Biermer's disease associated with AIHA in a patient of 66 years old.

  5. The thyroid, iodine and autoimmunity

    NARCIS (Netherlands)

    P. Mooij (Petra)

    1993-01-01

    textabstractAn excessive dietary iodine intake has also been described to lead to thyroid autoimmune reactivity: a. in individuals with a preexisting thyroid abnormality, such as an iodine deficient goitre, an excessive dietary iodine intake results in a proportion of the individuals in the developm

  6. Therapeutic apheresis in autoimmune diseases

    Science.gov (United States)

    Bambauer, Rolf; Latza, Reinhard; Bambauer, Carolin; Burgard, Daniel; Schiel, Ralf

    2013-01-01

    Systemic autoimmune diseases based on an immune pathogenesis produce autoantibodies and circulating immune complexes, which cause inflammation in the tissues of various organs. In most cases, these diseases have a bad prognosis without treatment. Therapeutic apheresis in combination with immunosuppressive therapies has led to a steady increase in survival rates over the last 35 years. Here we provide an overview of the most important pathogenic aspects indicating that therapeutic apheresis can be a supportive therapy in some systemic autoimmune diseases, such as systemic lupus erythematosus, antiphospholipid syndrome, rheumatoid arthritis, and inflammatory eye disease. With the introduction of novel and effective biologic agents, therapeutic apheresis is indicated only in severe cases, such as in rapid progression despite immunosuppressive therapy and/or biologic agents, and in patients with renal involvement, acute generalized vasculitis, thrombocytopenia, leucopenia, pulmonary, cardiac, or cerebral involvement. In mild forms of autoimmune disease, treatment with immunosuppressive therapies and/or biologic agents seems to be sufficient. The prognosis of autoimmune diseases with varying organ manifestations has improved considerably in recent years, due in part to very aggressive therapy schemes.

  7. Descemet stripping automated endothelial keratoplasty in Fuchs′ endothelial dystrophy versus pseudophakic bullous keratopathy

    Directory of Open Access Journals (Sweden)

    Mohammad Ali Javadi

    2016-01-01

    Full Text Available Purpose: To compare clinical and confocal scan outcomes after Descemet stripping automated endothelial keratoplasty (DSAEK performed for Fuchs′ endothelial dystrophy (FED versus pseudophakic bullous keratopathy (PBK. Methods: This retrospective comparative study included 47 consecutive eyes of 39 patients with the diagnosis of FED (n = 29, group 1 or PBK (n = 18, group 2 that underwent DSAEK. Clinical outcomes were compared between the study groups. At the final follow-up examination, confocal microscopy was used to measure and compare central corneal and graft thickness as well as endothelial cell density and morphology between the two groups. Results: Mean age at the time of surgery was 65.2 ± 11.8 and 69.4 ± 12.5 years in groups 1 and 2, respectively (P = 0.27. Follow-up period was 23.6 ± 14.0 months in group 1 and 25.6 ± 15.7 months in group 2 (P = 0.79. Postoperative best spectacle-corrected visual acuity was significantly better in group 1 than in group 2 until postoperative month 6. Afterwards, the two study groups were comparable in this regard. At the final follow-up examination, spherical equivalent refractive error was + 0.39 ± 1.46 diopters (D in group 1 and + 0.80 ± 1.47 D in group 2 (P = 0.45. Postoperative keratometric astigmatism was 1.02 ± 0.83 D and 2.36 ± 0.67 D, respectively (P < 0.001. Mean central graft thickness was 98.0 ± 33.3 μm in group 1 and 107.6 ± 28.0 μm in group 2 (P = 0.45. No statistically significant difference was observed between the two groups in terms of the postoperative endothelial cell density. Conclusion: The outcomes of DSAEK surgery were comparable between FED and PBK. All grafts were clear despite the lower than normal endothelial cell counts.

  8. Descemet Stripping Automated Endothelial Keratoplasty in Fuchs’ Endothelial Dystrophy versus Pseudophakic Bullous Keratopathy

    Science.gov (United States)

    Javadi, Mohammad Ali; Feizi, Sepehr; Jafari, Roya; Mirbabaee, Firooz; Ownagh, Vahid

    2016-01-01

    Purpose: To compare clinical and confocal scan outcomes after Descemet stripping automated endothelial keratoplasty (DSAEK) performed for Fuchs’ endothelial dystrophy (FED) versus pseudophakic bullous keratopathy (PBK). Methods: This retrospective comparative study included 47 consecutive eyes of 39 patients with the diagnosis of FED (n = 29, group 1) or PBK (n = 18, group 2) that underwent DSAEK. Clinical outcomes were compared between the study groups. At the final follow-up examination, confocal microscopy was used to measure and compare central corneal and graft thickness as well as endothelial cell density and morphology between the two groups. Results: Mean age at the time of surgery was 65.2 ± 11.8 and 69.4 ± 12.5 years in groups 1 and 2, respectively (P = 0.27). Follow-up period was 23.6 ± 14.0 months in group 1 and 25.6 ± 15.7 months in group 2 (P = 0.79). Postoperative best spectacle-corrected visual acuity was significantly better in group 1 than in group 2 until postoperative month 6. Afterwards, the two study groups were comparable in this regard. At the final follow-up examination, spherical equivalent refractive error was + 0.39 ± 1.46 diopters (D) in group 1 and + 0.80 ± 1.47 D in group 2 (P = 0.45). Postoperative keratometric astigmatism was 1.02 ± 0.83 D and 2.36 ± 0.67 D, respectively (P < 0.001). Mean central graft thickness was 98.0 ± 33.3 μm in group 1 and 107.6 ± 28.0 μm in group 2 (P = 0.45). No statistically significant difference was observed between the two groups in terms of the postoperative endothelial cell density. Conclusion: The outcomes of DSAEK surgery were comparable between FED and PBK. All grafts were clear despite the lower than normal endothelial cell counts. PMID:27994806

  9. Thyroid autoimmunity and polyglandular endocrine syndromes.

    Science.gov (United States)

    Wémeau, Jean-Louis; Proust-Lemoine, Emmanuelle; Ryndak, Amélie; Vanhove, Laura

    2013-01-01

    Even though autoimmune thyroiditis is considered as the most emblematic type of organ-specific autoimmune disorder of autoimmunity, autoimmune thyroid diseases can be associated with other autoimmune endocrine failures or non-endocrine diseases (namely vitiligo, pernicious anemia, myasthenia gravis, autoimmune gastritis, celiac disease, hepatitis). Thyroid disorders, which are the most frequent expression of adult polyendocrine syndrome type 2, occur concomitantly with or secondarily to insulinodependent diabetes, premature ovarian failure, Addison's disease (Schmidt syndrome, or Carpenter syndrome if associated with diabetes). Testicular failure and hypoparathyroidism are unusual. The disease is polygenic and multifactorial. Disorders of thyroid autoimmunity are, surprisingly, very rare in polyendocrine syndrome type 1 (or APECED) beginning during childhood. They are related to mutations of the AIRE gene that encodes for a transcriptional factor implicated in central and peripheral immune tolerance. Hypothyroidism can also be observed in the very rare IPEX and POEMS syndromes.

  10. AIRE-mutations and autoimmune disease.

    Science.gov (United States)

    Bruserud, Øyvind; Oftedal, Bergithe E; Wolff, Anette B; Husebye, Eystein S

    2016-12-01

    The gene causing the severe organ-specific autoimmune disease autoimmune polyendocrine syndrome type-1 (APS-1) was identified in 1997 and named autoimmune regulator (AIRE). AIRE plays a key role in shaping central immunological tolerance by facilitating negative selection of T cells in the thymus, building the thymic microarchitecture, and inducing a specific subset of regulatory T cells. So far, about 100 mutations have been identified. Recent advances suggest that certain mutations located in the SAND and PHD1 domains exert a dominant negative effect on wild type AIRE resulting in milder seemingly common forms of autoimmune diseases, including pernicious anemia, vitiligo and autoimmune thyroid disease. These findings indicate that AIRE also contribute to autoimmunity in more common organ-specific autoimmune disorders. Copyright © 2016 Elsevier Ltd. All rights reserved.

  11. Transplante de membrana amniótica na ceratopatia bolhosa Amniotic membrane transplantation for bullous keratopathy

    Directory of Open Access Journals (Sweden)

    Alessandra Guerra Daros Castellano

    2004-12-01

    pain relief and improvement of the recurrent epithelial side effects in patients with symptomatic bullous keratopathy and poor vision. METHODS: A prospective survey was conducted with 9 patients, from April/2000 to December/2001 at the "Hospital Universitário Evangélico de Curitiba - HUEC". Before the surgery, the medical records of each patient were studied and a complete ocular examination was performed. The patients were evaluated once a week or more frequently, including the first postoperative (PO, seventh PO, fourteenth PO and thirtieth PO day. A monthly evaluation was performed at the sixth month after the operation. RESULTS: The group consisted of 3 (33.3% males and 6 (66.6% females, with age from 29 to 74 years. All patients complained of pain in their eyes, 7 (77.7% had teary eyes, 8 (88.8% patients had photophobia and 4 (44.4% had red eyes. The visual acuity in the preoperative was finger counting in 6 (66.6% of the patients, hand moving in 2 (22.2% of the patients and amaurosis in 1 (11.1% patient. After the procedure, there was a reepithelialization in all patients between the twelfth and the twenty-first postoperative day. The patients seemed to recover from pain and photophobia in the first week after the amniotic membrane transplantation and remained well until the end of follow-up. CONCLUSION: The amniotic membrane has the potential to restore corneal surface in patients with symptomatic bullous keratopathy, reducing very quickly pain in these patients. However, the number of studied patients is small, but this treatment is an alternative that has encouraged us, as well as other researchers, due to the excellent obtained result.

  12. IMMUNOHISTOCHEMISTRY VERSUS IMMUNOFLUORESENCE IN THE DIAGNOSIS OF AUTOIMMUNE BLISTERING DISEASES

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    Ana Maria Abreu Velez

    2013-11-01

    Full Text Available Introduction: In patients with autoimmune skin blistering diseases (ABDs, the diagnostic gold standard has classically been direct and indirect immunofluorescence (DIF and IIF, despite inherent technical problems of autofluorescence. Aim: We sought to overcome autofluorescence issues and compare the reliability of immunofluorescence versus immunohistochemistry (IHC staining in the diagnoses of these diseases. Methods: We tested via IHC for anti-human IgG, IgM, IgA, IgD, IgE, Kappa light chains, Lambda light chains, Complement/C3c, Complement/C1q, Complement/C3d, albumin and fibrinogen in 30 patients affected by a new variant of endemic pemphigus foliaceus in El Bagre, Colombia (El Bagre-EPF, and 30 control biopsies from the endemic area. We also tested archival biopsies from patients with ABDs whose diagnoses were made clinically, histopathologically and by DIF/IIF studies from 2 independent dermatopathology laboratories in the USA. Specifically, we tested 34 patients with bullous pemphigoid (BP, 18 with pemphigus vulgaris (PV, 8 with pemphigus foliaceus (PF, 14 with dermatitis herpetiformis (DH and 30 control skin samples from plastic esthetic surgery reduction surgeries. Results: The diagnostic correlation between IHC and DIF-IIF was almost 98% in most cases. IHC revealed evidence of autofluorescence around dermal blood vessels, dermal eccrine glands and neurovascular packages feeding skin appendices in ABDs; this autofluorescence may represent a non-specific immune response. Strong patterns of positivity were seen also in endothelial-mesenchymal cell junction-like structures, as well as between dermal fibrohistiocytic cells. In PV, we noted strong reactivity to neurovascular packages supplying sebaceous glands, as well as apocrine glands with edematous changes. Conclusions: We suggest that IHC is as reliable as DIF or IIF for the diagnosis of ABDs; our findings further suggest that what has previously been considered DIF/IIF autofluorescence

  13. The profile of the dermatoses in children with the HIV virus at the Fundação de Medicina Tropical do Amazonas.

    Science.gov (United States)

    Dias, Eleonora Dantas; Cunha, Maria da Graça Souza; Talhari, Sinésio

    2012-01-01

    The Acquired Immunodeficiency Syndrome (AIDS) constitutes a sub-epidemic in Brazil. Due to the increasing number of women infected by the virus, the vertical transmission increased substantially, and due to the lack of adequate prophylactic treatment, many children are infected and show manifestations of the disease in early ages. Multiple systems are affected by the HIV virus, and the skin is often the first organ to be involved. The objective of this study is to analyze the clinic, dermatological and epidemiological profiles of children carriers of the virus in the City of Manaus aiming at identifying the most frequent dermatoses that affect these children and try to relate these dermatoses to the immunologic deterioration. A study was conducted where children carriers of the HIV virus from the Fundação Alfredo da Matta and Fundação de Medicina Tropical were studied from March 2007 to July 2008. These children were submitted to dermatological and laboratorial exams such as viral load dosage and CD4+ and CD8+ counts. During the study period, 70 HIV + children were examined; all of them had AIDS and had been contaminated by vertical transmission. The average number of dermatoses by children was 1.73, and 95.5% had at least one dermatosis during the study period. The most frequent manifestations were atopic dermatitis (22.9%), childhood prurigo (20%) and warts (18,6%). Children with HIV/AIDS have more skin disorders than children without HIV/AIDS. There was no statistical difference between the children in the group using ARVT and the group that wasn't using it.

  14. 大连地区化妆品皮肤病890例分析%Clinical analysis of 890 cases of cosmetic dermatoses in Dalian region

    Institute of Scientific and Technical Information of China (English)

    杨娜; 张荣鑫; 赵淑娟; 金玲; 黄畋; 涂彩霞

    2011-01-01

    目的 探讨化妆品皮肤病的临床特点及其相关化妆品类型.方法 对890例化妆品皮肤病进行分析,对可疑化妆品行斑贴试验.结果 临床表现主要为接触性皮炎占90%,依次为色素沉着占3.93%、痤疮样损害占3.37%、光敏性皮炎占2.24%、接触性荨麻疹占0.56%.可疑致病化妆品种类2019种,以护肤品为主,占92%.890例患者中斑贴试验阳性者346例,阳性率39%.结论 化妆品皮肤病的发生原因是多方面的,应加强化妆品知识宣传,建立一个完整的化妆品皮肤病监测系统.%Objective To investigate the clinical features of cosmetic dermatoses and causative cosmetics. Methods A total of 890 cases of cosmetic dermatosis were analyzed in this study. Patch test was performed to determine the causative cosmetics. Results The cosmetic dermatoses mainly manifested as contact dermatitis (90%), followed by hyperpigmentation (3.93%), acneiform lesions (3.37%), photosensitive dermatitis (2.24%), contact urticaria (0.56%). There were 2019 types of questionable cosmetics, which were predominated by skin care cosmetics (92%). Of the 890 patients, 346 (39%) were positive for patch test.Conclusions The causes of cosmetic dermatosis are various, and it is important to strengthen the promotion of cosmetic knowledge as well as to set up a complete monitoring system for cosmetic dermatoses.

  15. Autoimmune Polyglandular Syndrome Type 1

    OpenAIRE

    Ponranjini, Vedeswari C.; Jayachandran, S; L Kayal; K Bakyalakshmi

    2012-01-01

    Autoimmune Polyglandular Syndrome (APS) Type 1 is a rare hereditary disorder that damages organs in the body. This disease entity is the result of a mutation in the AIRE gene. It is characterized by three classic clinical features - hypoparathyroidism, Addison′s disease, and chronic mucocutaneous candidiasis. For a patient to be diagnosed as having APS Type 1 syndrome at least two of these features needs to be present. The third entity may develop as the disease progresses. We report a case o...

  16. Historical reflections on autoimmune hepatitis

    Institute of Scientific and Technical Information of China (English)

    Ian R Mackay

    2008-01-01

    Autoimmune hepatitis (AIH),initially known as chronic active or active chronic hepatitis (and by various other names),first came under clinical notice in the late 1940s.However,quite likely,chronic active hepatitis (CAH) had been observed prior to this and was attributed to a persistently destructive virus infection of the liver.An earlier (and controversial) designation in 1956 as lupoid hepatitis was derived from associated L.E.cell test positivity and emphasized accompanying multisystem features and immunological aberrations.Young women featured prominently in early descriptions of CAH.AIH was first applied in 1965 as a descriptive term.Disease-characteristic autoantibodies were defined from the early 1960s,notably antinuclear antibody (ANA),smooth muscle antibody (SMA) and liver-kidney microsomal (LKM) antibody.These are still widely used diagnostically but their relationship to pathogenesis is still not evident.A liver and disease specific autoantigen has long been searched for but unsuccessfully.Prolonged immunosuppressive therapy with predisolone and azathioprine in the 1960s proved beneficial and remains standard therapy today.AIH like many other autoimmune diseases is associated with particular HLA alleles especially with the "ancestral" B8,DR3 haplotype,and also with DR4.Looking forwards,AIH is one of the several enigmatic autoimmune diseases that,despite being (relatively) organ specific,are marked by autoimmune reactivities with non-organ-specific autoantigens.New paradigms are needed to explain the occurrence,expressions and pathogenesis of such diseases.

  17. Autoimmunity: Experimental and clinical aspects

    Energy Technology Data Exchange (ETDEWEB)

    Schwartz, R.S.; Rose, N.R.

    1986-01-01

    This book contains five parts and a section of poster papers. Each part contains several papers. Some of the papers are: Molecular Genetics and T Cells in Autoimmunity; Gene Conversion: A Mechanism to Explain HLA-D Region and Disease Association; Genetics of the Complement System; Speculation on the Role of Somatic Mutation in the Generation of Anti-DNA Antibodies; and Monoclonal Anti-DNA Antibodies: The Targets and Origins of SLE.

  18. Autoimmune Thyroiditis and Myasthenia Gravis

    Directory of Open Access Journals (Sweden)

    Angela Lopomo

    2017-07-01

    Full Text Available Autoimmune diseases (AIDs are the result of specific immune responses directed against structures of the self. In normal conditions, the molecules recognized as “self” are tolerated by immune system, but when the self-tolerance is lost, the immune system could react against molecules from the body, causing the loss of self-tolerance, and subsequently the onset of AID that differs for organ target and etiology. Autoimmune thyroid disease (ATD is caused by the development of autoimmunity against thyroid antigens and comprises Hashimoto’s thyroiditis and Graves disease. They are frequently associated with other organ or non-organ specific AIDs, such as myasthenia gravis (MG. In fact, ATD seems to be the most associated pathology to MG. The etiology of both diseases is multifactorial and it is due to genetic and environmental factors, and each of them has specific characteristics. The two pathologies show many commonalities, such as the organ-specificity with a clear pathogenic effect of antibodies, the pathological mechanisms, such as deregulation of the immune system and the implication of the genetic predisposition. They also show some differences, such as the mode of action of the antibodies and therapies. In this review that focuses on ATD and MG, the common features and the differences between the two diseases are discussed.

  19. Human Cytomegalovirus and Autoimmune Disease

    Directory of Open Access Journals (Sweden)

    Anne Halenius

    2014-01-01

    Full Text Available Human cytomegalovirus (HCMV represents a prototypic pathogenic member of the β-subgroup of the herpesvirus family. A range of HCMV features like its lytic replication in multiple tissues, the lifelong persistence through periods of latency and intermitting reactivation, the extraordinary large proteome, and extensive manipulation of adaptive and innate immunity make HCMV a high profile candidate for involvement in autoimmune disorders. We surveyed the available literature for reports on HCMV association with onset or exacerbation of autoimmune disease. A causative linkage between HCMV and systemic lupus erythematosus (SLE, systemic sclerosis (SSc, diabetes mellitus type 1, and rheumatoid arthritis (RA is suggested by the literature. However, a clear association of HCMV seroprevalence and disease could not be established, leaving the question open whether HCMV could play a coresponsible role for onset of disease. For convincing conclusions population-based prospective studies must be performed in the future. Specific immunopathogenic mechanisms by which HCMV could contribute to the course of autoimmune disease have been suggested, for example, molecular mimicry by UL94 in SSc and UL83/pp65 in SLE patients, as well as aggravation of joint inflammation by induction and expansion of CD4+/CD28− T-cells in RA patients. Further studies are needed to validate these findings and to lay the grounds for targeted therapeutic intervention.

  20. [Autoimmune hepatitis induced by isotretionine].

    Science.gov (United States)

    Guzman Rojas, Patricia; Gallegos Lopez, Roxana; Ciliotta Chehade, Alessandra; Scavino, Yolanda; Morales, Alejandro; Tagle, Martín

    2016-01-01

    We describe a case of a teenage patient with the diagnosis of drug induced autoimmune hepatitis. The patient is a 16 years old female, with the past medical history of Hashimoto’s hypothyroidism controlled with levothyroxine, who started treatment with Isotretionin (®Accutane) 20 mg q/12 hours for a total of 3 months for the treatment of severe acne. The physical examination was within normal limits and the results of the laboratory exams are: Baseline values of ALT 28 U/L, AST 28 U/L. Three months later: AST 756 U/L, ALT 1199U/L, alkaline phosphatase 114 U/L, with normal bilirrubin levels throughout the process. The serology studies were negative for all viral hepatitis; ANA titers were positive (1/160) and igG levels were also elevated. A liver biopsy was performed, and was compatible with the diagnosis of autoimmune hepatitis. Corticosteroid therapy was started with Prednisone 40 mg per day one week after stopping the treatment with isotretionin, observing an improvement in the laboratory values. We describe this case and review the world literature since there are no reported cases of Isotretinoin-induced autoimmune hepatitis.

  1. Autoimmune diseases and HIV infection

    Science.gov (United States)

    Virot, Emilie; Duclos, Antoine; Adelaide, Leopold; Miailhes, Patrick; Hot, Arnaud; Ferry, Tristan; Seve, Pascal

    2017-01-01

    Abstract To describe the clinical manifestations, treatments, prognosis, and prevalence of autoimmune diseases (ADs) in human immunodeficiency virus (HIV)-infected patients. All HIV-infected patients managed in the Infectious Diseases Department of the Lyon University Hospitals, France, between January 2003 and December 2013 and presenting an AD were retrospectively included. Thirty-six ADs were found among 5186 HIV-infected patients which represents a prevalence of 0.69% including immune thrombocytopenic purpura (n = 15), inflammatory myositis (IM) (n = 4), sarcoidosis (n = 4), Guillain–Barré syndrome (GBS) (n = 4), myasthenia gravis (n = 2), Graves’ disease (n = 2), and 1 case of each following conditions: systemic lupus erythematosus, rheumatoid arthritis, autoimmune hepatitis, Hashimoto thyroiditis and autoimmune hemolytic anemia. One patient presented 2 ADs. Thirty patients were known to be HIV-infected when they developed an AD. The AD preceded HIV infection in 2 patients. GBS and HIV infection were diagnosed simultaneously in 3 cases. At AD diagnosis, CD4 T lymphocytes count were higher than 350/mm3 in 63% of patients, between 200 and 350/mm3 in 19% and less than 200/mm3 in 19%. Twenty patients benefited from immunosuppressant treatments, with a good tolerance. ADs during HIV infection are uncommon in this large French cohort. Immune thrombocytopenic purpura, sarcoidosis, IM, and GBS appear to be more frequent than in the general population. Immunosuppressant treatments seem to be effective and well tolerated. PMID:28121924

  2. Autoimmunity in Chagas' heart disease

    Directory of Open Access Journals (Sweden)

    Edécio Cunha-Neto

    Full Text Available The time scale dissociation between high parasitemia and tissue pathology, allied to the absence of parasites in the heart lesions of chronic Chagas' disease cardiopathy, casted doubt on the direct participation of Trypanosoma cruzi in tissue lesions. Moreover, the heart tissue lesions in chronic Chagas' disease cardiopathy are associated to an inflammatory mononuclear cell infiltrate, presumably the ultimate effectors of tissue damage. It has been hypothesized that the inflammatory cell infiltrate could mediate a delayed hypersensitivity process directed to the heart tissue components, an autoimmune response triggered by immunological cross-reactivity in the course of a protective immune response against some T.cruzi antigen homologous to heart proteins. However, little is known about the efector role of the T cells in the infiltrate, or about the nature of the antigen that lead to their accumulation in tissue. In this paper, we will review the published evidence on autoimmunity and immunological cross-reactivity between T. cruzi and the mammalian host, along with data generated in our laboratory. The definition of the precise role played by autoimmunity in the pathogenesis of Chagas' disease cardiopathy may have important consequences both for immunoprophylaxis and for the therapeutic approach of chronic Chagas' disease.

  3. [Pulmonary arterial hypertension: a flavor of autoimmunity].

    Science.gov (United States)

    Perros, Frédéric; Humbert, Marc; Cohen-Kaminsky, Sylvia

    2013-01-01

    It is admitted that autoimmunity results from a combination of risks such as genetic background, environmental triggers, and stochastic events. Pulmonary arterial hypertension (PAH) shares with the so-called prototypic autoimmune diseases, genetic risk factors, female predominance and sex hormone influence, association with other chronic inflammatory and autoimmune diseases, defects in regulatory T cells function, and presence of autoantibodies. Case reports have been published indicating the beneficial effect of some immunosuppressive and anti-inflammatory therapies in PAH, supporting the potential role of immune mechanisms in the pathophysiology of the disease. In this review, we discuss the current knowledge on autoimmune mechanisms operating in PAH, especially mounting a local autoimmune response inside the pulmonary tissue, namely pulmonary lymphoid neogenesis. A better understanding of the role of autoimmunity in pulmonary vascular remodelling may help develop targeted immunomodulatory strategies in PAH.

  4. Angiogenesis-associated dermatoses and their treatment with traditional Chinese medicine%血管生成相关性皮肤病及其中药治疗

    Institute of Scientific and Technical Information of China (English)

    杨萍; 郑敏

    2008-01-01

    血管生成与一些皮肤病,如皮肤光老化、银屑病、寻常痤疮、酒渣鼻等密切相关,其发病机制主要为血管生成刺激因子和抑制因子的失衡,导致血管异常增生.很多中药成分能通过影响血管调控因素调节血管生成,从而有效地治疗一些与血管生成相关的皮肤病.探究中药影响血管生成的作用机制,将为血管增生性皮肤病提供新的治疗靶点.%Angiogenesis is closely associated with numerous skin disorders, such as photoaging, psoriasis, acne vulgaris, acne rosacea, and so on. It's mainly induced by the disequilibrium of angiogenesis-stimulating and-inhibiting factors, which can be affected by a great many of traditional Chinese medicines (TCMs). So, TCMs may be effective in the treatment of angiogenesis-associated dermatoses. To explore the mechanism underlying the influence of TCMs on angiogenesis will provide new therapeutic targets for angiogenesis-associated dermatoses.

  5. Epidemiology of autoimmune diseases in Denmark

    DEFF Research Database (Denmark)

    Eaton, William W.; Rose, N.R.; Kalaydijan, A.;

    2007-01-01

    An epidemiologic study of the autoimmune diseases taken together has not been done heretofore. The National Patient Register of Denmark is used to estimate the population prevalence of 31 possible or probable autoimmune diseases. Record linkage is used to estimate 465 pairwise co...... diseases and weak across diseases. These data confirm the importance of the autoimmune diseases as a group and suggest that common etiopathologies exist among them...

  6. Role of Complement in Autoimmune Hemolytic Anemia

    OpenAIRE

    Berentsen, Sigbjørn

    2015-01-01

    Summary The classification of autoimmune hemolytic anemias and the complement system are reviewed. In autoimmune hemolytic anemia of the warm antibody type, complement-mediated cell lysis is clinically relevant in a proportion of the patients but is hardly essential for hemolysis in most patients. Cold antibody-mediated autoimmune hemolytic anemias (primary cold agglutinin disease, secondary cold agglutinin syndrome and paroxysmal cold hemoglobinuria) are entirely complement-mediated disorder...

  7. Amplification of autoimmune disease by infection

    OpenAIRE

    Posnett, David N; Yarilin, Dmitry

    2005-01-01

    Reports of infection with certain chronic persistent microbes (herpesviruses or Chlamydiae) in human autoimmune diseases are consistent with the hypothesis that these microbes are reactivated in the setting of immunodeficiency and often target the site of autoimmune inflammation. New experimental animal models demonstrate the principle. A herpesvirus or Chlamydia species can be used to infect mice with induced transient autoimmune diseases. This results in increased disease severity and even ...

  8. Autoimmune diseases associated with neurofibromatosis type 1.

    Science.gov (United States)

    Nanda, Arti

    2008-01-01

    Associations of autoimmune diseases with neurofibromatosis type 1 have been rarely described. In the present report, we describe two patients of neurofibromatosis type 1 having an association with vitiligo in one, and alopecia areata and autoimmune thyroiditis in another. The associations of neurofibromatosis type 1 with vitiligo, alopecia areata, and autoimmune thyroiditis have not been reported earlier. Whether these associations reflect a causal relationship with neurofibromatosis type 1 or are coincidental needs to be settled.

  9. Severe autoimmune hemolytic anemia with renal neoplasm.

    Science.gov (United States)

    Rhodes, Emily C; Parikh, Sahil P; Bhattacharyya, Nishith

    2014-02-01

    Autoimmune hemolytic anemia is a type of hemolytic anemia characterized by autoantibodies directed against red blood cells shortening their survival. When autoimmune hemolytic anemia is secondary to a paraneoplastic process, severe anemia can occur leading to significant morbidity and even mortality. Here we discuss the literature and present the case of a child with autoimmune hemolytic anemia from a paraneoplastic syndrome secondary to a renal tumor.

  10. Immunogenetics and the cause of autoimmune disease.

    Science.gov (United States)

    Baxter, A G

    1997-01-01

    Autoimmune disease results from the action of environmental factors on a predisposed genotype. In this review, the role of genetic susceptibility in the aetiology of autoimmune disease is examined. As the genetics of autoimmune diabetes has been studied more intensively than that of other autoimmune diseases, supporting evidence is drawn principally from that example. Autoimmune diseases are not inherited as entities but as constitutions which confer an increased probability of developing disease. It is proposed that there are two components to autoimmune disease susceptibility. One confers susceptibility to autoimmunity per se, while the other determines tissue specificity. In this review, the concept of liability is introduced as a tool used in quantitative genetics and is applied to the analysis of autoimmune diabetes by considering a threshold model. In this example, empirically derived incidence figures are used to calculate heritability which is a relative measure of the influence of genetics and environmental factors. The validity of applying the concept of liability to diabetes is confirmed by examining the values of heritability calculated from empirical data obtained from different kindred relationships, and by confirming that the assumptions on which liability is based are supported by recent gene mapping data. Finally, the physiological significance of liability is considered and its significance to the cause of autoimmunity discussed.

  11. Microbiota at the crossroads of autoimmunity.

    Science.gov (United States)

    Shamriz, Oded; Mizrahi, Hila; Werbner, Michal; Shoenfeld, Yehuda; Avni, Orly; Koren, Omry

    2016-09-01

    Autoimmune diseases have a multifactorial etiology including genetic and environmental factors. Recently, there has been increased appreciation of the critical involvement of the microbiota in the pathogenesis of autoimmunity, although in many cases, the cause and the consequence are not easy to distinguish. Here, we suggest that many of the known cues affecting the function of the immune system, such as genetics, gender, pregnancy and diet, which are consequently involved in autoimmunity, exert their effects by influencing, at least in part, the microbiota composition and activity. This, in turn, modulates the immune response in a way that increases the risk for autoimmunity in predisposed individuals. We further discuss current microbiota-based therapies.

  12. Modulation of autoimmunity with artificial peptides

    Science.gov (United States)

    La Cava, Antonio

    2010-01-01

    The loss of immune tolerance to self antigens leads to the development of autoimmune responses. Since self antigens are often multiple and/or their sequences may not be known, one approach to restore immune tolerance uses synthetic artificial peptides that interfere or compete with self peptides in the networks of cellular interactions that drive the autoimmune process. This review describes the rationale behind the use of artificial peptides in autoimmunity and their mechanisms of action. Examples of use of artificial peptides in preclinical studies and in the management of human autoimmune diseases are provided. PMID:20807590

  13. Lúpus eritematoso sistêmico bolhoso na infância: relato de caso Bullous systemic lupus erythematosus in the childhood: case report

    Directory of Open Access Journals (Sweden)

    Mariana Discacciati Chiminazzo

    2007-06-01

    Full Text Available É apresentado caso de lúpus eritematoso bolhoso na infância. Doente do sexo feminino, com nove anos, apresentava erupção vesicobolhosa no tronco, região cervical, genital e membros. O exame anatomopatológico mostrou bolha subepidérmica com neutrófilos, a imunofluorescência direta revelou depósito linear de IgA, IgM, IgG e C3 na zona da membrana basal, e a indireta foi negativa. Os anticorpos antinucleares e o anti-Sm estavam positivos. Houve regressão do quadro com dapsona e prednisona. Trata-se de caso raro de lúpus eritematoso sistêmico (LES na infância que se iniciou com bolhas disseminadas. O LES bolhoso deve ser incluído no diagnóstico diferencial das erupções bolhosas na infância.We report a case of bullous systemic lupus erythematosus in a 9-year-old female, with a vesiculobullous eruption on the trunk, neck, genitals and limbs. A skin biopsy specimen showed subepidermal blister with neutrophils. Direct immunofluorescence revealed deposition of IgA, IgG, IgM and C3 at the epithelial basement membrane zone and indirect immunofluorescence was negative. Antinuclear antibodies and anti-Sm were positive. Resolution of the blisters occurred following treatment with dapsone and prednisone. This is a rare case of systemic lupus erythematous (SLE in the childhood, whose initial manifestation was a bullous eruption. Bullous SLE should be considered in the differential diagnosis of children presenting with generalized bullous eruption.

  14. Autoimmune pancreatitis--recent advances.

    Science.gov (United States)

    Novotný, I; Díte, P; Lata, J; Nechutová, H; Kianicka, B

    2010-01-01

    Autoimmune pancreatitis (AIP) is recognized as a distinct clinical entity, identified as a chronic inflammatory process of the pancreas in which the autoimmune mechanism is involved. Clinically and histologically, AIP has two subsets: type 1--lymphoplasmatic sclerosing pancreatitis with abundant infiltration of the pancreas and other affected organs with immunoglobulin G4-positive plasma cells, and type 2--duct centric fibrosis, characterized by granulocyte epithelial lesions in the pancreas without systemic involvement. In the diagnosis of AIP, two diagnostic criterions are used--the HISORt criteria and Asian Diagnostic Criteria. In the differential diagnosis, the pancreatic cancer must be excluded by endosonographically guided pancreatic biopsy. Typical signs of AIP are concomitant disorders in other organs (kidney, liver, biliary tract, salivary glands, colon, retroperitoneum, prostate). Novel clinicopathological entity was proposed as an 'IgG4-related sclerosing disease' (IgG4-RSC). Extensive IgG4-positive plasma cells and T lymphocyte infiltration is a common characteristics of this disease. Recently, IgG4-RSC syndrome was extended to a new entity, characterized by IgG4 hypergammaglobulinemia and IgG4-positive plasma cell infiltration, this being considered an expression of a lymphoproliferative disease, 'IgG4-positive multiorgan lymphoproliferative syndrome'. This syndrome includes Mikulicz's disease, mediastinal fibrosis, autoimmune hypophysitis, and inflammatory pseudotumor--lung, liver, breast. In the therapy of AIP, steroids constitute first-choice treatment. High response to the corticosteroid therapy is an important diagnostic criterion. In the literature, there are no case-control studies that determine if AIP predisposes to pancreatic cancer. Undoubtedly, AIP is currently a hot topic in pancreatology.

  15. [Narcolepsy as an autoimmune disease].

    Science.gov (United States)

    Sarkanen, Tomi; Vaarala, Outi; Julkunen, Ilkka; Partinen, Markku

    2015-01-01

    Narcolepsy is a sleep disorder of central origin. Hypocretin deficiency is the essential feature of type 1 narcolepsy. The biological background of type 2 narcolepsy (without cataplexy) is less clear. Infections or other external factors are thought to function as triggers of narcolepsy. After the H1N1 vaccination campaign, the incidence of narcolepsy increased clearly in countries where a vaccine boosted with the AS03 adjuvant was used. According to the current view, the increase of narcolepsy in connection with the pandemic vaccine especially in children and adolescents was associated with the virus component of the vaccine, but the adjuvant may also have boosted the development of autoimmune response.

  16. Helminth Immunomodulation in Autoimmune Disease.

    Science.gov (United States)

    Smallwood, Taylor B; Giacomin, Paul R; Loukas, Alex; Mulvenna, Jason P; Clark, Richard J; Miles, John J

    2017-01-01

    Helminths have evolved to become experts at subverting immune surveillance. Through potent and persistent immune tempering, helminths can remain undetected in human tissues for decades. Redirecting the immunomodulating "talents" of helminths to treat inflammatory human diseases is receiving intensive interest. Here, we review therapies using live parasitic worms, worm secretions, and worm-derived synthetic molecules to treat autoimmune disease. We review helminth therapy in both mouse models and clinical trials and discuss what is known on mechanisms of action. We also highlight current progress in characterizing promising new immunomodulatory molecules found in excretory/secretory products of helminths and their potential use as immunotherapies for acute and chronic inflammatory diseases.

  17. Autoimmune connective tissue disease: scleroderma.

    Science.gov (United States)

    Wilson, Helen; Vincent, Rachel

    Scleroderma is an umbrella term for a spectrum of rare and complex autoimmune connective tissue diseases, the cause and pathogenesis of which is only partially defined. Scleroderma can be divided into two main subgroups--systemic and localized--but the hallmark of both is skin fibrosis. As yet no drug has been found to be effective in reversing the disease process, however early intervention has been shown to give maximum benefit. Due to the chronic nature of the condition a multidisciplinary approach is essential and the nurse's input from an early stage is vital in supporting the patient to manage both their medical treatment and their activities of daily living.

  18. 重症大疱性药疹角质形成细胞凋亡的研究%Keratinocyte apoptosis in severe bullous drug eruptions

    Institute of Scientific and Technical Information of China (English)

    于燕华; 张美华

    2009-01-01

    Both Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) belong to severe bullous drug eruptions. Both of them are clinically characterized by extensive epidermal necrosis and exfoliation, and histologically characterized by generalized apoptosis of keratinocytes, a process that results in the separation of epidermis from dermis. There is evidence that keratinocyte apoptosis is mainly ascribed to perforin/granzyme B and Fas/FasL. Besides supportive treatment, the application of multiple antiapoptotic agents could be of potential benefit to the management of severe bullous drug eruptions. This article presents the antiapoptotic therapy of severe bullous drug eruptions.%重症多形红斑和中毒性表皮坏死松解症属于重症大疱性药疹,临床表现为广泛的表皮坏死剥脱,组织病理表现为广泛的角质形成细胞凋亡,表皮与真皮分离.研究认为,穿孔素/颗粒酶和Fas/FasL可能是参与诱导角质形成细胞凋亡的主要因素;提出可在支持疗法基础上联合应用多种抗凋亡药物.因此,文中对重症大疱性药疹的抗凋亡治疗进行综述.

  19. Effectiveness and safety of endothelial keratoplasty for pseudophakic and aphakic bullous keratopathy: a systematic review of randomized controlled trials and cohort studies

    Directory of Open Access Journals (Sweden)

    Bruno Harfuch

    2016-06-01

    Full Text Available ABSTRACT Objective: For nearly a century, penetrating keratoplasty has been the surgical technique of choice in the management of corneal changes. However, in recent years, several lamellar keratoplasty techniques have been developed, modified or improved, especially techniques for replacing the posterior portion, for the correction of bullous keratopathy. The aim of this study was to evaluate the effectiveness and safety of endothelial keratoplasty versus penetrating keratoplasty for pseudophakic and aphakic bullous keratopathy. Methods: A systematic review of the literature was carried out, and the main electronic databases were searched. The date of the most recent search was from the inception of the electronic databases to December 11, 2015. Two authors independently selected relevant clinical trials, assessed their methodological quality and extracted data. Results: The electronic search yielded a total of 893 published papers from the electronic databases. Forty-four full-text articles were retrieved for further consideration. Of these 44 full-text articles, 33 were excluded because they were all case series studies; therefore, ten studies (with one further publication met the inclusion criteria: one randomized clinical trial with two publications; three controlled studies; and six cohort studies. The clinical and methodological diversity found in the included studies meant that it was not possible to combine studies in a metaanalysis. Conclusions: There is no robust evidence that endothelial keratoplasty is more effective and safe than penetrating keratoplasty for improving visual acuity and decreasing corneal rejection for pseudophakic and aphakic bullous keratopathy. There is a need for further randomized controlled trials.

  20. Multiple Autoimmune Syndromes Associated with Psoriasis: A Rare Clinical Presentation

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    Sadia Masood

    2014-03-01

    Full Text Available Autoimmune diseases are known to have association with each other but it is very rare to see multiple autoimmune diseases in one patient. The combination of at least three autoimmune diseases in the same patient is referred to as multiple autoimmune syndrome. The case we are reporting features multiple autoimmune syndrome with five different conditions. The patient had type 1 diabetes mellitus, autoimmune hemolytic anemia, systemic lupus erythematosus, vitiligo, and psoriasis. Psoriasis has rarely been reported previously under the spectrum of autoimmune syndrome. Although the relationship of autoimmune conditions with each other has been explored in the past, this case adds yet another dimension to the unique evolution of autoimmune pathologies. The patient presented with a combination of five autoimmune diseases, which makes it consistent type three multiple autoimmune syndromes with the addition of psoriasis. The current case is unique in this aspect that the combination of these five autoimmune disorders has never been reported in the past.

  1. Cardiovascular Involvement in Autoimmune Diseases

    Directory of Open Access Journals (Sweden)

    Jenny Amaya-Amaya

    2014-01-01

    Full Text Available Autoimmune diseases (AD represent a broad spectrum of chronic conditions that may afflict specific target organs or multiple systems with a significant burden on quality of life. These conditions have common mechanisms including genetic and epigenetics factors, gender disparity, environmental triggers, pathophysiological abnormalities, and certain subphenotypes. Atherosclerosis (AT was once considered to be a degenerative disease that was an inevitable consequence of aging. However, research in the last three decades has shown that AT is not degenerative or inevitable. It is an autoimmune-inflammatory disease associated with infectious and inflammatory factors characterized by lipoprotein metabolism alteration that leads to immune system activation with the consequent proliferation of smooth muscle cells, narrowing arteries, and atheroma formation. Both humoral and cellular immune mechanisms have been proposed to participate in the onset and progression of AT. Several risk factors, known as classic risk factors, have been described. Interestingly, the excessive cardiovascular events observed in patients with ADs are not fully explained by these factors. Several novel risk factors contribute to the development of premature vascular damage. In this review, we discuss our current understanding of how traditional and nontraditional risk factors contribute to pathogenesis of CVD in AD.

  2. Novel Immunotherapies for Autoimmune Hepatitis

    Science.gov (United States)

    Cassim, Shamir; Bilodeau, Marc; Vincent, Catherine; Lapierre, Pascal

    2017-01-01

    Autoimmune hepatitis (AIH) is a multifactorial autoimmune disease of unknown pathogenesis, characterized by a loss of immunological tolerance against liver autoantigens resulting in the progressive destruction of the hepatic parenchyma. Current treatments are based on non-specific immunosuppressive drugs. Although tremendous progress has been made using specific biological agents in other inflammatory diseases, progress has been slow to come for AIH patients. While current treatments are successful in the majority of patients, treatment discontinuation is difficult to achieve, and relapses are frequent. Lifelong immunosuppression is not without risks, especially in the pediatric population; 4% of patient with type 1 AIH will eventually develop hepatocellular carcinoma with a 2.9% probability after 10 years of treatment. Therefore, future treatments should aim to restore tolerance to hepatic autoantigens and induce long-term remission. Promising new immunotherapies have been tested in experimental models of AIH including T and B cell depletion and regulatory CD4+ T cells infusion. Clinical studies on limited numbers of patients have also shown encouraging results using B-cell-depleting (rituximab) and anti-TNF-α (infliximab) antibodies. A better understanding of key molecular targets in AIH combined with effective site-specific immunotherapies could lead to long-term remission without blanket immunosuppression and with minimal deleterious side effects. PMID:28184367

  3. Autoimmune atrophic gastritis: current perspectives

    Science.gov (United States)

    Minalyan, Artem; Benhammou, Jihane N; Artashesyan, Aida; Lewis, Michael S; Pisegna, Joseph R

    2017-01-01

    At present there is no universally accepted classification for gastritis. The first successful classification (The Sydney System) that is still commonly used by medical professionals was first introduced by Misiewicz et al in Sydney in 1990. In fact, it was the first detailed classification after the discovery of Helicobacter pylori by Warren and Marshall in 1982. In 1994, the Updated Sydney System was proposed during the International Workshop on the Histopathology of Gastritis followed by the publication in The American Journal of Surgical Pathology by Dixon et al. Using the new classification, distinction between atrophic and nonatrophic gastritis was revised, and the visual scale grading was incorporated. According to the Updated Sydney System Classification, atrophic gastritis is categorized into multifocal (H. pylori, environmental factors, specific diet) and corpus-predominant (autoimmune). Since metaplasia is a key histological characteristic in patients with atrophic gastritis, it has been recommended to use the word “metaplastic” in both variants of atrophic gastritis: autoimmune metaplastic atrophic gastritis (AMAG) and environmental metaplastic atrophic gastritis. Although there are many overlaps in the course of the disease and distinction between those two entities may be challenging, the aim of this review article was to describe the etiology, epidemiology, pathogenesis, diagnosis, clinical manifestations and treatment in patients with AMAG. However, it is important to mention that H. pylori is the most common etiologic factor for the development of gastritis in the world. PMID:28223833

  4. Susceptibility Genes in Thyroid Autoimmunity

    Directory of Open Access Journals (Sweden)

    Yoshiyuki Ban

    2005-01-01

    Full Text Available The autoimmune thyroid diseases (AITD are complex diseases which are caused by an interaction between susceptibility genes and environmental triggers. Genetic susceptibility in combination with external factors (e.g. dietary iodine is believed to initiate the autoimmune response to thyroid antigens. Abundant epidemiological data, including family and twin studies, point to a strong genetic influence on the development of AITD. Various techniques have been employed to identify the genes contributing to the etiology of AITD, including candidate gene analysis and whole genome screening. These studies have enabled the identification of several loci (genetic regions that are linked with AITD, and in some of these loci, putative AITD susceptibility genes have been identified. Some of these genes/loci are unique to Graves' disease (GD and Hashimoto's thyroiditis (HT and some are common to both the diseases, indicating that there is a shared genetic susceptibility to GD and HT. The putative GD and HT susceptibility genes include both immune modifying genes (e.g. HLA, CTLA-4 and thyroid specific genes (e.g. TSHR, Tg. Most likely, these loci interact and their interactions may influence disease phenotype and severity.

  5. Propylthiouracil-induced autoimmune disease

    Directory of Open Access Journals (Sweden)

    Santosh Paiaulla

    2015-01-01

    Full Text Available Hyperthyroidism is a condition characterized by excessive production of thyroid hormones. Propylthiouracil (PTU is commonly used as first line drug in the management of hyperthyroidism. This is a case report of 24-year-old female, a known case of hyperthyroidism since 4 years, who came with a history of fever and myalgia since 3 days and dyspnea with coughing out of blood since 1 day. Patient was taking PTU (100 mg per day since 4 years for hyperthyroidism. Patient was immediately intubated for type-II respiratory failure. Diagnosed to be having PTU-induced autoimmune disease. PTU was stopped and treated with methylprednisolone and cyclophosphamide. Clinical features improved over a period of 8 days and discharged home successfully. Having a high suspicion for the onset of autoimmune disease in hyperthyroidism patients who are on PTU therapy and timely treatment with immunosuppressants and supportive care along with the withdrawal of the drug can make a difference in morbidity and mortality.

  6. Novel Immunotherapies for Autoimmune Hepatitis.

    Science.gov (United States)

    Cassim, Shamir; Bilodeau, Marc; Vincent, Catherine; Lapierre, Pascal

    2017-01-01

    Autoimmune hepatitis (AIH) is a multifactorial autoimmune disease of unknown pathogenesis, characterized by a loss of immunological tolerance against liver autoantigens resulting in the progressive destruction of the hepatic parenchyma. Current treatments are based on non-specific immunosuppressive drugs. Although tremendous progress has been made using specific biological agents in other inflammatory diseases, progress has been slow to come for AIH patients. While current treatments are successful in the majority of patients, treatment discontinuation is difficult to achieve, and relapses are frequent. Lifelong immunosuppression is not without risks, especially in the pediatric population; 4% of patient with type 1 AIH will eventually develop hepatocellular carcinoma with a 2.9% probability after 10 years of treatment. Therefore, future treatments should aim to restore tolerance to hepatic autoantigens and induce long-term remission. Promising new immunotherapies have been tested in experimental models of AIH including T and B cell depletion and regulatory CD4(+) T cells infusion. Clinical studies on limited numbers of patients have also shown encouraging results using B-cell-depleting (rituximab) and anti-TNF-α (infliximab) antibodies. A better understanding of key molecular targets in AIH combined with effective site-specific immunotherapies could lead to long-term remission without blanket immunosuppression and with minimal deleterious side effects.

  7. Cystic Lesions in Autoimmune Pancreatitis

    Directory of Open Access Journals (Sweden)

    Macarena Gompertz

    2015-11-01

    Full Text Available Autoimmune pancreatitis (AIP can be chronic or recurrent, but frequently completely reversible after steroid treatment. A cystic lesion in AIP is a rare finding, and it can mimic a pancreatic cystic neoplasm. Difficulties in an exact diagnosis interfere with treatment, and surgery cannot be avoided in some cases. We report the history of a 63-year-old male presenting with jaundice and pruritus. AIP was confirmed by imaging and elevated IgG4 blood levels, and the patient completely recovered after corticosteroid therapy. One year later, he presented with a recurrent episode of AIP with elevated IgG4 levels, accompanied by the appearance of multiple intrapancreatic cystic lesions. All but 1 of these cysts disappeared after steroid treatment, but the remaining cyst in the pancreatic head was even somewhat larger 1 year later. Pancreatoduodenectomy was finally performed. Histology showed the wall of the cystic lesion to be fibrotic; the surrounding pancreatic tissue presented fibrosis, atrophy and lymphoplasmacytic infiltration by IgG4-positive cells, without malignant elements. Our case illustrates the rare possibility that cystic lesions can be part of AIP. These pseudocysts appear in the pancreatic segments involved in the autoimmune disease and can be a consequence of the local inflammation or related to ductal strictures. Steroid treatment should be initiated, after which these cysts can completely disappear with recovery from AIP. Surgical intervention may be necessary in some exceptional cases.

  8. [Autoimmune Diseases of Digestive System].

    Science.gov (United States)

    Ivashkinl, V T; Sheptulina, A F; Raĭkhelson, K L; Losik, E A; Ivashkin, K V; Okhlobystin, A V; Baranskaia, E K; Polouvektova, E A; Shifrin, O S

    2015-01-01

    Autoimmune diseases of digestive system refer to pathological conditions, caused by autoimmune mechanisms, and their etiology remains unknown. This is a group of relatively rare diseases, however, during the last years a marked tendency towards the raise in incidence andprevalence is observed, which led to an increase in number of clinical investigations on etiology, pathogenesis, and, accordingly, development of new diagnostic methods and therapies. Results of such trials shown, for example, that the pathogenesis of chronic cholestatic liver diseases is associated with nuclear receptors function, while the main etiological and pathogenic factor of inflammatory bowel diseases represents gut microbiota. Despite new achievements in autoinmune diseases of digestive system research, therapies are low effective and are accompanied by a huge number of adverse events. The fact that these diseases may lead to malignant tumors is also worth noting. For example, patients with primary sclerosing cholangitis have a 160 times higher risk of cholangiocellular carcinoma, while 10-14% ofpatients with celiac disease may develop malignancies of esophagus, small and large intestine. Thus, these diseases require further investigation with a purpose of more accurate diagnostic methods for the detection of disease at early stages and new effective and safe therapies development.

  9. Gender and autoimmune comorbidity in multiple sclerosis

    DEFF Research Database (Denmark)

    Magyari, Melinda; Koch-Henriksen, Nils; Pfleger, Claudia C

    2014-01-01

    BACKGROUND: The female preponderance in incidence of multiple sclerosis (MS) calls for investigations into sex differences in comorbidity with other autoimmune diseases (ADs). OBJECTIVES: To determine whether male and female patients with MS have a higher frequency of autoimmune comorbidity than...

  10. Autoimmune pancreatitis exhibiting multiple mass lesions.

    Science.gov (United States)

    Shiokawa, Masahiro; Kodama, Yuzo; Hiramatsu, Yukiko; Kurita, Akira; Sawai, Yugo; Uza, Norimitsu; Watanabe, Tomohiro; Chiba, Tsutomu

    2011-09-01

    Our case is a first report of autoimmune pancreatitis with multiple masses within the pancreas which was pathologically diagnosed by endoscopic ultrasound-guided fine needle aspiration and treated by steroid. The masses disappeared by steroid therapy. Our case is informative to know that autoimmune pancreatitis sometimes exhibits multiple masses within the pancreas and to diagnose it without unnecessary surgery.

  11. Autoimmune Pancreatitis Exhibiting Multiple Mass Lesions

    Directory of Open Access Journals (Sweden)

    Masahiro Shiokawa

    2011-09-01

    Full Text Available Our case is a first report of autoimmune pancreatitis with multiple masses within the pancreas which was pathologically diagnosed by endoscopic ultrasound-guided fine needle aspiration and treated by steroid. The masses disappeared by steroid therapy. Our case is informative to know that autoimmune pancreatitis sometimes exhibits multiple masses within the pancreas and to diagnose it without unnecessary surgery.

  12. Chronic autoimmune urticaria : Where we stand ?

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    Goh C

    2009-01-01

    Full Text Available It is well-recognized that 30-40% of chronic idiopathic urticaria is autoimmune in nature. Chronic autoimmune urticaria is caused by anti-FcåRI and less frequently, by anti-IgE autoantibodies that lead to mast cell and basophil activation, thereby giving rise to the release of histamine and other proinflammatory mediators. Activation of the classical complement pathway and formation of C5a are important in dermal mast cell activation. C5a is also a neutrophil and eosinophil chemoattractant. Chronic autoimmune urticaria has been found to be associated with autoimmune thyroid disease. The autologous serum skin test is used as a screening test for chronic autoimmune urticaria and has a sensitivity and specificity of about 70 and 80%, respectively. The current gold standard diagnostic test is the basophil histamine release assay. The treatment of chronic autoimmune urticaria, as in chronic idiopathic urticaria, is with H1 antihistamines. Oral corticosteroids may be used during acute flares. Refractory cases have been shown to respond to cyclosporine and other immunomodulators. The prevalence of chronic autoimmune urticaria in Singapore is similar to that reported in Western countries at about 42%. The presence of thyroid autoimmunity appears to be higher than reported, with 22.5% of patients with chronic idiopathic urticaria here, exhibiting presence of thyroid autoantibodies.

  13. Autoimmune hepatitis and juvenile systemic lupus erythematosus

    NARCIS (Netherlands)

    Deen, M. E. J.; Porta, G.; Fiorot, F. J.; Campos, L. M. A.; Sallum, A. M. E.; Silva, C. A. A.

    2009-01-01

    Juvenile systemic lupus erythematosus (JSLE) and autoimmune hepatitis (AIH) are both autoimmune disorders that are rare in children and have a widespread clinical manifestation. A few case reports have shown a JSLE-AIH associated disorder. To our knowledge, this is the first study that simultaneousl

  14. [Type 1 diabetes and autoimmune polyendocrine syndromes].

    Science.gov (United States)

    Queiroz, Márcia S

    2008-03-01

    Type 1 diabetes (T1D) is associated with autoimmune thyroid disease (AIT), celiac disease (CD), Addison's disease (AD), and other autoimmune diseases. These diseases can occur simultaneously in defined syndromes with distinct pathophysiology and characteristics: autoimmune polyendocrine syndromes (APSs) and the immunodysregulation polyendocrinopathy enteropathy X-linked syndrome (IPEX). APSs were initially defined as a multiple endocrine gland insufficiency associated to an autoimmune disease in a patient. APS-1 is characterized by the evidence of chronic candidiasis, chronic hypoparathyroidism, AD and T1D could be present as part of this syndrome. The combination of autoimmune adrenal insufficiency with AIT and/or type 1 autoimmune diabetes mellitus defines APS-2. AIT associated to other autoimmune diseases (excluding AD and/or hypoparathyroidism) are the main characteristics of APS-3. Different clinical combinations of autoimmune diseases which were not included in the previous groups are the characteristics of APS-4. IPEX is a recessive disorder characterized by the neonatal onset of T1D, infections, enteropathy, thrombocytopenia and anemia, as well as endocrinopathy, eczema and cachexia. These disorders are not common, but their consequences can be life threatening when the diagnosis is overlooked, and the treatment is the same prescribed for isolated disease presentation.

  15. Interferon-¿ regulates oxidative stress during experimental autoimmune encephalomyelitis

    DEFF Research Database (Denmark)

    Espejo, C.; Penkowa, Milena; Saez-Torres, I.;

    2002-01-01

    Neurobiology, experimental autoimmune encephalomyelitis IFN-d, multiple sclerosis, neurodegeneration, oxidative stress......Neurobiology, experimental autoimmune encephalomyelitis IFN-d, multiple sclerosis, neurodegeneration, oxidative stress...

  16. Autoimmune diseases in women with Turner's syndrome

    DEFF Research Database (Denmark)

    Jørgensen, Kristian T; Rostgaard, Klaus; Bache, Iben

    2010-01-01

    OBJECTIVE: In terms of number of X chromosomes, women with Turner's syndrome cytogenetically resemble men. An increased risk of autoimmune diseases has been observed among women with Turner's syndrome. This study was undertaken to investigate whether the autoimmune disease profile in women...... with Turner's syndrome is characterized by diseases with a female or male predominance. METHODS: Using the Danish Cytogenetic Central Register, the Danish National Patient Register, and the Danish Civil Registration System, we estimated relative risk of 46 different autoimmune diseases in a cohort of 798...... Danish women with Turner's syndrome followed up for 12,461 person-years between 1980 and 2004. Standardized incidence ratios (SIRs) of first hospitalization for autoimmune disease and 95% confidence intervals (95% CIs) were used as measures of relative risk. RESULTS: The overall risk of autoimmune...

  17. Hepatitis A vaccine associated with autoimmune hepatitis

    Institute of Scientific and Technical Information of China (English)

    PA Berry; G Smith-Laing

    2007-01-01

    To describe a case of probable relapsing autoimmune hepatitis associated with vaccination against hepatitis A virus (HAV). A case report and review of literature were written concerning autoimmune hepatitis in association with hepatitis A and other hepatotropic viruses. Soon after the administration of formalin-inactivated hepatitis A vaccine, a man who had recently recovered from an uncharacterized but self-limiting hepatitic illness,experienced a severe deterioration (AST 1687 U/L, INR 1.4). Anti-nuclear antibodies were detectable, and liver biopsy was compatible with autoimmune hepatitis. The observation supports the role of HAV as a trigger of autoimmune hepatitis. Studies in helper T-cell activity and antibody expression against hepatic proteins in the context of hepatitis A infection are summarized, and the concept of molecular mimicry with regard to other forms of viral hepatitis and autoimmunity is briefly explored.

  18. [Treatment with tacrolimus in autoimmune diseases].

    Science.gov (United States)

    Sádaba, B; Azanza, J R; García Quetglas, E; Fernández, V

    2004-01-01

    Tacrolimus is an immunosuppressive drug used most successfully as a primary drug to suppress the rejection of transplants. Tacrolimus may also be useful as a novel therapy for autoimmune disease. There are various reports in the bibliography about the use of tacrolimus in the treatment of some autoimmune diseases: inflammatory bowel disease, autoimmune hepatitis, cutaneous, neurologic, renal, endocrine or eye disease. In this review of more than 130 papers, we discuss the rationale for the use of tacrolimus in autoimmune disease and report the clinical experience with the drug in the management of a variety of autoimmune diseases. But, although there are a lot questions that require future research (dose, duration of treatment, when to begin tacrolimus treatment, how to monitor it, etc.), there is also wide experience with tacrolimus in the treatment of this type of disease.

  19. Recurrence of autoimmune liver diseases after livertransplantation

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    Liver transplantation (LT) is the most effective treatmentmodality for end stage liver disease caused by manyetiologies including autoimmune processes. That said,the need for transplantation for autoimmune hepatitis(AIH) and primary biliary cirrhosis (PBC), but not forprimary sclerosing cholangitis (PSC), has decreasedover the years due to the availability of effective medicaltreatment. Autoimmune liver diseases have superiortransplant outcomes than those of other etiologies. WhileAIH and PBC can recur after LT, recurrence is of limitedclinical significance in most, but not all cases. RecurrentPSC, however, often progresses over years to a stagerequiring re-transplantation. The exact incidence andthe predisposing factors of disease recurrence remaindebated. Better understanding of the pathogenesis andthe risk factors of recurrent autoimmune liver diseasesis required to develop preventive measures. In thisreview, we discuss the current knowledge of incidence,diagnosis, risk factors, clinical course, and treatmentof recurrent autoimmune liver disease (AIH, PBC, PSC)following LT.

  20. Diagnosis and classification of autoimmune gastritis.

    Science.gov (United States)

    Toh, Ban-Hock

    2014-01-01

    Autoimmune gastritis is a silent and highly prevalent disease that only becomes clinically manifested with progression to corpus atrophy and development of iron deficient or B12-deficient (pernicious) anaemia. Autoimmune gastritis is associated with autoimmune thyroiditis and type 1 diabetes mellitus. Corpus atrophy may be complicated by gastric carcinoids and gastric cancer. Laboratory diagnosis of autoimmune gastritis rests on serum biomarkers of antibody to parietal cell H/K ATPase and intrinsic factor and corpus atrophy on serum biomarkers of gastrin and pepsinogen levels. Subjects with asymptomatic parietal cell antibody should be regularly assessed for serum biomarkers for progression to corpus atrophy, development of iron and B12 deficiency anaemia and for associated autoimmune thyroiditis and type 1 diabetes mellitus.

  1. Association between autoimmune pancreatitis and systemic autoimmune diseases

    Institute of Scientific and Technical Information of China (English)

    Viktória Terzin; Imre F(o)ldesi; László Kovács; Gyula Pokorny; Tibor Wittmann; László Czakó

    2012-01-01

    AIM:To investigate the association between autoimmune pancreatitis (AIP) and systemic autoimmune diseases (SAIDs) by measurement of serum immunoglobulin G4 (IgG4).METHODS:The serum level of IgG4 was measured in 61 patients with SAIDs of different types who had not yet participated in glucocorticosteroid treatment.Patients with an elevated IgG4 level were examined by abdominal ultrasonography (US) and,in some cases,by computer tomography (CT).RESULTS:Elevated serum IgG4 levels (919 ± 996 mg/L) were detected in 17 (28%) of the 61 SAID patients.10 patients had Sj(o)gren's syndrome (SS) (IgG4:590 ±232 mg/L),2 of them in association with Hashimoto's thyroiditis,and 7 patients (IgG4:1388 ± 985.5 mg/L)had systemic lupus erythematosus (SLE).The IgG4 level in the SLE patients and that in patients with SS were not significantly different from that in AIP patients (783 ± 522 mg/L).Abdominal US and CT did not reveal any characteristic features of AIP among the SAID patients with an elevated IgG4 level.CONCLUSION:The serum IgG4 level may be elevated in SAIDs without the presence of AIP.The determination of serum IgG4 does not seem to be suitable for the differentiation between IgG4-related diseases and SAIDs.

  2. Homozygous ALOXE3 Nonsense Variant Identified in a Patient with Non-Bullous Congenital Ichthyosiform Erythroderma Complicated by Superimposed Bullous Majocchi’s Granuloma: The Consequences of Skin Barrier Dysfunction

    Directory of Open Access Journals (Sweden)

    Tao Wang

    2015-09-01

    Full Text Available Non-bullous congenital ichthyosiform erythroderma (NBCIE is a hereditary disorder of keratinization caused by pathogenic variants in genes encoding enzymes important to lipid processing and terminal keratinocyte differentiation. Impaired function of these enzymes can cause pathologic epidermal scaling, significantly reduced skin barrier function. In this study, we have performed a focused, genetic analysis of a probrand affected by NBCIE and extended this to his consanguineous parents. Targeted capture and next-generation sequencing was performed on NBCIE associated genes in the proband and his unaffected consanguineous parents. We identified a homozygous nonsense variant c.814C>T (p.Arg272* in ALOXE3 (NM_001165960.1 in the proband and discovered that his parents are both heterozygous carriers of the variant. The clinical manifestations of the proband’s skin were consistent with NBCIE, and detailed histopathological assessment revealed epidermal bulla formation and Majocchi’s granuloma. Infection with Trichophyton rubrum was confirmed by culture. The patient responded to oral terbinafine antifungal treatment. Decreased skin barrier function, such as that caused by hereditary disorders of keratinization, can increase the risk of severe cutaneous fungal infections and the formation of Majocchi’s granuloma and associated alopecia. Patients with NBCIE should be alerted to the possible predisposition for developing dermatophytoses and warrant close clinical follow-up.

  3. Diagnostic criteria of autoimmune hepatitis.

    Science.gov (United States)

    Liberal, Rodrigo; Grant, Charlotte R; Longhi, Maria Serena; Mieli-Vergani, Giorgina; Vergani, Diego

    2014-01-01

    Autoimmune hepatitis (AIH) is a chronic immune-mediated liver disorder characterised by female preponderance, elevated transaminase and immunoglobulin G levels, seropositivity for autoantibodies and interface hepatitis. Presentation is highly variable, therefore AIH should be considered during the diagnostic workup of any increase in liver enzyme levels. A set of inclusion and exclusion criteria for the diagnosis of AIH have been established by the International Autoimmune Hepatitis Group (IAIHG). There are two main types of AIH: type 1, positive for anti-nuclear (ANA) and/or anti-smooth muscle antibodies (SMAs) and type 2, defined by the presence of anti-liver kidney microsomal antibody type 1 (LKM-1) and/or anti-liver cytosol type 1 (LC-1) autoantibodies. The central role of autoantibodies in the diagnosis of AIH has led the IAIHG to produce a consensus statement detailing appropriate and effective methods for their detection. Autoantibodies should be tested by indirect immunofluorescence at an initial dilution of 1/40 in adults and 1/10 in children on a freshly prepared rodent substrate that includes kidney, liver and stomach sections to allow for the simultaneous detection of all reactivities relevant to AIH. Anti-LKM-1 is often confused with anti-mitochondrial antibody (AMA) if rodent kidney is used as the sole immunofluorescence substrate. The identification of the molecular targets of anti-LKM-1 and AMA has led to the establishment of immuno-assays based on the use of the recombinant or purified autoantigens. Perinuclear anti-nuclear neutrophil antibody (p-ANNA) is an additional marker of AIH-1; anti soluble liver antigen (SLA) antibodies are specific for autoimmune liver disease, can be present in AIH-1 and AIH-2 and are associated with a more severe clinical course. Anti-SLA are detectable by ELISA or radio-immuno-assays, but not by immunofluorescence. AIH is exquisitely responsive to immunosuppressive treatment, which should be instituted promptly to

  4. The Immunogenetics of Autoimmune Cholestasis.

    Science.gov (United States)

    Trivedi, Palak J; Hirschfield, Gideon M

    2016-02-01

    The immune-mediated hepatobiliary diseases, primary biliary cirrhosis and primary sclerosing cholangitis are relatively rare, albeit and account for a significant amount of liver transplant activity and liver-related mortality globally. Precise disease mechanisms are yet to be described although a contributory role of genetic predisposition is firmly established. In addition to links with the major histocompatibility complex, a number of associations outside this region harbor additional loci which underscore the fundamental role of breaks in immune tolerance and mucosal immunogenicity in the pathogenesis of autoimmune biliary disease. We provide an overview of these key discoveries before discussing putative avenues of therapeutic exploitation based on existing findings. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. Helminth Immunomodulation in Autoimmune Disease

    Directory of Open Access Journals (Sweden)

    John J. Miles

    2017-04-01

    Full Text Available Helminths have evolved to become experts at subverting immune surveillance. Through potent and persistent immune tempering, helminths can remain undetected in human tissues for decades. Redirecting the immunomodulating “talents” of helminths to treat inflammatory human diseases is receiving intensive interest. Here, we review therapies using live parasitic worms, worm secretions, and worm-derived synthetic molecules to treat autoimmune disease. We review helminth therapy in both mouse models and clinical trials and discuss what is known on mechanisms of action. We also highlight current progress in characterizing promising new immunomodulatory molecules found in excretory/secretory products of helminths and their potential use as immunotherapies for acute and chronic inflammatory diseases.

  6. Prevalência de dermatoses em escolares na região do ABC paulista Prevalence of dermatosis in scholars in the region of ABC paulista

    Directory of Open Access Journals (Sweden)

    Cristina Marta Maria Laczynski

    2011-06-01

    Full Text Available FUNDAMENTOS: A literatura mostra que a prevalência de doenças dermatológicas em crianças e adolescentes com idades de 7-14 anos é consideravelmente significante, bem como a falta de tratamento e possível agravamento da situação. OBJETIVOS: Realização de uma análise investigativa para apontar a prevalência de dermatoses em indivíduos de 714 anos, assim como para verificar se estas estão diretamente ligadas ou não a suas respectivas situações socioeconômicas. MÉTODOS: Analisaram-se 200 crianças e adolescentes com idades de 7-14 anos, entre os meses de agosto e novembro de 2006, sendo 100 crianças em duas escolas públicas localizadas na periferia e 100 em duas escolas particulares localizadas em bairro de classe média alta na cidade de Santo André, Grande São Paulo. Para tanto, utilizou-se o método investigativo, com exame clínico de toda a superfície corpórea, de mucosas e de gânglios palpáveis, sendo a amostra populacional analisada de acordo com o teste qui-quadrado. RESULTADOS: 87,5% das crianças apresentaram algum tipo de dermatose; encontraram-se 46 dermatoses, destacando-se: a nevos melânicos (pBACKGROUND: Literature shows that prevalence of skin diseases in children and adolescents aged 7-14 years is very significant, as well as lack of treatment worsening the situation. OBJECTIVES: An investigative analysis was conducted to determine the prevalence of skin diseases in individuals 714 years and focus on whether or not they are related directly to their respective socio-economic status. METHODS: 200 children and adolescents were examined aged 7-14 years,between August and November 2006,100 children in two public schools located in the periphery and 100 in two private schools located in upper middleclass neighborhood in Santo André,located in São Paulo.Investigative method used was the clinical examination of the whole skin, mucous areas and palpable ganglia,and the population sample analyzed according to

  7. Successful Treatment of Chronic Staphylococcus aureus-Related Dermatoses with the Topical Endolysin Staphefekt SA.100: A Report of 3 Cases

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    Joan E.E. Totté

    2017-05-01

    Full Text Available Staphylococcus aureus plays an important role in skin and soft tissue infections and contributes to the pathophysiology of complex skin disorders such as atopic dermatitis. Bacterial resistance against commonly used antibiotics has increased considerably in the last decades demanding alternative treatment approaches. We present 3 cases where patients with chronic and recurrent S. aureus-related dermatoses were successfully treated with Staphefekt SA.100. Staphefekt SA.100 is a recombinant phage endolysin for topical skin application that specifically targets both methicillin-sensitive and methicillin-resistant S. aureus. As a consequence of its specific mechanism of action, bacterial resistance is unlikely to develop. In our 3 cases, resistance induction was not observed. Our results indicate that targeted treatment with Staphefekt might be an attractive alternative for (long-term classical antibiotic therapy, and confirmatory randomized controlled trials are warranted to evaluate its clinical efficacy and safety.

  8. Rett syndrome: An autoimmune disease?

    Science.gov (United States)

    De Felice, Claudio; Leoncini, Silvia; Signorini, Cinzia; Cortelazzo, Alessio; Rovero, Paolo; Durand, Thierry; Ciccoli, Lucia; Papini, Anna Maria; Hayek, Joussef

    2016-04-01

    Rett syndrome (RTT) is a devastating neurodevelopmental disease, previously included into the autistic spectrum disorders, affecting almost exclusively females (frequency 1:10,000). RTT leads to intellective deficit, purposeful hands use loss and late major motor impairment besides featuring breathing disorders, epilepsy and increased risk of sudden death. The condition is caused in up to 95% of the cases by mutations in the X-linked methyl-CpG binding protein 2 (MECP2) gene. Our group has shown a number of previously unrecognized features, such as systemic redox imbalance, chronic inflammatory status, respiratory bronchiolitis-associated interstitial lung disease-like lung disease, and erythrocyte morphology changes. While evidence on an intimate involvement of MeCP2 in the immune response is cumulating, we have recently shown a cytokine dysregulation in RTT. Increasing evidence on the relationship between MeCP2 and an immune dysfunction is reported, with, apparently, a link between MECP2 gene polymorphisms and autoimmune diseases, including primary Sjögren's syndrome, systemic lupus erythematosus, rheumatoid arthritis, and systemic sclerosis. Antineuronal (i.e., brain proteins) antibodies have been shown in RTT. Recently, high levels of anti-N-glucosylation (N-Glc) IgM serum autoantibodies [i.e., anti-CSF114(N-Glc) IgMs] have been detected by our group in a statistically significant number of RTT patients. In the current review, the Authors explore the current evidence, either in favor or against, the presence of an autoimmune component in RTT.

  9. Natural killer T cells and immune dermatoses%自然杀伤T细胞与皮肤免疫性疾病的研究进展

    Institute of Scientific and Technical Information of China (English)

    李霞; 李凯; 李春英; 高天文

    2012-01-01

    Natural killer T (NKT) cells display the properties of both T and NK cells.They protect the host from pathogens,tumors,alloantigens,and autoantigens by regulating the Th1/Th2 balance and maintaining immune homeostasis.Recent studies have shown that NKT cells play an important role in immune dermatoses.The authors review advances in the relationship of NKT cells with allergic contact dermatitis,atopic dermatitis,psoriasis,systemic lupus erythematosus,which may provide new ideas for basic researches and clinical practice related to immune dermatoses.%自然杀伤T细胞能表现出T细胞和自然杀伤细胞的双重作用,在机体抵御病原体、肿瘤、异体抗原及自身抗原过程中自然杀伤细胞调节Th1/Th2平衡,协助机体免疫应答和维持免疫稳态.近年研究表明,自然杀伤T细胞在皮肤免疫性疾病中起关键作用,概述自然杀伤T细胞与过敏性接触性皮炎、特应性皮炎、银屑病和系统性红斑狼疮的研究进展,以期为皮肤免疫性疾病的研究和医疗领域提供新思路.

  10. 嗜酸粒细胞增多综合征和嗜酸性皮肤病%Hypereosinophilic syndrome and eosinophilic dermatoses

    Institute of Scientific and Technical Information of China (English)

    章星琪

    2012-01-01

    Hypereosinophilic syndrome (HES) constitutes a heterogeneous group of disorders characterized by persistent peripheral blood eosinophilia,associated with organ damage or dysfunction.There are many subtypes of HES,including myeloproliferative,lymphocytic,overlap,familial,associated,and undefined variants,which may be of benign or malignant origins.Eosinophilic dermatoses refer to a heterogeneous group of diseases characterized by an inflammatory infiltration of eosinophils in skin tissue,such as insect bites,drug eruption, pregnancy-related dermatoses, urticaria, angioedema, allergic contact dermatitis, and atopic dermatitis,as well as a group of diseases with distinct histological features,such as Well's syndrome,Kimura's disease,eosinophilic pustular folliculitis,granuloma faciale,etc.%嗜酸粒细胞增多综合征是一组以外周血嗜酸粒细胞持续性升高,伴有器官损害或功能不全的疾病,可分为髓系增生性、淋巴细胞性、重叠型、家族性、伴随现象和未分类等临床亚型,其中包括良性和恶性疾病.嗜酸性皮病是指以皮肤组织炎性浸润中存在嗜酸粒细胞为特征的一组皮肤病,常见的有昆虫叮咬、药疹、妊娠性皮病、荨麻疹、血管性水肿、过敏性接触性皮炎和特应性皮炎等,还包括一些具有组织病理特点的疾病,如Well综合征、木村病、嗜酸粒细胞增多性脓疱性毛囊炎、面部肉芽肿等.

  11. How pregnancy can affect autoimmune diseases progression?

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    Piccinni, Marie-Pierre; Lombardelli, Letizia; Logiodice, Federica; Kullolli, Ornela; Parronchi, Paola; Romagnani, Sergio

    2016-01-01

    Autoimmune disorders are characterized by tissue damage, caused by self-reactivity of different effectors mechanisms of the immune system, namely antibodies and T cells. Their occurrence may be associated with genetic and/or environmental predisposition and to some extent, have implications for fertility and obstetrics. The relationship between autoimmunity and reproduction is bidirectional. This review only addresses the impact of pregnancy on autoimmune diseases and not the influence of autoimmunity on pregnancy development. Th17/Th1-type cells are aggressive and pathogenic in many autoimmune disorders and inflammatory diseases. The immunology of pregnancy underlies the role of Th2-type cytokines to maintain the tolerance of the mother towards the fetal semi-allograft. Non-specific factors, including hormonal changes, favor a switch to Th2-type cytokine profile. In pregnancy Th2, Th17/Th2 and Treg cells accumulate in the decidua but may also be present in the mother's circulation and can regulate autoimmune responses influencing the progression of autoimmune diseases.

  12. Experimental drugs for treatment of autoimmune myocarditis

    Institute of Scientific and Technical Information of China (English)

    Han Lina; Guo Shuli; Wang Yutang; Yang Liming; Liu Siyu

    2014-01-01

    Objective To review the experimental drugs for the treatment of autoimmune myocarditis.Data sources The literatures published in English about different kinds of experimental drugs based on different therapeutic mechanisms for the treatment of autoimmune myocarditis were obtained from PubMed from 2002 to 2013.Study selection Original articles regarding the experimental drugs for treatment of autoimmune myocarditis were selected.Results This study summarized the effects of the experimental drugs for the treatment of autoimmune myocarditis,such as immunomodulators and immunosuppressants,antibiotics,Chinese medicinal herbs,cardiovascular diseases treatment drugs,etc.These drugs can significantly attenuate autoimmune myocarditis-induced inflammation and fibrosis,alleviate autoimmune myocarditis-triggered overt lymphocyte proliferation,and meanwhile reduce Th1 cytokines (IFN-γ and IL-2) and increase Th2 cytokines (IL-4 and IL-10).Conclusion This study summarized recent advances in autoimmune myocarditis treatment and further proposes that traditional Chinese medicine and immune regulators will play important roles in the future.

  13. Criteria for Environmentally Associated Autoimmune Diseases

    Science.gov (United States)

    Pollard, K. Michael; Parks, Christine G.; Germolec, Dori R.; Leung, Patrick S.C.; Selmi, Carlo; Humble, Michael C.; Rose, Noel R.

    2012-01-01

    Increasing evidence supports a role for the environment in the development of autoimmune diseases, as reviewed in the accompanying three papers from the National Institute of Environmental Health Sciences Expert Panel Workshop. An important unresolved issue, however, is the development of criteria for identifying autoimmune disease phenotypes for which the environment plays a causative role, herein referred to as environmentally associated autoimmune diseases. There are several different areas in which such criteria need to be developed, including: 1) identifying the necessary and sufficient data to define environmental risk factors for autoimmune diseases meeting current classification criteria; 2) establishing the existence of and criteria for new environmentally associated autoimmune disorders that do not meet current disease classification criteria; and 3) identifying in clinical practice specific environmental agents that induce autoimmune disease in individual patients. Here we discuss approaches that could be useful for developing criteria in these three areas, as well as factors that should be considered in evaluating the evidence for criteria that can distinguish individuals with such disorders from individuals without such disorders with high sensitivity and specificity. Current studies suggest that multiple lines of complementary evidence will be important and that in many cases there will be clinical, serologic, genetic, epigenetic, and/or other laboratory features that could be incorporated as criteria for environmentally associated autoimmune diseases to improve diagnosis and treatment and possibly allow for preventative strategies in the future. PMID:22771005

  14. Polyglandular Autoimmune Syndrome in pregnancy: case report

    Science.gov (United States)

    Pecorino, Basilio; Teodoro, Maria Cristina; Scollo, Paolo

    2016-01-01

    Type III Polyglandular Autoimmune Syndrome is a multiple endocrine disorders disease determined by autoimmunity; it can be diagnosed if a patient is affected by Type 1 Diabetes Mellitus and another autoimmune disease, except Addison Disease, for example Autoimmune Hashimoto Thyroiditis or Celiac Disease. R.D., 34-year-old woman (gravida 2 para 1), was referred to the High Risk Pregnancy Outpatient Clinic at Cannizzaro Hospital in Catania at 8 weeks' gestation. She was affected from type III Polyglandular Autoimmune Disease (Type 1 Diabetes Mellitus, Autoimmune Hashimoto Thyroiditis and Celiac Disease). Pre-conception glycated hemoglobin and thyrotropin levels were normal. This pregnancy was characterized by glycemic instability and the need to increase the insulin units every month. The patient was hospitalized at 32+6 weeks for monitoring fetus and mother health because of inadequate glycemic control and the high insulin dosage required. She was delivered by caesarean section at 36+6 weeks because of uterine contractions, the previous cesarean section, glycemic instability and the gestational age. She delivered a baby boy, birth-weight 3300 g, Apgar 8-9. She was discharged in the fourth day after delivery with good maternal and child prognosis. Literature data and the experience derived by this case report suggest some recommendations to improve obstetrics and neonatologist outcome in the patients affected from type III Polyglandular Autoimmune Syndrome: pre-conception counseling, thyrotropin assay every 4-6 weeks, gluten-free diet, fasting and post-prandial blood glucose level targets. PMID:27917035

  15. Diagnosis and classification of autoimmune orchitis.

    Science.gov (United States)

    Silva, C A; Cocuzza, M; Carvalho, J F; Bonfá, E

    2014-01-01

    Autoimmune orchitis is characterized by testis inflammation and the presence of specific antisperm antibodies (ASA). It is classified in two categories. Primary autoimmune orchitis is defined by infertility and asymptomatic orchitis associated with ASA (100%) directed to the basement membrane or seminiferous tubules in infertile men, without any systemic disease and usually asymptomatic. Secondary autoimmune orchitis is characterized by symptomatic orchitis and/or testicular vasculiti`s associated with a systemic autoimmune disease, particularly vasculitis. These patients typically demonstrate testicular pain, erythema and/or swelling. ASA in secondary autoimmune orchitis have been reported in up to 50% of patients, especially in systemic lupus erythematosus patients. The pathogenesis of primary as well as secondary autoimmune orchitis is still unknown. Although the etiology is likely to be multifactorial, testicular inflammation, infection or trauma may induce T cell response with pro-inflammatory cytokine production with a consequent blood-testis-barrier permeability alteration, ASA production and apoptosis of spermatocytes and spermatids. ASA is known to cause immobilization and/or agglutination of spermatozoa, which may block sperm-egg interaction resulting in infertility. Assisted reproduction has been used as an efficient option in primary cases and immunosuppressive therapy for secondary autoimmune orchitis, although there is no double-blind, randomized trial to confirm the efficacy of any treatment regimens for these conditions.

  16. Cutting-edge issues in autoimmune orchitis.

    Science.gov (United States)

    Silva, Clovis A; Cocuzza, Marcello; Borba, Eduardo F; Bonfá, Eloísa

    2012-04-01

    Autoimmune orchitis is a relevant cause of decreased fecundity in males, and it is defined as a direct aggression to the testis with the concomitant presence of anti-sperm antibodies (ASA). The presence of these specific antibodies has been observed in approximately 5-12% of infertile male partners. Primary autoimmune orchitis is defined by isolated infertility with ASA but without evidence of a systemic disease. Secondary causes of orchitis and/or testicular vasculitis are uniformly associated with autoimmune diseases, mainly in primary vasculitis such as polyarteritis nodosa, Behçet's disease, and Henoch-Schönlein purpura. The overall frequencies of acute orchitis and ASA in rheumatic diseases are 2-31% and 0-50%, respectively. The pathogenesis of primary/secondary autoimmune orchitis is not completely understood but probably involves the access of immune cells to the testicular microenvironment due to inflammation, infection or trauma, leading to apoptosis of spermatocytes and spermatids. Glucocorticoids and immunosuppressive drugs are indicated in autoimmune orchitis-associated active systemic autoimmune diseases. However, there are no standardized treatment options, and the real significance of ASA in infertile men is still controversial. Assisted reproductive technologies such as intrauterine insemination, in vitro fertilization, and intracytoplasmic sperm injection (ICSI) are therapeutic options for male infertility associated with these autoantibodies. ICSI is considered to be the best choice for patients with severe sperm autoimmunity, particularly in males with low semen counts or motility.

  17. A CONTROVERSIAL ON THE DIAGNOSIS OF CHRONIC BULLOUS TYPE MUCOCUTANEOUS DISEASE INVOLVING ORAL MUCOSA (A CASE REPORT

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    Isadora Gracia

    2006-04-01

    Full Text Available A case of chronic bullous type mucocutaneous disease involving oral mucosa was reported from a 56 years old man with never healing oral ulcers and wound on the perianal skin for three years. There were also red and black spots on the limb and back skin and a lesion on nail. Painful oral lesion consisted of mucous erosion, desquamative gingivitis, and sloughing area on palate and tongue. The patient is diabetic. The first perianal skin diagnosis was granulomatous candidasis with differential diagnosis pemphigus vegetates and acuminarum condiloma. However the histopathologic examination did not support these diagnosis. After several histopathologic examinations, the latest perianal skin diagnosis was lichen planus with differential diagnosis granulomatous vasculitis, bowenoid papulosis and pyodema gangrenosum. Other skin diagnosis was erythema multiforme. Oral diagnosis was mucous membrane pemphigoid with differential diagnosis lichen planus, Behçet's syndrome and erythema multiforme. Oral histopathologic examinations showed a sub-epithelial blister, which supported mucous membrane pemphigoid. A lip balm, prednisone 5 mg oral rinse and multivitamins were given but oral improvement started after blood sugar level was controlled. Conclusion: It is not yet known whether skin and oral mucous lesions are from the same disease or not.

  18. A new diagnosis of Williams-Beuren syndrome in a 49-year-old man with severe bullous emphysema.

    Science.gov (United States)

    Wojcik, Monica H; Carmichael, Nikkola; Bieber, Frederick R; Wiener, Daniel C; Madan, Rachna; Pober, Barbara R; Raby, Benjamin A

    2017-08-01

    Williams-Beuren syndrome (WBS) is a chromosomal microdeletion syndrome typically presenting with intellectual disability, a unique personality, a characteristic facial appearance, and cardiovascular disease. Several clinical features of WBS are thought to be due to haploinsufficiency of elastin (ELN), as the ELN locus is included within the WBS critical region at 7q11.23. Emphysema, a disease attributed to destruction of pulmonary elastic fibers, has been reported in patients without WBS who have pathogenic variants in ELN but only once (in one patient) in WBS. Here we report a second adult WBS patient with emphysema where the diagnosis of WBS was established subsequent to the discovery of severe bullous emphysema. Haploinsufficiency of ELN likely contributed to this pulmonary manifestation of WBS. This case emphasizes the contribution of rare genetic variation in cases of severe emphysema and provides further evidence that emphysema should be considered in patients with WBS who have respiratory symptoms, as it may be under-recognized in this patient population. © 2017 Wiley Periodicals, Inc.

  19. The use of typing methods and infection prevention measures to control a bullous impetigo outbreak on a neonatal ward

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    Koningstein Maike

    2012-11-01

    Full Text Available Abstract Background We describe an outbreak of Bullous Impetigo (BI, caused by a (methicillin susceptible, fusidic acid resistant Staphylococcus aureus (SA strain, spa-type t408, at the neonatal and gynaecology ward of the Jeroen Bosch hospital in the Netherlands, from March-November 2011. Methods We performed an outbreak investigation with revision of the hygienic protocols, MSSA colonization surveillance and environmental sampling for MSSA including detailed typing of SA isolates. Spa typing was performed to discriminate between the SA isolates. In addition, Raman-typing was performed on all t408 isolates. Results Nineteen cases of BI were confirmed by SA positive cultures. A cluster of nine neonates and three health care workers (HCW with SA t408 was detected. These strains were MecA-, PVL-, Exfoliative Toxin (ETA-, ETB+, ETAD-, fusidic acid-resistant and methicillin susceptible. Eight out of nine neonates and two out of three HCW t408 strains yielded a similar Raman type. Positive t408 HCW were treated and infection control procedures were reinforced. These measures stopped the outbreak. Conclusions We conclude that treatment of patients and HCW carrying a predominant SA t408, and re-implementing and emphasising hygienic measures were effective to control the outbreak of SA t408 among neonates.

  20. Cutting edge issues in autoimmune gastritis.

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    Toh, Ban-Hock; Chan, James; Kyaw, Tin; Alderuccio, Frank

    2012-06-01

    Autoimmune gastritis is the outcome of a pathological CD4 T cell-mediated autoimmune response directed against the gastric H/K-ATPase. Silent initially, the gastric lesion becomes manifest in humans by the development of megaloblastic pernicious anemia arising from vitamin B12 deficiency. Cutting edge issues in this disease relate to its epidemiology, immunogenetics, a role for Helicobacter pylori as an infective trigger through molecular mimicry, its immunopathogenesis, associated organ-specific autoimmune diseases, laboratory diagnosis, and approaches to curative therapy.