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Sample records for subepidermal blistering disease

  1. AUTOIMMUNE BASEMENT MEMBRANE AND SUBEPIDERMAL BLISTERING DISEASES

    OpenAIRE

    Ana Maria Abreu Velez; Daniel Alberto Vasquez-Hincapie; Howard, Michael S.

    2013-01-01

    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, ...

  2. AUTOIMMUNE BASEMENT MEMBRANE AND SUBEPIDERMAL BLISTERING DISEASES

    Directory of Open Access Journals (Sweden)

    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

  3. Association between the subepidermal autoimmune blistering diseases linear IgA disease and the pemphigoid group and inflammatory bowel disease: two case reports and literature review.

    Science.gov (United States)

    Shipman, A R; Reddy, H; Wojnarowska, F

    2012-07-01

    We report two patients with subepidermal autoimmune blistering diseases and inflammatory bowel disease (IBD) [one with linear IgA disease (LAD) and ulcerative colitis (UC), and the other with mucous membrane pemphigoid (MMP) and Crohn disease (CD)], and present a review of all previously reported cases. We reviewed the literature, and found 48 cases of patients with autoimmune blistering diseases and IBD. The blistering diseases were LAD (25 patients), bullous pemphigoid (BP) (21), MMP (1) and pemphigoid gestationis (1), while the IBD types comprised UC (40) and CD (8). We describe the clinical and immunopathological features and demographic characteristics of the patients. In all but one case, the diagnosis of IBD predated the development of the skin condition. The association was more common with LAD than BP. The immunopathogenesis of IBD and autoimmune blistering diseases is discussed and a link between them hypothesized, namely, that the presentation of multiple antigens to the immune system during the unregulated inflammation in the bowel wall results in excitation of the immune system and recognition of autologous antigens. © The Author(s). CED © 2012 British Association of Dermatologists.

  4. Steroid-sparing effect of mycophenolate mofetil in the treatment of a subepidermal blistering autoimmune disease in a dog : clinical communication

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    P.J. Ginel

    2010-05-01

    Full Text Available A 7-year-old female Cocker spaniel-cross was referred with an 8-month history of mucocutaneous erosive dermatitis. On physical examination, skin lesions affected the eyelids and periocular area, lips and vulva. Lesions were symmetrical with small diffuse superficial ulcers, haemorrhagic crusts, adherent purulent exudation in haired skin, and alopecia with hyperpigmentation and scarring. Histopathologic evaluation showed multiple, non-intact dermoepidermal junction vesicles and ulceration associated with a dermal lichenoid infiltrate. Immunohistochemistry showed strong to moderate reactivity in the dermoepidermal junction for the antibodies directed against canine IgG, human IgG lambda light chains and C3, respectively. A diagnosis of autoimmune subepidermal blistering dermatosis was made. Treatment with oral prednisone at 2 mg/kg and mycophenolate mofetil (MMF at 20 mg/kg twice daily was initiated and after 4 weeks the ulcers and erosions were cured. During the rest of treatment, MMF was maintained at 10 mg/kg twice daily and prednisone could be tapered to 0.25 mg/kg once every other day without recurrences. In conclusion, this case report shows that MMF was well tolerated and might be effective as steroid-sparing agent in the long-term treatment of this autoimmune subepidermal blistering disease.

  5. Subepidermal blistering induced by human autoantibodies to BP180 requires innate immune players in a humanized bullous pemphigoid mouse model.

    Science.gov (United States)

    Liu, Zhi; Sui, Wen; Zhao, Minglang; Li, Zhuowei; Li, Ning; Thresher, Randy; Giudice, George J; Fairley, Janet A; Sitaru, Cassian; Zillikens, Detlef; Ning, Gang; Marinkovich, M Peter; Diaz, Luis A

    2008-12-01

    Bullous pemphigoid (BP) is a cutaneous autoimmune inflammatory disease associated with subepidermal blistering and autoantibodies against BP180, a transmembrane collagen and major component of the hemidesmosome. Numerous inflammatory cells infiltrate the upper dermis in BP. IgG autoantibodies in BP fix complement and target multiple BP180 epitopes that are highly clustered within a non-collagen linker domain, termed NC16A. Anti-BP180 antibodies induce BP in mice. In this study, we generated a humanized mouse strain, in which the murine BP180NC14A is replaced with the homologous human BP180NC16A epitope cluster region. We show that the humanized NC16A (NC16A+/+) mice injected with anti-BP180NC16A autoantibodies develop BP-like subepidermal blisters. The F(ab')(2) fragments of pathogenic IgG fail to activate the complement cascade and are no longer pathogenic. The NC16A+/+ mice pretreated with mast cell activation blocker or depleted of complement or neutrophils become resistant to BP. These findings suggest that the humoral response in BP critically depends on innate immune system players.

  6. Crescentic glomerulonephritis and subepidermal blisters with autoantibodies to alpha5 and alpha6 chains of type IV collagen.

    Science.gov (United States)

    Ghohestani, Reza F; Rotunda, Sherry L; Hudson, Billy; Gaughan, William J; Farber, John L; Webster, Guy; Uitto, Jouni

    2003-05-01

    We describe a novel autoimmune disease characterized by severe subepidermal bullous eruption and crescentic glomerulonephritis with autoantibodies directed against the noncollagenous domain of the alpha5 and alpha6 chains of type IV collagen. Biopsy of perilesional skin revealed a subepidermal blister with marked polymorphonuclear infiltrate with linear deposits of IgA and C3. Light microscopy of a kidney biopsy specimen revealed a crescentic glomerulonephritis, and immunofluorescence microscopy showed linear basement membrane staining for IgA (3+), C3 (1+), and IgG (1+). No electron-dense deposits were observed by transmission electron microscopy. The patient's autoantibodies reacted with normal human skin and kidney: IgA (3+) and IgG (1+) antibodies stained the basement membrane zones of skin, renal glomerulus, and some tubules. The identity of the target antigen was determined by immunochemical analyses of candidate antigens using the patient's autoantibodies. The patient's IgA and IgG autoantibodies reacted with a 185- to 190-kDa antigen from a human dermal extract that was distinguished from the other dermal or epidermal antigens, including the 145- to 290-kDa (type VII collagen) epidermolysis bullosa acquisita antigen, the 165- to 200-kDa alpha3 laminin mucous membrane cicatricial pemphigoid antigen, and the 230-kDa and the 180-kDa bullous pemphigoid antigens. Patient's IgA and IgG autoantibodies further reacted with the alpha5(IV) and weakly with the alpha6(IV) chains of type IV collagen by Western blot and ELISA. This report expands the repertoire of bullous skin disorders and provides an explanation for the association of anti-type IV collagen autoantibodies and glomerulonephritis with subepidermal blisters.

  7. Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management.

    Science.gov (United States)

    Amber, Kyle T; Murrell, Dedee F; Schmidt, Enno; Joly, Pascal; Borradori, Luca

    2017-08-04

    Autoimmune subepidermal blistering diseases of the skin and mucosae constitute a large group of sometimes devastating diseases, encompassing bullous pemphigoid, gestational pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. Their clinical presentation is polymorphic. These autoimmune blistering diseases are associated with autoantibodies that target distinct components of the basement membrane zone of stratified epithelia. These autoantigens represent structural proteins important for maintenance of dermo-epidermal integrity. Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease of the skin and mucosae. Although the disease typically presents with a generalized blistering eruption associated with itch, atypical variants with either localized bullous lesions or "non-bullous" presentations are observed in approximately 20% of patients. A peculiar form of BP typically associated with pregnancy is pemphigoid gestationis. In anti-p200 pemphigoid, patients present with tense blisters on erythematosus or normal skin resembling BP, with a predilection for acral surfaces. These patients have antibodies targeting the 200-kDa basement membrane protein. Epidermolysis bullosa is a rare autoimmune blistering disease associated with autoantibodies against type VII collagen that can have several phenotypes including a classical form mimicking dystrophic epidermolysis bullosa, an inflammatory presentation mimicking BP, or mucous membrane pemphigoid-like lesions. Mucous membrane pemphigoid (MMP) is the term agreed upon by international consensus for an autoimmune blistering disorder, which affects one or more mucous membrane and may involve the skin. The condition involves a number of different autoantigens in the basement membrane zone. It may result in severe complications from scarring, such as blindness and strictures. Diagnosis of these diseases relies on direct immunofluorescence microscopy studies

  8. AUTOIMMUNE EPIDERMAL BLISTERING DISEASES

    OpenAIRE

    Ana Maria Abreu Velez; Juliana Calle; Howard, Michael S.

    2013-01-01

    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 Er...

  9. [Autoimmune blistering diseases].

    Science.gov (United States)

    Duvert-Lehembre, S; Joly, P

    2014-03-01

    Autoimmune blistering diseases are characterized by the production of pathogenic autoantibodies that are responsible for the formation of epidermal blisters. Major advances in the understanding of the pathogenesis of these disorders have allowed the development of new therapeutic agents. Recent epidemiologic data showed that bullous pemphigoid mainly affects elderly patients. Bullous pemphigoid is often associated with degenerative neurologic disorders. A major increase in the incidence of bullous pemphigoid has been observed in France. Treatment of bullous pemphigoid is mainly based on superpotent topical corticosteroids. The role of desmosomal proteins has been demonstrated in the initiation, propagation and persistence of the autoimmune response in pemphigus. Several studies have shown a correlation between anti-desmoglein antibody titers and disease activity. Pemphigus susceptibility genes have been identified. Oral corticosteroids remain the mainstay of pemphigus treatment. Dramatic and long-lasting improvement has been recently obtained with rituximab in recalcitrant types of pemphigus. Other autoimmune junctional blistering diseases are rare entities, whose prognosis can be severe. Their diagnosis has been improved by the use of new immunological assays and immunoelectronic microscopy. Immunosupressants are widely used in severe types in order to prevent mucosal sequelae. Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  10. AUTOIMMUNE EPIDERMAL BLISTERING DISEASES

    Directory of Open Access Journals (Sweden)

    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

  11. A subepidermal blistering disorder

    African Journals Online (AJOL)

    Y Moolla. Dr Yusuf Moolla is a specialist physician in the Department of Internal Medicine at Addington Hospital, Durban, South Africa. He recently obtained an MMed from the University of KwaZulu-Natal for his work on HIV infection. Corresponding author: Y Moolla (moollayusuf@hotmail.com). A young woman presented ...

  12. Epidermolysis bullosa acquisita in a 17-year-old boy with Crohn's disease

    National Research Council Canada - National Science Library

    Russo, Irene; Ferrazzi, Anna; Zanetti, Irene; Alaibac, Mauro

    2015-01-01

    Epidermolysis bullosa acquisita is a rare, acquired, autoimmune subepidermal blistering disease of the skin, characterised by blisters and erosions, especially in trauma-prone sites and extensor skin...

  13. The use of skin substrates deficient in basement membrane molecules for the diagnosis of subepidermal autoimmune bullous disease

    NARCIS (Netherlands)

    Vodegel, RM; Kiss, M; De Jong, MCJM; Pas, HH; Altmayer, A; Molnar, K; Husz, S; Van der Meer, JB; Jonkman, MF

    A case is presented of subepidermal, autoimmune bullous disease in which the initial examinations suggested the combination of epidermolysis bullosa acquisita and bullous pemphigoid. The diagnosis of epidermolysis bullosa acquisita was made by indirect immunofluorescence microscopy: the patient's

  14. Passive transfer of collagen XVII-specific antibodies induces sustained blistering disease in adult mice

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    Chiriac Mircea Teodor

    2013-01-01

    Full Text Available Abstract Background Bullous pemphigoid is a subepidermal blistering disorder associated with tissue-bound and circulating autoantibodies directed mainly to the hemidesmosomal component collagen XVII. While recapitulating the main immunopathological features of the human disease, frank skin blistering does not develop in the absence of skin rubbing in experimental pemphigoid models that have been established in neonatal mice. Moreover, due to their experimental design they only allow for short-term disease observation. In the present study we aimed to establish a model that reproduces the frank skin blistering seen in patients and allows for longer observation times. Methods Rabbit and sheep antibodies specific to several fragments of collagen XVII were generated and the purified antibodies were passively transferred into adult mice. Results Collagen XVII-specific IgG bound to the basal membrane of the skin and mucous membranes activating murine complement in vivo. Mice injected with collagen XVII-specific antibodies, in contrast to mice receiving control antibodies, developed frank skin blistering disease, reproducing human bullous pemphigoid at the clinical, histological and immunopathological levels. Titres of circulating IgG in the serum of mice correlated with the extent of the clinical disease. Mice receiving sheep antibodies specific to murine collagen XVII showed an early onset and a more active disease when compared to litter mates receiving specific rabbit antibodies. Conclusion This novel animal model for bullous pemphigoid should facilitate further investigations of the pathogenesis of bullous pemphigoid and the development of innovative therapies for this disease.

  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. Reduced Skin Blistering in Experimental Epidermolysis Bullosa Acquisita After Anti-TNF Treatment

    NARCIS (Netherlands)

    Hirose, Misa; Kasprick, Anika; Beltsiou, Foteini; Dieckhoff, Katharina Schulze; Schulze, Franziska Sophie; Samavedam, Unni K. J. S. R. L.; Hundt, Jennifer E.; Pas, Hendri H.; Jonkman, Marcel F.; Schmidt, Enno; Kalies, Kathrin; Zillikens, Detlef; Ludwig, Ralf J.; Bieber, Katja

    2016-01-01

    Epidermolysis bullosa acquisita (EBA) is a difficult-to-treat subepidermal autoimmune blistering skin disease (AIBD) with circulating and tissue-bound anti-type VII collagen antibodies. Different reports have indicated increased concentration of tumor necrosis factor a (TNF) in the serum and blister

  17. Salt split technique: A useful tool in the diagnosis of subepidermal bullous disorders

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    De Abhishek

    2010-01-01

    Full Text Available Background: Direct immunofluorescence (DIF is the gold standard in the diagnosis of immunobullous diseases. However, it cannot reliably differentiate various subtypes of subepidermal immune- bullous diseases (SIBD. Salt split technique (SST could be used under such circumstances to differentiate them. There is paucity of reports in the Indian literature regarding the SST. Aim: This study was designed to evaluate the utility of direct SST in subepidermal blistering diseases. Materials and Methods: Fourteen clinically diagnosed cases of subepidermal blistering diseases were included in the study. Two perilesional punch biopsies were taken one each for DIF and salt split study. Results: Linear basement membrane zone band with IgG and/or C 3 was seen in 14 cases of patients BP. Salt split study showed epidermal or mixed pattern of deposits in 12 patients and exclusive floor pattern in two patients. The diagnosis was revised in these two patients to epidermolysis bullosa acquisita. Conclusion: SST is a simple, inexpensive procedure and should be routinely employed in the diagnosis of subepidermal bullous diseases.

  18. Psychosocial impact of inherited and autoimmune blistering diseases

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    Swaranjali V. Jain, B Med Sci (Hons MD

    2018-03-01

    Full Text Available Inherited and autoimmune blistering diseases are rare, chronic, and often severe disorders that have the potential to significantly affect patients’ quality of life. The effective management of these conditions requires consideration of the physical, emotional, and social aspects of the disease. Self-esteem is integral to patients’ ability to cope with their illness, participate in treatment, and function in society. This article discusses quality-of-life studies of patients with blistering diseases with a particular focus on self-esteem issues that patients may face.

  19. Coma blisters in two postoperative patients.

    Science.gov (United States)

    Chacon, Anna H; Farooq, Uzma; Choudhary, Sonal; Yin, Natalie; Nolan, Bridgit; Shiman, Michael; Milikowski, Clara; Izakovic, Jan; Elgart, George W

    2013-05-01

    Coma blisters are self-limited cutaneous bullae that occur in the setting of loss of consciousness because of a drug, illness, or accident, with the most common settings being barbiturate overdose and neurological disorders. The etiology behind coma blisters is poorly understood and is not related to underlying infections or autoimmune conditions. The clinical presentation consists of bullae, erosions, and violaceous plaques usually involving sites of pressure. The skin lesions usually occur within 48-72 hours of the start of a coma and resolve within 2-4 weeks. We present one case of a 5-month-old infant with severe valvular disease who required surgical repair. He was placed on extra corporeal membrane oxygenation and developed multiple tense coma blisters during the course of therapy. Skin biopsy revealed a noninflammatory subepidermal blister with necrosis of the overlying epidermis and necrosis of the eccrine ducts. We also present a second case of an 18-year-old female patient who underwent surgical resection of a benign mandibular tumor. She subsequently developed bullae on both arms 4 days after surgery. The skin biopsy showed a necrotic epidermis, a subepidermal blister, and diffuse necrosis of the eccrine coils.

  20. Paraneoplastic Pemphigus and Autoimmune Blistering Diseases Associated with Neoplasm: Characteristics, Diagnosis, Associated Neoplasms, Proposed Pathogenesis, Treatment.

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    Kartan, Saritha; Shi, Vivian Y; Clark, Ashley K; Chan, Lawrence S

    2017-02-01

    Autoimmune paraneoplastic and neoplasm-associated skin syndromes are characterized by autoimmune-mediated cutaneous lesions in the presence of a neoplasm. The identification of these syndromes provides information about the underlying tumor, systemic symptoms, and debilitating complications. The recognition of these syndromes is particularly helpful in cases of skin lesions presenting as the first sign of the malignancy, and the underlying malignancy can be treated in a timely manner. Autoimmune paraneoplastic and neoplasm-associated bullous skin syndromes are characterized by blister formation due to an autoimmune response to components of the epidermis or basement membrane in the context of a neoplasm. The clinical manifestations, histopathology and immunopathology findings, target antigens, associated neoplasm, current diagnostic criteria, current understanding of pathogenesis, and treatment options for a selection of four diseases are reviewed. Paraneoplastic pemphigus manifests with clinically distinct painful mucosal erosions and polymorphic cutaneous lesions, and is often associated with lymphoproliferative neoplasm. In contrast, bullous pemphigoid associated with neoplasm presents with large tense subepidermal bullae of the skin, and mild mucosal involvement, but without unique clinical features. Mucous membrane pemphigoid associated with neoplasm is a disorder of chronic subepithelial blisters that evolve into erosions and ulcerations that heal with scarring, and involves stratified squamous mucosal surfaces. Linear IgA dermatosis associated with neoplasm is characterized by annularly grouped pruritic papules, vesicles, and bullae along the extensor surfaces of elbows, knees, and buttocks. Physicians should be aware that these autoimmune paraneoplastic and neoplasm-associated syndromes can manifest distinct or similar clinical features as compared with the non-neoplastic counterparts.

  1. Paraneoplastic Pemphigus. A Life-Threatening Autoimmune Blistering Disease.

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    Tirado-Sánchez, A; Bonifaz, A

    2017-08-08

    Paraneoplastic pemphigus (PNP), a subset of pemphigus, is a unique autoimmune blistering condition that can affect multiple organs other than the skin. It is a life-threatening disease associated with an underlying malignancy, most commonly of lymphoproliferative origin. The clinical picture may resemble pemphigus, pemphigoid, erythema multiforme, graft-versus-host disease, or lichen planus. The earliest and most consistent finding is a painful, severe, chronic and often recalcitrant stomatitis. Treatment of PNP is difficult. Immunosuppressive agents are required to decrease blistering, and treating the underlying tumor may control autoantibody production. In this review, we included essential diagnostic aspects of PNP and the most useful treatment options in the dermatologist practice. Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

  2. Modern diagnosis of autoimmune blistering skin diseases.

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    Schmidt, Enno; Zillikens, Detlef

    2010-12-01

    The diagnostic gold standard of autoimmune bullous diseases is the detection of autoantibodies in skin or mucous membranes by direct immunofluorescence microscopy of a perilesional biopsy. The molecular characterisation of several target antigens within the last 10 years has, however, fostered the development of sensitive and specific diagnostic tools that allow the serological diagnosis in about 90% of patients. Based on the recombinant immunodominant portions of the target antigens, ELISA systems are commercially available for the detection of circulating antibodies against desmoglein 1, desmoglein 3, envoplakin, BP180, and BP230. Autoantibodies against the soluble ectodomain of BP180 (LAD-1), laminin 332, type VII collagen, and most recently, laminin γ1 can be detected by Western blotting with recombinant or cell-derived forms of these proteins. The definite differentiation between the various immunobullous disorders that comprise about a dozen entities is increasingly important since more diverse treatment options are employed. Exact diagnosis is also pivotal for the prognosis, since some autoimmune bullous diseases may indicate an underlying tumor. Association with a malignancy has been shown in paraneoplastic pemphigus (in 100%) and anti-laminin 332 mucous pemphigoid (in 25%) In pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, autoantibodies to desmoglein 3, desmoglein 1, and BP180, respectively, have been shown to correlate with the disease activity. The detection of serum autoantibodies during the course of the disease may thus be helpful in guiding treatment decisions in these patients. Copyright © 2010 Elsevier B.V. All rights reserved.

  3. Early Description of Diet-Induced Blistering Skin Diseases in Medieval Persia: Avicenna's Point of View.

    Science.gov (United States)

    Atarzadeh, Fatemeh; Daneshfard, Babak; Dastgheib, Ladan; Jaladat, Amir-Mohammad; Amin, Gholamreza

    2016-01-01

    Pemphigus is an autoimmune blistering skin disease that is strongly associated with different environmental factors. Among these, nutritional factors are considered to trigger pemphigus; however, their role may be underestimated. Investigated more recently in conventional medicine, this causative bond between dietary factors and blistering skin diseases was mentioned by Persian scholars such as Avicenna a thousand years ago. Avicenna, a well-known Persian physician and philosopher, who could be considered a pioneer in dermatology, discussed skin diseases in a chapter in The Canon of Medicine. He accounted for some nutritional triggers for skin blisters (mentioned as "hot swellings"), such as onion, garlic, leek, pepper, and wine. His precise description of causative factors based on principles of traditional Persian medicine (TPM) is appreciable and might well lead us to find more efficient ways for the prevention and treatment of blistering skin diseases.

  4. Blistering mucocutaneous diseases of the oral mucosa--a review: part 1. Mucous membrane pemphigoid.

    Science.gov (United States)

    Darling, Mark R; Daley, Tom

    2005-12-01

    Oral mucous membranes may be affected by a variety of blistering mucocutaneous diseases. In this paper, we review the clinical manifestations, typical microscopic and immunofluorescence features, pathogenesis, biological behaviour and treatment of mucous membrane pemphigoid (MMP). As MMP is a relatively common condition, the general dentist must be able to diagnose, treat and monitor the progress of their affected patients or refer when appropriate.

  5. Chronic bullous disease of childhood and a paecilomyces lung infection in chronic granulomatous disease

    NARCIS (Netherlands)

    Sillevis Smitt, J. H.; Leusen, J. H.; Stas, H. G.; Teeuw, A. H.; Weening, R. S.

    1997-01-01

    A 12 year old boy suffering from p67-phox deficient chronic granulomatous disease presented with a bullous skin disease and a lung infection with paecilomyces species. The histopathology of a bullous lesion showed subepidermal blister formation and microabcesses containing eosinophils in the dermal

  6. Suction blistering the lesional skin of vitiligo patients reveals useful biomarkers of disease activity.

    Science.gov (United States)

    Strassner, James P; Rashighi, Mehdi; Ahmed Refat, Maggi; Richmond, Jillian M; Harris, John E

    2017-05-01

    Vitiligo is an autoimmune disease of the skin with limited treatment options; there is an urgent need to identify and validate biomarkers of disease activity to support vitiligo clinical studies. To investigate potential biomarkers of disease activity directly in the skin of vitiligo subjects and healthy subjects. Patient skin was sampled via a modified suction-blister technique, allowing for minimally invasive, objective assessment of cytokines and T-cell infiltrates in the interstitial skin fluid. Potential biomarkers were first defined and later validated in separate study groups. In screening and validation, CD8+ T-cell number and C-X-C motif chemokine ligand (CXCL) 9 protein concentration were significantly elevated in active lesional compared to nonlesional skin. CXCL9 protein concentration achieved greater sensitivity and specificity by receiver operating characteristic analysis. Suction blistering also allowed for phenotyping of the T-cell infiltrate, which overwhelmingly expresses C-X-C motif chemokine receptor 3. A small number of patients were enrolled for the study, and only a single patient was used to define the treatment response. Measuring CXCL9 directly in the skin might be effective in clinical trials as an early marker of treatment response. Additionally, use of the modified suction-blister technique supports investigation of inflammatory skin diseases using powerful tools like flow cytometry and protein quantification. Copyright © 2016 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  7. Fracture Blisters

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    Uebbing, Claire M

    2011-02-01

    Full Text Available Fracture blisters are a relatively uncommon complication of fractures in locations of the body, such as the ankle, wrist elbow and foot, where skin adheres tightly to bone with little subcutaneous fat cushioning. The blister that results resembles that of a second degree burn.These blisters significantly alter treatment, making it difficult to splint or cast and often overlying ideal surgical incision sites. Review of the literature reveals no consensus on management; however, most authors agree on early treatment prior to blister formation or delay until blister resolution before attempting surgical correction or stabilization. [West J Emerg Med. 2011;12(1;131-133.

  8. Epidermolysis bullosa acquisita in a 17-year-old boy with Crohn's disease

    OpenAIRE

    Russo, Irene; Ferrazzi, Anna; Zanetti, Irene; Alaibac, Mauro

    2015-01-01

    Epidermolysis bullosa acquisita is a rare, acquired, autoimmune subepidermal blistering disease of the skin, characterised by blisters and erosions, especially in trauma-prone sites and extensor skin surface, scarring with formation of milia, skin fragility and nail dystrophy. Epidermolysis bullosa acquisita is extremely rare in childhood and it has been reported to be frequently associated with Crohn's disease. Furthermore, autoantibodies against type VII collagen have been found in a large ...

  9. Chitosan-induced immunity in Camellia sinensis (L.) O. Kuntze against blister blight disease is mediated by nitric-oxide.

    Science.gov (United States)

    Chandra, Swarnendu; Chakraborty, Nilanjan; Panda, Koustubh; Acharya, Krishnendu

    2017-06-01

    Blister blight disease, caused by an obligate biotrophic fungal pathogen, Exobasidium vexans Massee is posing a serious threat for tea cultivation in Asia. As the use of chemical pesticides on tea leaves substantially increases the toxic risks of tea consumption, serious attempts are being made to control such pathogens by boosting the intrinsic natural defense responses against invading pathogens in tea plants. In this study, the nature and durability of resistance offered by chitosan and the possible mechanism of chitosan-induced defense induction in Camellia sinensis (L.) O. Kuntze plants against blister blight disease were investigated. Foliar application of 0.01% chitosan solution at 15 days interval not only reduced the blister blight incidence for two seasons, but also maintained the induced expressions of different defense related enzymes and total phenol content compared to the control. Defense responses induced by chitosan were found to be down regulated under nitric oxide (NO) deficient conditions in vivo, indicating that the observed chitosan-induced resistance is probably activated via NO signaling. Such role of NO in host defense response was further established by application of the NO donor, sodium nitroprusside (SNP), which produced similar defense responses accomplished through chitosan treatment. Taken together, our results suggest that increased production of NO in chitosan-treated tea plants may play a critical role in triggering the innate defense responses effective against plant pathogens, including that causing the blister blight disease. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  10. [Application of blistering cupping].

    Science.gov (United States)

    Gu, Xingui; Chen, Zelin; Chen, Bo; Fan, Yihua; Chen, Xianghong

    2016-11-12

    Blistering cupping is special as eliminating wind and dampness as well as removing phlegm and blood stasis, and it achieves effects through suction. In this paper we reviewed relevant literature combined with clinical experience so as to summarize its operation attention through exploring the origin, mechanism and application. We divide the progress into the blistering period, the phlegm-stasis-eliminating period, and the escharosis period according to the changes of bubble and the things pulled out. Blistering cupping creates ways to eliminate concrete unhealthy influences through smoothing meridians and collaterals, such as phlegm and retained fluid, dampness and blood stasis. Thus chronic diseases are relieved. Also,we propose the rules of "blistering acupoints being related to disease location as well as the nature of acupoints and diseases". The therapy has been used to treat diseases of respiratory system, osteoarticular, skin and subcutaneous tissue, mental and behavioral disorders, and tumor, among which the effects of intractable diseases of respiratory and osteoarticular systems are definite. It deserves to be further explored and promoted.

  11. Prothrombotic state and impaired fibrinolysis in bullous pemphigoid, the most frequent autoimmune blistering disease.

    Science.gov (United States)

    Marzano, A V; Tedeschi, A; Polloni, I; Crosti, C; Cugno, M

    2013-01-01

    Bullous pemphigoid (BP) is a potentially life-threatening autoimmune blistering disease that is burdened with an increased risk of cardiovascular events. In BP, there is an interplay between inflammation and coagulation both locally, which contributes to skin damage, and systemically, which leads to a prothrombotic state. Fibrinolysis is an important defence mechanism against thrombosis, but has only been studied locally in BP and no systemic data are available. The aim of this observational study was to evaluate systemic fibrinolysis and coagulation activation in patients with BP. We measured parameters of fibrinolysis and coagulation by immunoenzymatic methods in plasma from 20 patients with BP in an active phase and during remission after corticosteroid treatment. The controls were 20 age- and sex-matched healthy subjects. Plasma levels of plasminogen activator inhibitor type 1 (PAI-1) antigen, PAI-1 activity and tissue plasminogen activator (t-PA) antigen were significantly higher in the BP patients with active disease than in healthy controls (P = 0·0001 for all), as were the plasma levels of the fibrin fragment d-dimer and prothrombin fragment F1+2 (P = 0·0001 for both). During remission after treatment, levels of PAI-1 antigen and PAI-1 activity decreased significantly (P = 0·008 and P = 0·006, respectively), and there was also a significant decrease in plasma levels of d-dimer (P = 0·0001) and F1+2 (P = 0·0001). Fibrinolysis is inhibited in patients with active BP, due mainly to an increase in plasma levels of PAI-1. Corticosteroids not only induce the regression of BP lesions, but also reduce the inhibition of fibrinolysis, which may contribute to decreasing thrombotic risk. © 2012 British Society for Immunology.

  12. CYCLO-OXYGENASE 2 IS PRESENT IN THE MAJORITY OF LESIONAL SKIN FROM PATIENTS WITH AUTOINMUNE BLISTERING DISEASES

    Directory of Open Access Journals (Sweden)

    Ana Maria Abreu Velez

    2013-10-01

    Full Text Available Introduction: The in situ immune response within skin biopsies from patients affected by autoimmune skin blistering diseases (ABDs is not well characterized. Aim: Based on the fact that the ABD immune response is considered an adaptive immune response, both an innate immune response and inflammation would be expected in these diseases. Our investigation investigates the presence of cyclo-oxygenase-2 (COX-2, since this enzyme is commonly involved in innate immune responses. Methods: We utilized immunohistochemistry (IHC to evaluate the presence of COX-2 in lesional skin biopsies of patients affected by ABDs. We tested 30 patients with endemic pemphigus foliaceus (EPF, 15 controls from the endemic area, and 15 biopsies from healthy controls from the USA. We also tested archival biopsies from patients with selected ABDs, including 20 patients with bullous pemphigoid, 20 with pemphigus vulgaris, 8 with pemphigus foliaceus and 12 with dermatitis herpetiformis. Results: Most ABD biopsies stained positive for COX-2 in the lesional blister and/or the dermal inflammatory infiltrate, accentuated in the upper neurovascular plexus. In BP and EPF, the COX-2 staining was also seen in the sweat glands. All controls were negative. Conclusions: We document that COX-2 is expressed in lesional skin of patients with ABDs.

  13. CD1a, HAM56, CD68 and S-100 are present in lesional skin biopsies from patients affected by autoimmune blistering diseases

    Directory of Open Access Journals (Sweden)

    Ana Maria Abreu Velez

    2014-04-01

    Full Text Available Introduction: Previous research on autoimmune skin blistering diseases (ABD has primarily focused on the humoral immune response; moreover, little attention has been given to the potential role of the antigen presenting cells (APCs in lesional skin. Aim: The purpose of our study was to immunophenotype selected APC in the lesional skin of ABDs, utilizing immunohistochemistry (IHC stains. Materials and Methods: We utilized IHC to stain for dendritic cells (DC, staining with CD1a, CD68, HAM56, and S-100 in lesional skin from 30 patients with endemic pemphigus foliaceus (EPF, 15 controls from the EPF endemic area, and 15 healthy controls from the USA. We also tested archival biopsies from patients with selected ABD, including 30 patients with bullous pemphigoid (BP, 20 with pemphigus vulgaris (PV, 8 with pemphigus foliaceus (PF and 14 with dermatitis herpetiformis (DH and 2 with epidermolysis bullosa acquisita (EBA. Results: Cells stained by CD68, HAM56 and S-100 were present in the majority of the ABD skin biopsies; these cells were located primarily in perivascular infiltrates surrounding dermal vessels subjacent to the blisters. However, these cells were also noted within the blisters, in vessels supplying dermal eccrine glands and ducts, and in areas of dermal endothelial-mesenchymal cell junction-like structures, especially in BP cases. In our CD1a staining, the number and location of positive staining cells varied with each disease, being abundant in most ABD in the epidermis suprajacent to the blisters, or in the epidermis surrounding the blister site if the blister site epidermis was missing. In the control biopsies, most did not display positive IHC staining, with the exception of a few CD1a positive cells in the epidermis Conclusion: Our findings confirm positive IHC staining for APCs in areas of the skin besides the disease blisters. Our findings suggest that the antigen presentation in ABD proceeds in areas distant from the blister site

  14. Decontamination Data - Blister Agents

    Data.gov (United States)

    U.S. Environmental Protection Agency — Decontamination efficacy data for blister agents on various building materials using various decontamination solutions. This dataset is associated with the following...

  15. Origin of the subepidermal tissue in Piper L. leaves.

    Science.gov (United States)

    Nakamura, A T; Simão, E; Silva, L; Torres, G A

    2015-05-01

    Studies on the anatomy of Piper leaves demonstrate the presence of a subepidermal tissue distinct from the adjacent epidermis, which cells show thin walls and hyaline contents. Some authors consider such cells a hypodermal tissue, while others refer to them as components of a multiple epidermis. In this study, the nature of this subepidermal tissue was investigated through the analysis of leaf ontogeny in three Piper species. The analysis showed that the referred tissue originates from the ground meristem and, thus, should be considered a hypodermis. The studied species suggests that the role of the hypodermis would be to protect the photosynthetic apparatus from excess light, regulating the intensity of light reaching the chlorophyll parenchyma.

  16. WHITE BLISTER SPECIES (Albuginaceae ON WEEDS

    Directory of Open Access Journals (Sweden)

    Karolina Vrandečić

    2011-06-01

    Full Text Available The obligate fungi inside the family Albuginaceae are widespread world wide and cause white rust or white blister disease. Mycopopulation of weeds has been researched within the project „The role of weeds in epidemiology of row-crop diseases“. The aim of this research was to identify white blister species occurring on weeds in Eastern Croatia. Weed plants with disease symptoms characteristic for white blister species have been collected since 2001 on location Slavonia and Baranja country. Determination of white blister species was based on morphological characters of pathogen and the host. Wilsoniana bliti was determined on Amaranthus retroflexus and Amaranthus hybridus leaves. Capsella bursa pastoris is a host for Albugo candida. Ambrosia artemisiifolia is a host for Pustula sp. and Cirsium arvense was found to be host for Pustula spinulosa. Wilsoniana portulaceae was determined on Portulaca oleracea.

  17. Blisters: First Aid

    Science.gov (United States)

    ... 5, 2014. Brennan FH. Treatment and prevention of foot friction blisters. ACSM's Health & Fitness Journal. 2013;17:45. Khodaee M, et al. Common ultramarathon injuries and illnesses: Race day management. Current Sports Medicine Reports. 2012;11: ...

  18. Epidermolysis bullosa acquisita in a 17-year-old boy with Crohn's disease.

    Science.gov (United States)

    Russo, Irene; Ferrazzi, Anna; Zanetti, Irene; Alaibac, Mauro

    2015-07-10

    Epidermolysis bullosa acquisita is a rare, acquired, autoimmune subepidermal blistering disease of the skin, characterised by blisters and erosions, especially in trauma-prone sites and extensor skin surface, scarring with formation of milia, skin fragility and nail dystrophy. Epidermolysis bullosa acquisita is extremely rare in childhood and it has been reported to be frequently associated with Crohn's disease. Furthermore, autoantibodies against type VII collagen have been found in a large number of patients with Crohn's disease without epidermolysis bullosa acquisita. We report a case of a 17-year-old boy affected by Crohn's disease who presented with milia on infiltrated erythematous plaques over the back of the hands. The diagnosis of epidermolysis bullosa acquisita was confirmed by histopathology, direct and indirect immunofluorescence analysis and ELISA. 2015 BMJ Publishing Group Ltd.

  19. ROULEAUX AND AUTOAGGLUTINATION OF ERYTHROCYTES ASSOCIATED WITH FIBRIN-LIKE MATERIAL IN SKIN BIOPSIES FORM PATIENTS WITH AUTOIMMUNE BLISTERING DISEASES

    Directory of Open Access Journals (Sweden)

    Ana Maria Abreu Velez

    2013-11-01

    Full Text Available Introduction: Autoimmune bullous skin diseases (ABDs represent a heterogeneous group of disorders of the skin and mucosa; these disorders are commonly associated with deposits of immunoglobulins, complement, and fibrinogen, usually directed against distinct adhesion molecules. Methods: We utilized hematoxylin and eosin (H & E stained tissues sections to evaluate for the presence of rouleaux in lesional skin biopsies of patients affected by ABDs including patients with endemic and nonendemic pemphigus foliaceus, bullous pemphigoid (BP, pemphigus vulgaris (PV, dermatitis herpetiformis (DH, and a group of controls taken from routine biopsies seen in our practice. Results: Most autoimmune bullous skin diseases biopsies showed rouleaux formation within and around post-capillary venules in the superficial vascular plexus in association with a pinkish brush-like material that resembles fibrin or other amorphous eosinophilic material. Discussion: We document that rouleaux and the pinkish aggregates are present in within biopsies taken from lesional skin in the majority of patients with ABDs and speculate that this maybe as result of the exocytosis of inflammatory cells, antibodies that form when exposed to the extracellular matrix which is already edematous in most ABDs. In addition red blood cells in the presence of plasma proteins or other macromolecules may form aggregates. Further studies are needed.

  20. Changes of human skin in subepidermal wound healing process.

    Science.gov (United States)

    Sugata, Keiichi; Kitahara, Takashi; Takema, Yoshinori

    2008-11-01

    The wound healing process involves unexplained mechanisms. An aberration in this process is known to cause dermal disorders such as keloid or hypertrophic scars, but the mechanism by which these scars are formed remains to be elucidated. Here we examined the usefulness of a non-invasive optical imaging device to clarify mechanisms of wound healing and of scar formation. An 8 mm experimental wound was made in the forearms of six subjects by a suction blister method. To observe chronological changes associated with wound healing, horizontal cross-sectional images were non-invasively obtained of the wounded area from the skin surface down to 129 microm below at 21.5 microm intervals using in vivo laser confocal scanning microscopy (LCSM). The wounds were covered with a new epidermis by week 2, at which time the dermal papilla count decreased while the thickness from the skin surface to the apex of the dermal papilla increased. The count and the thickness returned to the initial levels when the wound was healed. In two out of six subjects, fibrous tissues were observed in the upper dermis, whereas in one other subject, melanocyte-like dendritic cells were observed in the epidermis-dermis border in later phases of wound healing. This non-invasive method using in vivo LCSM revealed chronological changes in the dermis and epidermis during wound healing. In addition, although a scar was not formed in any of study subjects, this microscopy revealed aspects similar to the fibrous tissue overgrowth or to melanocyte migration, both of which may relate to wound healing. These results indicate the usefulness of this non-invasive method in studies of wound healing and of scar formation.

  1. Effectiveness and side effects of anti-CD20 therapy for autoantibody-mediated blistering skin diseases: A comprehensive survey of 71 consecutive patients from the Initial use to 2007

    Directory of Open Access Journals (Sweden)

    Jennifer D Peterson

    2008-11-01

    Full Text Available Jennifer D Peterson1, Lawrence S Chan2,3,41Department of Dermatology, Texas Tech University Health Sciences Center at Lubbock, Lubbock, TX, USA; 2Department of Dermatology; 3Department of Microbiology/Immunology, University of Illinois at Chicago, Chicago, IL, USA; 4Medicine Service, Jesse Brown VA Medical Center, Chicago, IL, USAAbstract: In order to examine the efficacy and side effects of the monoclonal antibody anti-CD20 (rituximab on autoimmune blistering skin diseases, we performed a comprehensive survey of 71 consecutive patients from initial use up to 2007, using the PubMed database. A heterogeneous group of patients, including 51 patients with pemphigus vulgaris, one with pemphigus vegetans, nine with pemphigus foliaceus, five with paraneoplastic pemphigus, four with epidermolysis bullosa acquisita, and one with both bullous pemphigoid and graft vs host disease was included in this survey. Overall the monoclonal antibody seems to be effective in that 69% of patients showed complete response, 25% of patients showed partial response, whereas 6% of patients showed progressive disease. Six deaths occurred in association with the treatment, with four of these deaths in patients with paraneoplastic pemphigus, a disease characteristically resistant to conventional medication and with a high mortality rate. Of note, 11 patients who received combined rituximab and intravenous immune globulin treatments had the best outcome: complete response without any serious side effects. Therefore further investigation on rituximab with controlled clinical trial is a worthy pursuit.Keywords: blistering diseases, skin, anti-CD20, pemphigus, epidermolysis bullosa acquisita

  2. White pine blister rust resistance research in Minnesota and Wisconsin

    Science.gov (United States)

    Andrew David; Paul Berrang; Carrie Pike

    2012-01-01

    The exotic fungus Cronartium ribicola causes the disease white pine blister rust on five-needled pines throughout North America. Although the effects of this disease are perhaps better known on pines in the western portion of the continent, the disease has also impacted regeneration and growth of eastern white pine (Pinus strobus L. ...

  3. Non-infectious ulcerating oral mucous membrane diseases.

    Science.gov (United States)

    Altenburg, Andreas; Krahl, Dieter; Zouboulis, Christos C

    2009-03-01

    Non-infectious ulcerative oral mucous membrane diseases are difficult to separate at first glance: they can appear as aphthous, bullous, lichenoid, drug-induced or toxic-irritative reactions. The overall considerations of history, localization of lesions, clinical and histological features, as well as direct and indirect immunofluorescence examination are required for the correct diagnosis. Some disorders start preferably at the oral mucosa, like pemphigus vulgaris and Adamantiades-Behçet disease, while others, such as cicatricial pemphigoid and habitual aphthosis generally are confined to the mucous membranes. This overview summarizes clinical and diagnostic features, differential diagnoses and current therapeutic possibilities of non-infectious inflammatory stomatopathies, which possess a specific position among skin diseases in distinction to infectious or neoplastic oral ulcers. This group of diseases includes aphthous lesions, lichen planus mucosae, lupus erythematosus, disorders with intraepidermal or subepidermal formation of blisters including pemphigus, bullous pemphigoid, erythema multiforme and variants as well as allergic or toxic contact stomatitis.

  4. Biology and pathology of Ribes and their implications for management of white pine blister rust

    Science.gov (United States)

    P. J. Zambino

    2010-01-01

    Ribes (currants and gooseberries) are telial hosts for the introduced and invasive white pine blister rust fungus, Cronartium ribicola. Knowledge of wild and introduced Ribes helps us understand the epidemiology of blister rust on its aecial hosts, white pines, and develop disease control and management strategies. Ribes differ by species in their contribution to...

  5. White pines, Ribes, and blister rust: a review and synthesis

    Science.gov (United States)

    Brian W. Geils; Kim E. Hummer; Richard S. Hunt

    2010-01-01

    For over a century, white pine blister rust (Cronartium ribicola) has linked white pines (Strobus) with currants and gooseberries (Ribes) in a complex and serious disease epidemic in Asia, Europe, and North America. Because of ongoing changes in climate, societal demands for forests and their amenities, and scientific advances in genetics and proteomics, our current...

  6. White pine blister rust in the interior Mountain West

    Science.gov (United States)

    Kelly Burns; Jim Blodgett; Dave Conklin; Brian Geils; Jim Hoffman; Marcus Jackson; William Jacobi; Holly Kearns; Anna Schoettle

    2010-01-01

    White pine blister rust is an exotic, invasive disease of white, stone, and foxtail pines (also referred to as white pines or five-needle pines) in the genus Pinus and subgenus Strobus (Price and others 1998). Cronartium ribicola, the fungus that causes WPBR, requires an alternate host - currants and gooseberries in the genus Ribes and species of Pedicularis...

  7. Meeting Report of the Pathogenesis of Pemphigus and Pemphigoid Meeting in Munich, September 2016

    NARCIS (Netherlands)

    Schmidt, Enno; Spindler, Volker; Eming, Ruediger; Amagai, Masayuki; Antonicelli, Frank; Baines, John F.; Belheouane, Meriem; Bernard, Philippe; Borradori, Luca; Caproni, Marzia; Di Zenzo, Giovanni; Grando, Sergei; Harman, Karen; Jonkman, Marcel F.; Koga, Hiroshi; Ludwig, Ralf J.; Kowalczyk, Andrew P.; Mueller, Eliane J.; Nishie, Wataru; Pas, Hendri; Payne, Aimee S.; Sadik, Christian D.; Seppanen, Allan; Setterfield, Jane; Shimizu, Hiroshi; Sinha, Animesh A.; Sprecher, Eli; Sticherling, Michael; Ujiie, Hideyuki; Zillikens, Detlef; Hertl, Michael; Waschke, Jens

    Autoimmune blistering diseases are a heterogeneous group of about a dozen complex disorders that are characterized by intraepidermal (pemphigus) and subepidermal blistering (pemphigoid diseases and dermatitis herpetiformis). The Pathogenesis of Pemphigus and Pemphigoid Meeting, organized by the

  8. Radiation blistering in metals and alloys

    Energy Technology Data Exchange (ETDEWEB)

    Das, S.K.; Kaminsky, M.

    1975-08-25

    Radiation blistering in solids has been identified as a process leading to damage and erosion of irradiated surfaces. Some of the major parameters governing the blistering process in metals and some metallic alloys are the type of projectile and its energy, total dose, dose rate, target temperature, channeling condition of the projectile, orientation of the irradiated surface plane, and target material and its microstructure. Experimental results and models proposed for blister formation and rupture are reviewed. The blistering phenomenon is important as an erosion process in applications such as fusion reactor technology (plasma-wall interactions) and accelerator technology (erosion of components and targets). A description of methods for the reduction of surface erosion caused by blistering is included.

  9. Effectiveness and side effects of anti-CD20 therapy for autoantibody-mediated blistering skin diseases: A comprehensive survey of 71 consecutive patients from the Initial use to 2007

    Science.gov (United States)

    Peterson, Jennifer D; Chan, Lawrence S

    2009-01-01

    In order to examine the efficacy and side effects of the monoclonal antibody anti-CD20 (rituximab) on autoimmune blistering skin diseases, we performed a comprehensive survey of 71 consecutive patients from initial use up to 2007, using the PubMed database. A heterogeneous group of patients, including 51 patients with pemphigus vulgaris, one with pemphigus vegetans, nine with pemphigus foliaceus, five with paraneoplastic pemphigus, four with epidermolysis bullosa acquisita, and one with both bullous pemphigoid and graft vs host disease was included in this survey. Overall the monoclonal antibody seems to be effective in that 69% of patients showed complete response, 25% of patients showed partial response, whereas 6% of patients showed progressive disease. Six deaths occurred in association with the treatment, with four of these deaths in patients with paraneoplastic pemphigus, a disease characteristically resistant to conventional medication and with a high mortality rate. Of note, 11 patients who received combined rituximab and intravenous immune globulin treatments had the best outcome: complete response without any serious side effects. Therefore further investigation on rituximab with controlled clinical trial is a worthy pursuit. PMID:19436603

  10. [Ruptured cerebral artery blister aneurysm].

    Science.gov (United States)

    Vega Valdés, Pedro; Murias Quintana, Eduardo; Meilán Martínez, Angela; Gutiérrez Morales, Julio; Lopez Garcia, Antonio

    2013-01-01

    We report the case of a young patient with subarachnoid haemorrhage secondary to a ruptured blister-like aneurysm. Since this kind of aneurysms have fragile walls without a well-defined neck, their treatment is difficult. We initially planned the deployment of a flow-diverter stent, but an angiogram obtained after 10 days revealed a morphological change of the aneurysm. Therefore, we finally deployed a conventional stent and introduced 2 micro coils into the point of rupture, obtaining a good morphological result without rebleeding. Follow-up at 1 and 6 months did not observe regrowth of the aneurysm. We offer a brief introduction and discussion of this pathology and its treatment. Copyright © 2010 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

  11. The shape of telephone cord blisters

    Science.gov (United States)

    Ni, Yong; Yu, Senjiang; Jiang, Hongyuan; He, Linghui

    2017-01-01

    Formation of telephone cord blisters as a result of buckling delamination is widely observed in many compressed film-substrate systems. Here we report a universal morphological feature of such blisters characterized by their sequential sectional profiles exhibiting a butterfly shape using atomic force microscopy. Two kinds of buckle morphologies, light and heavy telephone cord blisters, are observed and differentiated by measurable geometrical parameters. Based on the Föppl-von Kármán plate theory, the observed three-dimensional features of the telephone cord blister are predicted by the proposed approximate analytical model and simulation. The latter further replicates growth and coalescence of the telephone cord into complex buckling delamination patterns observed in the experiment.

  12. How To Prevent and Treat Blisters

    Science.gov (United States)

    ... occur anywhere on the body where body parts rub together or rub against clothing. Fortunately, blisters can be prevented by ... areas: This helps reduce friction when your skin rubs together or rubs against clothing. Stop your activity ...

  13. Accuracy of ultrasound, thermography and subepidermal moisture in predicting pressure ulcers: a systematic review.

    Science.gov (United States)

    Oliveira, A L; Moore, Z; O Connor, T; Patton, D

    2017-05-02

    Our aims were to: establish the clinical significance of ultrasound, thermography, photography and subepidermal moisture (SEM) measurement; determine the accuracy of ultrasound, thermography, photography and SEM measurement in detecting skin/tissue damage; determine the relative accuracy of one of these assessment methods over another; make recommendations for practice pertaining to assessment of early skin/tissue damage. The following databases, Cochrane Wounds Group Specialised Register, The Cochrane Central Register of Controlled Trials, Ovid MEDLINE, Ovid EMBASE, Elsevier version, EBSCO CINAHL, ClinicalTrials.gov , WHO International Clinical Trials Registry (ICTR) and The EU Clinical Trials Register were searched for terms including; thermography, ultrasound, subepidermal moisture, photograph and pressure ulcer. We identified four SEM, one thermography and five ultrasound studies for inclusion in this review. Data analysis indicated that photography was not a method which allowed for the early prediction of PU presence. SEM values increased with increasing tissue damage, with the sacrum and the heels being the most common anatomical locations for the development of erythema and stage I PUs. Thermography identified temperature changes in tissues and skin that may give an indication of early PU development; however the data were not sufficiently robust. Ultrasound detected pockets of fluid/oedema at different levels of the skin that were comparable with tissue damage. Thus, SEM and ultrasound were the best methods for allowing a more accurate assessment of early skin/tissue damage. Using the EBL Critical Appraisal Tool the overall validities of the studies varied between 33.3-55.6%, meaning that there is potential for bias within all the included studies. All of the studies were situated at level IV, V and VII of the evidence pyramid. Although the methodological quality of the studies warrants consideration, these studies showed the potential that SEM and

  14. Failure analysis of blistered organic coatings on gray iron castings

    Science.gov (United States)

    Tianen, Matthew N.

    This study investigates the blistering failure of a two part coating consisting of talc-filled polyester resin and polyurethane primer on large gray iron castings. Surface metallography was performed and failed coating was characterized by scanning electron microscopy. Corrosion products were found inside of coating blisters. The proposed blistering mechanism is osmosis as a result of soluble species produced by the corrosion. It was believed that excessively thin primer layers resulted in a poor barrier to permeation of water, leading to blisters, and that a basecoat containing a corrosion inhibitor like zinc phosphate would reduce blistering. These hypotheses were tested with designed experiments using environmental testing in humidity and submersion environments. Thicker primer layers resulted in significant reductions in blistering and prolonged the time required before blister formation. A basecoat containing zinc phosphate was not found to be effective at reducing blistering in this coating system.

  15. Blistering behavior in Mo/Si multilayers

    NARCIS (Netherlands)

    Kuznetsov, Alexey; Gleeson, Michael; van de Kruijs, Robbert Wilhelmus Elisabeth; Bijkerk, Frederik; Kuznetsov, A.S.

    2011-01-01

    This paper is concerned with mapping the characteristics of blistering induced on Mo/Si multilayers as a result of irradiation by hydrogen species generated in a thermal capillary cracker. The nature and extent of the damage observed is dependent on exposure conditions such as the sample

  16. Blister rust resistance among 19 families of whitebark pine, Pinus albicaulis, from Oregon and Washington – early results from an artificial inoculation trial

    Science.gov (United States)

    Angelia Kegley; Richard A. Sniezko; Robert Danchok; Douglas P. Savin

    2012-01-01

    Whitebark pine is considered one of the most susceptible white pine species to white pine blister rust, the disease caused by the non-native pathogen Cronartium ribicola. High mortality from blister rust and other factors in much of the range in the United States and Canada have raised serious concerns about the future viability of this high-...

  17. Strong partial resistance to white pine blister rust in sugar pine

    Science.gov (United States)

    Bohun B. Kinloch, Jr.; Deems Burton; Dean A. Davis; Robert D. Westfall; Joan Dunlap; Detlev Vogler

    2012-01-01

    Quantitative resistance to white pine blister rust in 128 controlled- and open-pollinated sugar pine families was evaluated in a “disease garden”, where alternate host Ribes bushes were interplanted among test progenies. Overall infection was severe (88%), but with great variation among and within families: a 30-fold range in numbers of infections...

  18. Histology of white pine blister rust in needles of resistant and susceptible eastern white pine

    Science.gov (United States)

    Joel A. Jurgens; Robert A. Blanchette; Paul J. Zambino; Andrew David

    2003-01-01

    White pine blister rust, Cronartium ribicola, has plagued the forests of North America for almost a century. Over past decades, eastern white pine (Pinus strobus) that appear to tolerate the disease have been selected and incorporated into breeding programs. Seeds from P. strobus with putative resistance were...

  19. Ion implantation induced blistering of rutile single crystals

    Energy Technology Data Exchange (ETDEWEB)

    Xiang, Bing-Xi [School of Physics, Shandong University, Jinan, Shandong 250100 (China); Jiao, Yang [College of Physics and Electronics, Shandong Normal University, Jinan, Shandong 250100 (China); Guan, Jing [School of Physics, Shandong University, Jinan, Shandong 250100 (China); Wang, Lei [School of Physics, Shandong University, Jinan, Shandong 250100 (China); Key Laboratory of Nanodevices and Applications, Suzhou Institute of Nano-Tech and Nano-Bionics, Chinese Academy of Sciences (China)

    2015-07-01

    The rutile single crystals were implanted by 200 keV He{sup +} ions with a series fluence and annealed at different temperatures to investigate the blistering behavior. The Rutherford backscattering spectrometry, optical microscope and X-ray diffraction were employed to characterize the implantation induced lattice damage and blistering. It was found that the blistering on rutile surface region can be realized by He{sup +} ion implantation with appropriate fluence and the following thermal annealing.

  20. Reliable site for suction blister induction and harvesting

    Directory of Open Access Journals (Sweden)

    Laxmisha Chandrashekar

    2005-01-01

    Full Text Available Background: Suction blister grafting is a useful modality of treatment of patients with resistant and stable vitiligo. However, there have been no detailed studies to find out the best donor site for blister formation. Methods: The study was conducted between the period of October 2004 and February 2005 in the dermatology department at a tertiary care center. Nine patients with vitiligo (focal vitiligo, 3; mucosal vitiligo, 2; acrofacial vitiligo, 2; vitiligo vulgaris, 1; and segmental vitiligo, 1 were selected for blister harvesting and grafting. The blisters were raised using the method described by Gupta et al. Results: Suction blisters were attempted to be raised at 52 sites, but only 38 blisters could be raised, 24 complete and 14 incomplete. Blisters were raised in all the three cases on the flexor aspect of the arm (100%, 15 of 17 cases (88.2% on the flexor aspect of the forearm, 4 of 5 cases (80% on the abdomen, 11 of 16 cases (68.7% on the anterolateral thigh, and less frequently over leg or foot. Complete blisters were formed in 13/15 cases (86.6% on the flexor aspect of the forearm, 6/11 cases (54.5% on the anterolateral thigh, and in all cases over leg. Conclusion: The flexor aspect of the forearm is a good site for suction blister harvesting.

  1. Evaluation of microbial quality of selected blister-packed ...

    African Journals Online (AJOL)

    Ten brands of blister-packed paracetamol tablet and twenty brands of paracetamol syrup marketed in Nigeria were evaluated for their microbial quality. While no microbial contaminant was isolated from all blistered-packed paracetamol tablets, ten of syrups were contaminated with organisms such as Escherichia coli, ...

  2. General mechanism for helium blistering involving displaced atom transport

    Energy Technology Data Exchange (ETDEWEB)

    McDonell, W.R.

    1979-01-01

    A mechanism developed to account for formation of vertically elongated blisters in high displacement environments produced by /sup 252/Cf alpha particles and fission fragments has been extended to formation of done-shaped blisters in the low displacement environments produced by simple helium ion beams. In this mechanism, transport of displaced atoms to relieve compressive stresses in the helium-implanted layer allows interconnections of small, subsurface bubbles to form the blister cavity. The same transport may cause thickening of the blister caps at low implantation energies. The transition from dome-shaped to vertically elongated blistering occurs between the 300 and 3000 displacements per helium atom produced by simple helium ions and /sup 252/Cf radiations respectively.

  3. Blister formation in Mo/Si multilayered structures induced by hydrogen ions

    NARCIS (Netherlands)

    Van Den Bos, R. A.J.M.; Lee, C. J.; Benschop, J. P.H.; Bijkerk, F.

    2017-01-01

    We report on blister formation in nanometer thick Mo/Si multilayer structures due to exposure to hydrogen ion fluxes. The influence of hydrogen flux and ion energy for blister formation have been measured and compared to a blister model. The blister number density increases significantly around 100

  4. Suction blister grafting - Modifications for easy harvesting and grafting

    Directory of Open Access Journals (Sweden)

    2012-01-01

    Full Text Available Suction blister grafting is a simple modality of treatment of patients with resistant and stable vitiligo. But raising the blisters may be time consuming and transferring to the recipient site may be difficult as the graft is ultrathin. By doing some modifications we can make the technique simpler and easier. We can decrease the blister induction time by intradermal injection of saline, exposure to Wood′s lamp, intrablister injection of saline. By these methods we can decrease the blister induction time from 2-3 hrs to 45-90 minutes. After harvesting the graft, it can be transferred to the recipient area by taking the graft on a sterile glass slide, on the gloved finger, rolling the graft over a sterile syringe and then spreading on the recipient area, or taking on the sterile wrapper of paraffin dressing and then placing over the recipient area.

  5. The Causes of Blistering in Boat Building Materials

    Science.gov (United States)

    1986-08-01

    polymerization was initiated using methyl ethyl kcetone peroxide according to manufacturer’s instructions. One set of samples was Initiated with BPO ...advertising or sales promotion purposes. Citation of trade names and manufacturers does not constitute endorsement or approval of such products. TABLE OF...and severity of blisters on boats does not exist. While we have seen over 100 cases of coat blisters, there Is no statistically established correlation

  6. Superficial Dsg2 Expression Limits Epidermal Blister Formation Mediated by Pemphigus Foliaceus Antibodies and Exfoliative Toxins

    Directory of Open Access Journals (Sweden)

    Donna Brennan

    2010-01-01

    Full Text Available Cell-cell adhesion mediated by desmosomes is crucial for maintaining proper epidermal structure and function, as evidenced by several severe and potentially fatal skin disorders involving impairment of desmosomal proteins. Pemphigus foliaceus (PF and staphylococcal scalded skin syndrome (SSSS are subcorneal blistering diseases resulting from loss of function of the desmosomal cadherin, desmoglein 1 (Dsg1. To further study the pathomechanism of these diseases and to assess the adhesive properties of Dsg2, we employed a recently established transgenic (Tg mouse model expressing Dsg2 in the superficial epidermis. Neonatal Tg and wild type (WT mice were injected with purified ETA or PF Ig. We showed that ectopic expression of Dsg2 reduced the extent of blister formation in response to both ETA and PF Ig. In response to PF Ig, we observed either a dramatic loss or a reorganization of Dsg1-α, Dsg1-β, and, to a lesser extent, Dsg1-γ, in WT mice. The Inv-Dsg2 Tg mice showed enhanced retention of Dsg1 at the cell-cell border. Collectively, our data support the role for Dsg2 in cell adhesion and suggest that ectopic superficial expression of Dsg2 can increase membrane preservation of Dsg1 and limit epidermal blister formation mediated by PF antibodies and exfoliative toxins.

  7. Distribution and frequency of a gene for resistance to white pine blister rust in natural populations of sugar pine

    Science.gov (United States)

    Bohun B. Kinloch Jr.

    1992-01-01

    The gametic frequency of a dominant allcle (R) for resistance to white pine blister rust, a disease caused by an introduced pathogen (Cronartium ribicola), in natural populations of sugar pine was estimated by the kind of leaf symptom expressed after artificial inoculation of wind-pollinated seedlings from susceptible seed-parent...

  8. Bullous impetigo and pregnancy: Case report and review of blistering conditions in pregnancy.

    Science.gov (United States)

    Cohen, Philip R

    2016-04-18

     Bullous impetigo results from Staphylococcus aureus (S. aureus) release of exfoliative toxins type A and type B thatresults in flaccid, easily ruptured, bullae in the upper layers of the epidermis.  Physiologic, gestation-associated, and incidental skin changes can occur in pregnancy.  Blisters in pregnant women can occur secondary to either common skin disorders orspecific dermatoses of pregnancy.  To describe a pregnant woman with bullous impetigo and review bullous conditions in pregnant women.  PubMed was used to search the following terms, separately and in combination:  blister, blistering, bullous, gestationis, herpes, herpetiformis, impetigo, pemphigoid, pregnancy, pregnant, psoriasis, pustular, virus. All papers were reviewed and relevant manuscripts, along with their reference citations, were evaluated.  Flaccid, easily rupturing, pustules, which developed into superficial annular erosions with peripheral scale and central healing appeared in a woman of 7-weeks gestation and allergy to penicillin on her lower abdomen, suprapubic region, perineum, buttocks, and proximal legs.  A bacterial culture subsequently isolated methicillin-susceptible S. aureus.  All of the lesions resolved after treatment with clindamycin.  Bullous impetigo should be considered in the differential diagnosis of common skin diseases presenting as blistersin pregnant women.

  9. The flavonoid luteolin inhibits Fcγ-dependent respiratory burst in granulocytes, but not skin blistering in a new model of pemphigoid in adult mice.

    Directory of Open Access Journals (Sweden)

    Eva Oswald

    Full Text Available Bullous pemphigoid is an autoimmune blistering skin disease associated with autoantibodies against the dermal-epidermal junction. Passive transfer of antibodies against BP180/collagen (C XVII, a major hemidesmosomal pemphigoid antigen, into neonatal mice results in dermal-epidermal separation upon applying gentle pressure to their skin, but not in spontaneous skin blistering. In addition, this neonatal mouse model precludes treatment and observation of diseased animals beyond 2-3 days. Therefore, in the present study we have developed a new disease model in mice reproducing the spontaneous blistering and the chronic course characteristic of the human condition. Adult mice were pre-immunized with rabbit IgG followed by injection of BP180/CXVII rabbit IgG. Mice pre-immunized against rabbit IgG and injected 6 times every second day with the BP180/CXVII-specific antibodies (n = 35 developed spontaneous sustained blistering of the skin, while mice pre-immunized and then treated with normal rabbit IgG (n = 5 did not. Blistering was associated with IgG and complement C3 deposits at the epidermal basement membrane and recruitment of inflammatory cells, and was partly dependent on Ly-6G-positive cells. We further used this new experimental model to investigate the therapeutic potential of luteolin, a plant flavonoid with potent anti-inflammatory and anti-oxidative properties and good safety profile, in experimental BP. Luteolin inhibited the Fcγ-dependent respiratory burst in immune complex-stimulated granulocytes and the autoantibody-induced dermal-epidermal separation in skin cryosections, but was not effective in suppressing the skin blistering in vivo. These studies establish a robust animal model that will be a useful tool for dissecting the mechanisms of blister formation and will facilitate the development of more effective therapeutic strategies for managing pemphigoid diseases.

  10. Study on thickness distribution of thermoformed medical PVC blister

    Science.gov (United States)

    Li, Yiping

    2017-08-01

    Vacuum forming has many advantages over other plastic forming processes due to its cost effectiveness, time efficiency, higher product precision, and more design flexibility. Nevertheless, when pressures greater than the atmospheric value are required to force the thermo-plastic into more intimate contact with the mold surface, pressure forming is a better choice. This paper studies the process of air-pressure thermoforming of plastic sheet, and focuses on medical blister PVC products. ANSYS POLYFLOW tool is used to simulate the process and analyze the wall thickness distribution of the blister. The influence of mold parameters on the wall thickness distribution of thermoformed part is thus obtained through simulation. Increasing radius between mold and side wall at the bottom of blister and draft prove to improve the wall thickness distribution.

  11. The ‘Sticky Elastica’: delamination blisters beyond small deformations

    KAUST Repository

    Wagner, Till J. W.

    2013-01-01

    We consider the form of an elastic loop adhered to a rigid substrate: the \\'Sticky Elastica\\'. In contrast to previous studies of the shape of delamination \\'blisters\\', the theory developed accounts for deflections with large slope (i.e. geometrically nonlinear). Starting from the classical Euler Elastica we provide numerical results for the dimensions of such blisters for a variety of end-end confinements and develop asymptotic expressions that reproduce these results well, even up to the point of self-contact. Interestingly, we find that the width of such blisters does not grow monotonically with increased confinement. Our theoretical predictions are confirmed by simple desktop experiments and suggest a new method for the measurement of the elastocapillary length for deformations that cannot be considered small. We discuss the implications of our results for applications such as flexible electronics. © 2013 The Royal Society of Chemistry.

  12. Exposure to a First World War blistering agent.

    Science.gov (United States)

    Le, H Q; Knudsen, S J

    2006-04-01

    Sulfur mustards act as vesicants and alkylating agents. They have been used as chemical warfare since 1917 during the first world war. This brief report illustrates the progression of injury on a primary exposed patient to a first world war blistering agent. This case documents the rapid timeline and progression of symptoms. It emphasises the importance of appropriate personal protective equipment and immediate medical response plan with rapid decontamination and proper action from military and civilian medical treatment facilities. This case reports the first US active duty military exposure to a blistering agent in the age of global terrorism.

  13. Resistance of three interspecific white pine hybrids to blister rust

    Science.gov (United States)

    R. Z. Callaham

    1962-01-01

    Three white pine hybrids exposed to infection by white pine blister rust (Cronartium ribicola Fischer) since 1946 have inherited the relative resistance of their parental species. The hybrids were produced from controlled pollinations in 1940 and 1941 at the Institute of Forest Genetics, Placerville, Calif. Twelve seedlings of each hybrid were...

  14. Microbiological Quality of Blister Pack Tablets in Community ...

    African Journals Online (AJOL)

    Purpose: To investigate the microbiological quality of blister-packed tablets manufactured and marketed in Jordan in order to assess Good Manufacturing ... Six of the products with the highest stratified bacterial count were manufactured by one company and were also found to be contaminated with Aeromonas species.

  15. Blister rust control in the management of western white pine

    Science.gov (United States)

    Kenneth P. Davis; Virgil D. Moss

    1940-01-01

    The forest industry of the western white pine region depends on the production of white pine as a major species on about 2,670,000 acres of commercial forest land. Continued production of this species and maintenance of the forest industry at anything approaching its present level is impossible unless the white pine blister rust is controlled. Existing merchantable...

  16. White pines, Ribes, and blister rust: integration and action

    Science.gov (United States)

    R. S. Hunt; B. W. Geils; K. E. Hummer

    2010-01-01

    The preceding articles in this series review the history, biology and management of white pine blister rust in North America, Europe and eastern Asia. In this integration, we connect and discuss seven recurring themes important for understanding and managing epidemics of Cronartium ribicola in the white pines (five-needle pines in subgenus Strobus). Information and...

  17. Computer simulation of white pine blister rust epidemics

    Science.gov (United States)

    Geral I. McDonald; Raymond J. Hoff; William R. Wykoff

    1981-01-01

    A simulation of white pine blister rust is described in both word and mathematical models. The objective of this first generation simulation was to organize and analyze the available epidemiological knowledge to produce a foundation for integrated management of this destructive rust of 5-needle pines. Verification procedures and additional research needs are also...

  18. Flame figures associated with eosinophilic dermatosis of hematologic malignancy: is it possible to distinguish the condition from eosinophilic cellulitis in patients with hematoproliferative disease?

    Science.gov (United States)

    Qiao, Jianjun; Sun, Chang-E; Zhu, Weifang; Zhu, Dingxian; Fang, Hong

    2013-01-01

    Eosinophilic dermatosis of hematologic malignancy is a multifaceted dermatosis with a wide morphological spectrum, presenting as pruritic, erythematous, papular and occasionally vesicular, urticarial, nodular eruptions. Histopathologically eosinophil infiltration in the super and deep dermis was found. We reported a case of eosinophilic dermatosis of hematologic malignancy presented as urticarial and vesicular lesions in a patient with chronic lymphocytic leukemia. A skin biopsy revealed a prominent subepidermal blister and a diffuse infiltrate of eosinophils with flame figures in the dermis and subcutaneous tissue. Although flame figures associated with eosinophilic dermatosis of hematologic malignancy is rarely reported, we believe that it would not seem unusual to find them in this skin disease. Eosinophilic cellulitis, which share clinical and histological features with eosinophilic dermatosis of hematologic malignancy, has also been described as showing an association with hematoproliferative diseases. In order to clearly describe eosinophilic dermatosis in patients with hematologic malignancies, the terminology eosinophilic dermatosis of hematologic malignancy, instead of eosinophilic cellulitis, would be a more suitable term in patients with eosinophilic dermatosis.

  19. Absent skin at birth with blistering: Bart's Syndrome?

    Directory of Open Access Journals (Sweden)

    Amina Asfiya M Iqbal

    2017-01-01

    Full Text Available Bart's Syndrome is a disorder characterised by aplasia cutis congenita and epidermolysis bullosa. We report a case of a 4-day-old baby who had absent skin over the legs along with blistering and nail dystrophy. The diagnosis of Bart's Syndrome was made based on history and clinical examination. However, detailed investigations and histopathological confirmation is needed for final diagnosis. The management is conservative and needs multidisciplinary support.

  20. Blister-Like Malformations an Tea Seedlings 307

    Indian Academy of Sciences (India)

    Shaw, Dorothy, E. .. “Condition resembling blister blight of tea on tea seedlings . in quarantine in New Guinea,” RAD. Plant Prat. Haiti,. 1965, 13, 56~64. Venkata Ram, C. S. “Report of the plant pathologist,” AR. UPASI Sci. Dept. Tea Sect. for 1964~65, 1965, 18-28. Venkataxamani, K. S. . . “Report of the botanist? A.R. UPASI ...

  1. Blister growth in zirconium alloys: experimentation and modeling

    Energy Technology Data Exchange (ETDEWEB)

    Domizzi, G. [Comision Nacional de Energia Atomica, Buenos Aires (Argentina). Dept. de Materiales; Enrique, R.A. [Instituto Balseiro, Universidad Nacional de Cuyo and Comision Nacional de Energia Atomica, 8400 San Carlos de Bariloche (Argentina); Ovejero-Garcia, J. [Comision Nacional de Energia Atomica, Buenos Aires (Argentina). Dept. de Materiales; Buscaglia, G.C. [Instituto Balseiro, Universidad Nacional de Cuyo and Comision Nacional de Energia Atomica, 8400 San Carlos de Bariloche (Argentina)]|[Centro Atomico Bariloche, Comision Nacional de Energia Atomica, 8400 San Carlos de Bariloche (Argentina)

    1996-04-01

    Hydrogen redistribution in the presence of a cold spot is considered, with hydrogen concentrations above the solid-solubility limit and thus with hydrogen flowing through a hydride-matrix mixture. Fully-hydrided regions (frequently called blisters) grow in the samples, beginning at the cold spot. Under equivalent conditions, the experiment is carried out on several Zr-2.5% Nb samples, allowing for the hydrogen migration times to vary from 1.10{sup 5} to 6.10{sup 5} s, so as to construct a blister-growth curve. Metallographic examination of the samples is performed before and after the imposition of the thermal gradient. A mathematical model is then presented, and the corresponding equations are numerically solved by means of a finite element method, refining the discretization so as to render approximation errors unimportant. Agreement between model and experiment is shown to be quite good for migration times greater than 3.10{sup 5} s. For shorter times, implying small blisters around the cold spot, discrepancies arise between model and experiment, which are attributed to errors in estimating the local temperature field near the sample surface. (orig.).

  2. Pharmacokinetic Profile of Meropenem, Administered at 500 Milligrams Every 8 Hours, in Plasma and Cantharidin-Induced Skin Blister Fluid

    Science.gov (United States)

    Maglio, Dana; Teng, Renli; Thyrum, Per T.; Nightingale, Charles H.; Nicolau, David P.

    2003-01-01

    The pharmacokinetic disposition of meropenem, administered at 500 mg every 8 h, in plasma and cantharidin-induced blister fluid is described. Peak meropenem concentrations in blister fluid lagged behind peak meropenem concentrations in plasma, while a lower elimination rate from blister fluid was also noted. The mean penetration of meropenem into blister fluid was 67%. The pharmacokinetic profile of meropenem in blister fluid supports the utility of this dose in the management of skin and soft tissue infections. PMID:12709358

  3. Boat Hull Blisters: Repair Techniques and Long Term Effects on Hull Degradation

    Science.gov (United States)

    1988-08-01

    blistering of boat hulls is a serious problem which affects many fiber- glass polyester boats. It can range from a surficial cosmetic problem to a deep...something present in the resin itself. We have reported blistering vhen sorbitol is added to the resin. Pritchard has reported on the role of excess glycol...Is blistering and water absorption only a cosmetic and surficial problem or does deep seated damage occur after prolonged water exposure? The

  4. Real-time imaging of suction blistering in human skin using optical coherence tomography

    Science.gov (United States)

    Carvalho, Joana C.O.; Palero, Jonathan A.; Jurna, Martin

    2015-01-01

    Separation of skin epidermis from the dermis by suction blistering has been used with high success rate for autologous skin epidermal grafting in burns, chronic wounds and vitiligo transplantation treatment. Although commercial products that achieve epidermal grafting by suction blistering are presently available, there is still limited knowledge and understanding on the dynamic process of epidermal-dermal separation during suction blistering. In this report we integrated a suction system to an Optical Coherence Tomography (OCT) which allowed for the first time, real-time imaging of the suction blistering process in human skin. We describe in this report the evolution of a suction blister where the growth is modeled with a Boltzmann sigmoid function. We further investigated the relationship between onset and steady-state blister times, blister growth rate, applied suction pressure and applied local skin temperature. Our results show that while the blister time is inversely proportional to the applied suction pressure, the relationship between the blister time and the applied temperature is described by an exponential decay. PMID:26713194

  5. Assessment of imperfect detection of blister rust in whitebark pine within the Greater Yellowstone Ecosystem

    Science.gov (United States)

    Wright, Wilson J.; Irvine, Kathryn M.

    2017-01-01

    We examined data on white pine blister rust (blister rust) collected during the monitoring of whitebark pine trees in the Greater Yellowstone Ecosystem (from 2004-2015). Summaries of repeat observations performed by multiple independent observers are reviewed and discussed. These summaries show variability among observers and the potential for errors being made in blister rust status. Based on this assessment, we utilized occupancy models to analyze blister rust prevalence while explicitly accounting for imperfect detection. Available covariates were used to model both the probability of a tree being infected with blister rust and the probability of an observer detecting the infection. The fitted model provided strong evidence that the probability of blister rust infection increases as tree diameter increases and decreases as site elevation increases. Most importantly, we found evidence of heterogeneity in detection probabilities related to tree size and average slope of a transect. These results suggested that detecting the presence of blister rust was more difficult in larger trees. Also, there was evidence that blister rust was easier to detect on transects located on steeper slopes. Our model accounted for potential impacts of observer experience on blister rust detection probabilities and also showed moderate variability among the different observers in their ability to detect blister rust. Based on these model results, we suggest that multiple observer sampling continue in future field seasons in order to allow blister rust prevalence estimates to be corrected for imperfect detection. We suggest that the multiple observer effort be spread out across many transects (instead of concentrated at a few each field season) while retaining the overall proportion of trees with multiple observers around 5-20%. Estimates of prevalence are confounded with detection unless it is explicitly accounted for in an analysis and we demonstrate how an occupancy model can be used

  6. Activated charcoal and baking soda to reduce odor associated with extensive blistering disorders

    Directory of Open Access Journals (Sweden)

    Chakravarthi Arun

    2008-01-01

    Full Text Available Background: Skin disease leading to extensive blistering and loss of skin is associated with a characteristic smell. Odor can cause physiologic disturbances such as increase in heart rate and respiratory rate. It can also cause nausea and vomiting and is disturbing to bystanders. Aims: To test odor reducing capability of activated charcoal. Methods: In this blinded experimental study we used putrefied amniotic membrane to produce odor and studied the effectiveness of activated charcoal and soda-bi-carbonate to reduce odor. Results: Statistical analysis with Kruskal Wall′s Chi Square Test and Man Whitney U test showed significant reduction of odor using activated charcoal by itself or along with soda-bi-carbonate. Conclusion: We recommend the usage of activated charcoal with/without soda bicarbonate as an inexpensive practical measure to reduce foul odor associated with extensive skin loss.

  7. Activated charcoal and baking soda to reduce odor associated with extensive blistering disorders.

    Science.gov (United States)

    Chakravarthi, Arun; Srinivas, C R; Mathew, Anil C

    2008-01-01

    Skin disease leading to extensive blistering and loss of skin is associated with a characteristic smell. Odor can cause physiologic disturbances such as increase in heart rate and respiratory rate. It can also cause nausea and vomiting and is disturbing to bystanders. To test odor reducing capability of activated charcoal. In this blinded experimental study we used putrefied amniotic membrane to produce odor and studied the effectiveness of activated charcoal and soda-bi-carbonate to reduce odor. Statistical analysis with Kruskal Wall's Chi Square Test and Man Whitney U test showed significant reduction of odor using activated charcoal by itself or along with soda-bi-carbonate. We recommend the usage of activated charcoal with/without soda bicarbonate as an inexpensive practical measure to reduce foul odor associated with extensive skin loss.

  8. Ion effects in hydrogen-induced blistering of Mo/Si multilayers

    NARCIS (Netherlands)

    Kuznetsov, Alexey; Gleeson, M.A.; Bijkerk, Frederik

    2013-01-01

    The role that energetic (>800 eV) hydrogen ions play in inducing and modifying the formation of blisters in nanoscale Mo/Si multilayer samples is investigated. Such samples are confirmed to be susceptible to blistering by two separate mechanisms. The first is attributed to the segregation of H atoms

  9. Ion effects in hydrogen-induced blistering of Mo/Si multilayers

    NARCIS (Netherlands)

    Kuznetsov, A. S.; Gleeson, M. A.; F. Bijkerk,

    2013-01-01

    The role that energetic (>800 eV) hydrogen ions play in inducing and modifying the formation of blisters in nanoscale Mo/Si multilayer samples is investigated. Such samples are confirmed to be susceptible to blistering by two separate mechanisms. The first is attributed to the segregation of

  10. Screening conventional fungicides...control of blister rust on sugar pine in California

    Science.gov (United States)

    Clarence R. Quick

    1967-01-01

    After 5 years, 4 of 14 fungicides tested showed varying pr of development into satisfactory direct control of blister rust. Little promise of systemic control was found. Trees treated were second-growth sugar pine in a mixed conifer forest in eastern Shasta County, California, where blister rust has been intensifying for many years. Most trees received basal-stem...

  11. Sock systems to prevent foot blisters and the impact on overuse injuries of the knee joint.

    Science.gov (United States)

    Van Tiggelen, Damien; Wickes, Simon; Coorevits, Pascal; Dumalin, Mich; Witvrouw, Erik

    2009-02-01

    The incidence of foot blisters and other overuse injuries of the lower limb is very high during basic military training (BMT). One hundred and eighty-nine subjects were divided into two intervention groups wearing alternative sock systems and one control group. Overall, 57% of the 173 recruits who completed the training, developed foot blisters. Binary logistic regression revealed the type of sock, race, previous hiking or military experience, and known orthopedic foot conditions to be predictive variables for foot blisters. Fifty-three percent of the 173 recruits also developed another overuse injury of the lower limb (25.4% related to the knee joint). Previous military or hiking experience and the association of foot blisters revealed to be predictive for the overuse injuries of the knee joint. The results of the present study suggest associated foot blisters are also an important factor in the development of overuse injuries of the knee joint during BMT.

  12. Blister formation on rough and technical tungsten surfaces exposed to deuterium plasma

    Science.gov (United States)

    Manhard, Armin; Balden, Martin; von Toussaint, Udo

    2017-12-01

    Up to now, blister formation on rough or technical tungsten surfaces exposed to hydrogen isotope plasma was believed to be completely suppressed. The few dedicated experiments on this issue that can be found in literature appear to support that claim. Using a novel technique of 3D difference imaging of tungsten surfaces, we now demonstrate that roughness introduced by chemical etching, i.e. without the associated mechanical deformation layer introduced by grinding, only moderately reduces blistering. A technical surface with comparable roughness produced by precision grinding (R a  ⩽  1.6 µm) led to a strong reduction in blister size and density, but blisters were found nevertheless. In this article we give a detailed description of the investigated rough W surfaces and present a statistical evaluation of blistering on these surfaces after exposure to a low-temperature deuterium plasma.

  13. Mesotherapy for skin rejuvenation: assessment of the subepidermal low-echogenic band by ultrasound evaluation with cross-sectional B-mode scanning.

    Science.gov (United States)

    Lacarrubba, Francesco; Tedeschi, Aurora; Nardone, Beatrice; Micali, Giuseppe

    2008-01-01

    Skin-targeted ultrasound is a noninvasive technique that has been extensively used to evaluate age-related dermal changes, and the presence of a subepidermal low-echogenic band (SLEB) has been related to chronic UVR exposure in several studies. Since SLEB echogenicity is photoage-related, the aim of this study was to evaluate, through ultrasound imaging, the effects on skin photoaging of mesotherapy, a treatment approach currently used in cosmetic dermatology for skin rejuvenation. Twenty women (mean age: 46.7 range 40-60 years) with physical signs of moderate photoaging on the dorsum of the hands were enrolled and treated with multiple microinjections of hyaluronic acid (HA) salts of biotechnological origin (1.000 Kd) every week for 4 weeks. In all subjects, ultrasound evaluation was performed at each visit and 1 week after the last treatment to evaluate SLEB echogenicity changes during treatment. At the end of study, a statistically significant (p mesotherapy with HA may be an effective treatment for skin photoaging, as confirmed by ultrasound. Follow-up investigations on larger series of patients are necessary to further evaluate the safety, effectiveness, and duration of effect of this possible therapeutic approach to skin photoaging.

  14. Blistering mechanisms of atomic-layer-deposited AlN and Al2O3 films

    Science.gov (United States)

    Broas, Mikael; Jiang, Hua; Graff, Andreas; Sajavaara, Timo; Vuorinen, Vesa; Paulasto-Kröckel, Mervi

    2017-10-01

    Blistering of protective, structural, and functional coatings is a reliability risk pestering films ranging from elemental to ceramic ones. The driving force behind blistering comes from either excess hydrogen at the film-substrate interface or stress-driven buckling. Contrary to the stress-driven mechanism, the hydrogen-initiated one is poorly understood. Recently, it was shown that in the bulk Al-Al2O3 system, the blistering is preceded by the formation of nano-sized cavities on the substrate. The stress- and hydrogen-driven mechanisms in atomic-layer-deposited (ALD) films are explored here. We clarify issues in the hydrogen-related mechanism via high-resolution microscopy and show that at least two distinct mechanisms can cause blistering in ALD films.

  15. Synergistic helium and deuterium blistering in tungsten–tantalum composites

    Energy Technology Data Exchange (ETDEWEB)

    Dias, M., E-mail: marta.dias@itn.pt [Associação Euratom/IST, Instituto de Plasmas e Fusão Nuclear, Instituto Superior Técnico, Universidade de Lisboa, Av. Rovisco Pais, 1049-001 Lisboa (Portugal); Mateus, R.; Catarino, N.; Franco, N. [Associação Euratom/IST, Instituto de Plasmas e Fusão Nuclear, Instituto Superior Técnico, Universidade de Lisboa, Av. Rovisco Pais, 1049-001 Lisboa (Portugal); Nunes, D. [CENIMAT-I3N, Departamento de Ciência dos Materiais, Faculdade de Ciências e Tecnologia, FCT, Universidade Nova de Lisboa, 2829-516 Caparica (Portugal); Correia, J.B. [LNEG, Laboratório Nacional de Energia e Geologia, Estrada do Paço do Lumiar, 1649-038 Lisboa (Portugal); Carvalho, P.A. [Associação Euratom/IST, Instituto de Plasmas e Fusão Nuclear, Instituto Superior Técnico, Universidade de Lisboa, Av. Rovisco Pais, 1049-001 Lisboa (Portugal); ICEMS, Instituto Superior Técnico, Universidade de Lisboa, Av. Rovisco Pais, 1049-001 Lisboa (Portugal); Hanada, K. [AIST, National Institute of Advanced Industrial Science and Technology, 1-2-1 Namiki, Tsukuba, 305-8564 Ibaraki (Japan); Sârbu, C. [National Institute of Materials and Physics, 105bis Atomistilor street, 077125 Magurele-Ilfov (Romania); and others

    2013-11-15

    Abstruct: Tungsten–tantalum composites with 10 and 20 at.% Ta were prepared by ball milling W powder with Ta fibers and by consolidating the milled materials with spark plasma sintering. The composites were implanted at room temperature with He{sup +} (30 keV with a fluence 5 × 10{sup 21} at/m{sup 2}) and/or D{sup +} (15 keV with a fluence 5 × 10{sup 21} at/m{sup 2}) ion beams. The materials were studied by scanning and high-resolution transmission electron microscopy, both coupled with energy dispersive X-ray spectroscopy, and by X-ray diffraction, Rutherford backscattering spectrometry and nuclear reaction analysis. The microstructure observations revealed that the milling operation resulted in severe fragmentation of the Ta fibers. Furthermore, during the consolidation process the Ta phase acted as oxygen getter and reduced the W oxide present in the original material. The surface of the tungsten–tantalum composites implanted with D{sup +} remained essentially unaltered, while the materials implanted with He{sup +} evidenced blisters on the Ta-rich regions. D retention in the composites increased with He{sup +} pre-implantation.

  16. Management of bullous pemphigoid : the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology

    NARCIS (Netherlands)

    Feliciani, C.; Joly, P.; Jonkman, M. F.; Zambruno, G.; Zillikens, D.; Ioannides, D.; Kowalewski, C.; Jedlickova, H.; Karpati, S.; Marinovic, B.; Mimouni, D.; Uzun, S.; Yayli, S.; Hertl, M.; Borradori, L.

    Bullous pemphigoid is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or generalized bullous lesions. In up to 20% of affected patients, bullae may be completely absent, and

  17. Transcriptome Analysis Reveals Candidate Genes involved in Blister Blight defense in Tea (Camellia sinensis (L) Kuntze)

    Science.gov (United States)

    Jayaswall, Kuldip; Mahajan, Pallavi; Singh, Gagandeep; Parmar, Rajni; Seth, Romit; Raina, Aparnashree; Swarnkar, Mohit Kumar; Singh, Anil Kumar; Shankar, Ravi; Sharma, Ram Kumar

    2016-07-01

    To unravel the molecular mechanism of defense against blister blight (BB) disease caused by an obligate biotrophic fungus, Exobasidium vexans, transcriptome of BB interaction with resistance and susceptible tea genotypes was analysed through RNA-seq using Illumina GAIIx at four different stages during ~20-day disease cycle. Approximately 69 million high quality reads were assembled de novo, yielding 37,790 unique transcripts with more than 55% being functionally annotated. Differentially expressed, 149 defense related transcripts/genes, namely defense related enzymes, resistance genes, multidrug resistant transporters, transcription factors, retrotransposons, metacaspases and chaperons were observed in RG, suggesting their role in defending against BB. Being present in the major hub, putative master regulators among these candidates were identified from predetermined protein-protein interaction network of Arabidopsis thaliana. Further, confirmation of abundant expression of well-known RPM1, RPS2 and RPP13 in quantitative Real Time PCR indicates salicylic acid and jasmonic acid, possibly induce synthesis of antimicrobial compounds, required to overcome the virulence of E. vexans. Compendiously, the current study provides a comprehensive gene expression and insights into the molecular mechanism of tea defense against BB to serve as a resource for unravelling the possible regulatory mechanism of immunity against various biotic stresses in tea and other crops.

  18. Hand Blisters in Major League Baseball Pitchers: Current Concepts and Management.

    Science.gov (United States)

    McNamara, Andrew R; Ensell, Scott; Farley, Timothy D

    2016-01-01

    Friction blisters are a common sequela of many athletic activities. Their significance can range from minor annoyance to major performance disruptions. The latter is particularly true in baseball pitchers, who sustain repeated trauma between the baseball seams and the fingers of the pitching hand, predominately at the tips of the index and long fingers. Since 2010, 6 Major League Baseball (MLB) players accounted for 7 stints on the disabled list (DL) due to blisters. These injuries resulted in a total of 151 days spent on the DL. Since 2012, 8 minor league players spent time on the DL due to blisters. Moreover, there have been several documented and publicized instances of professional baseball pitchers suffering blisters that did not require placement on the DL but did result in injury time and missed starts. The purpose of this article is to review the etiology and pathophysiology of friction blisters with particular reference to baseball pitchers; provide an overview of past and current prevention methods; and discuss our experience in treating friction blisters in MLB pitchers.

  19. 2D simulation of hydride blister cracking during a RIA transient with the fuel code ALCYONE

    Directory of Open Access Journals (Sweden)

    Sercombe Jérôme

    2016-01-01

    Full Text Available This paper presents 2D generalized plain strain simulations of the thermo-mechanical response of a pellet fragment and overlying cladding during a RIA transient. A fictitious hydride blister of increasing depth (25 to 90% of the clad thickness is introduced at the beginning of the calculation. When a pre-determined hoop stress is exceeded at the clad outer surface, radial cracking of the blister is taken into account in the simulation by a modification of the mechanical boundary conditions. The hoop stress criterion is based on Finite Element simulations of laboratory hoop tensile tests performed on highly irradiated samples with a through-wall hydride blister. The response of the remaining clad ligament (beneath the cracked blister to the pellet thermal expansion is then studied. The simulations show that plastic strains localize in a band orientated at ∼45° to the radial direction, starting from the blister crack tip and ending at the clad inner wall. This result is in good agreement with the ductile shear failures of the clad ligaments observed post-RIA transients. Based on a local plastic strain failure criterion in the shear band, ALCYONE simulations are then used to define the enthalpy at failure in function of the blister depth.

  20. A suction blister model reliably assesses skin barrier restoration and immune response.

    Science.gov (United States)

    Smith, Tracey J; Wilson, Marques A; Young, Andrew J; Montain, Scott J

    2015-02-01

    Skin wound healing models can be used to detect changes in immune function in response to interventions. This study used a test-retest format to assess the reliability of a skin suction blister procedure for quantitatively evaluating human immune function in repeated measures type studies. Up to eight suction blisters (~30 mm(2)) were induced via suction on each participant's left and right forearm (randomized order; blister session 1 and 2), separated by approximately one week. Fluid was sampled from each blister, and the top layer of each blister was removed to reveal up to eight skin wounds. Fluid from each wound was collected 4, 7 and 24h after blisters were induced, and proinflammatory cytokines were measured. Transepidermal water loss (TEWL), to assess skin barrier recovery, was measured daily at each wound site until values were within 90% of baseline values (i.e., unbroken skin). Sleep, stress and inflammation (i.e., factors that affect wound healing and immune function), preceding the blister induction, were assessed via activity monitors (Actical, Philips Respironics, Murrysville, Pennsylvania), the Perceived Stress Scale (PSS) and C-reactive protein (CRP), respectively. Area-under-the-curve and TEWL, between blister session 1 and 2, were compared using Pearson correlations and partial correlations (controlling for average nightly sleep, PSS scores and CRP). The suction blister method was considered reliable for assessing immune response and skin barrier recovery if correlation coefficients reached 0.7. Volunteers (n=16; 12 M; 4F) were 23 ± 5 years [mean ± SD]. Time to skin barrier restoration was 4.9 ± 0.8 and 4.8 ± 0.9 days for sessions 1 and 2, respectively. Correlation coefficients for skin barrier restoration, IL-6, IL-8 and MIP-1α were 0.9 (Psuction blister method is sufficiently reliable for assessing skin barrier restoration and immune responsiveness. This data can be used to determine sample sizes for cross-sectional or repeated

  1. Molecular mechanisms of blister formation in bullous impetigo and staphylococcal scalded skin syndrome

    Science.gov (United States)

    Hanakawa, Yasushi; Schechter, Norman M.; Lin, Chenyan; Garza, Luis; Li, Hong; Yamaguchi, Takayuki; Fudaba, Yasuyuki; Nishifuji, Koji; Sugai, Motoyuki; Amagai, Masayuki; Stanley, John R.

    2002-01-01

    Bullous impetigo due to Staphylococcus aureus is one of the most common bacterial infections of man, and its generalized form, staphylococcal scalded skin syndrome (SSSS), is a frequent manifestation of staphylococcal epidemics in neonatal nurseries. Both diseases are mediated by exfoliative toxins (ETs), which show exquisite pathologic specificity in blistering only the superficial epidermis. We show that these toxins act as serine proteases with extremely focused molecular specificity to cleave mouse and human desmoglein 1 (Dsg1) once after glutamic acid residue 381 between extracellular domains 3 and 4. Mutation of the predicted catalytically active serine to alanine completely inhibits cleavage. The mutated ETs bind specifically to Dsg1 by immunofluorescence colocalization and by coimmunoprecipitation. Thus, ETs, through specific recognition and proteolytic cleavage of one structurally critical peptide bond in an adhesion molecule, cause its dysfunction and allow S. aureus to spread under the stratum corneum, the main barrier of the skin, explaining how, although they circulate through the entire body in SSSS, they cause pathology only in the superficial epidermis. PMID:12093888

  2. Non-pathogenic pemphigus foliaceus (PF) IgG acts synergistically with a directly pathogenic PF IgG to increase blistering by p38MAPK-dependent desmoglein 1 clustering✩

    Science.gov (United States)

    Yoshida, Kenji; Ishii, Ken; Shimizu, Atsushi; Yokouchi, Mariko; Amagai, Masayuki; Shiraishi, Ken; Shirakata, Yuji; Stanley, John R.; Ishiko, Akira

    2017-01-01

    Background Pemphigus foliaceus (PF) is an autoimmune blistering disease caused by autoantibodies (Abs) against desmoglein 1 (Dsg1). PF sera contain polyclonal Abs which are heterogeneous mixture of both pathogenic and non-pathogenic Abs, as shown by isolation of monoclonal Abs (mAbs). Objective To investigate how pathogenic and non-pathogenic anti-Dsg1 Abs contribute to blister formation in PF. Methods Using organ-cultured human skin, we compared the effect of a single pathogenic anti-Dsg1 IgG mAb, a single non-pathogenic anti-Dsg1 IgG mAb, and their mixture on blister formation as analyzed by histology, subcellular localization of IgG deposits and desmosomal proteins by confocal microscopy, and desmosomal structure by electron microscopy. In addition, we measured keratinocyte adhesion by an in vitro dissociation assay. Results 24 h after injection, a single pathogenic anti-Dsg1 IgG caused a subcorneal blister with IgG and Dsg1 localized linearly on the cell surface of keratinocytes. A single non-pathogenic anti-Dsg1 IgG bound linearly on the keratinocytes but did not induce blisters. A pathogenic and a non-pathogenic IgG mAb injected together caused an aberrant granular pattern of IgG and Dsg1 in the lower epidermis with blister formation in the superficial epidermis. Electron microscopy demonstrated that the mixture of mAbs shortened desmosomal lengths more than a single mAb in the basal and spinous layers. Furthermore, although Dsg1 clustering required both cross-linking of Dsg1 molecules by the non-pathogenic IgG plus a pathogenic antibody, the latter could be in the form of a monovalent single chain variable fragment, suggesting that loss of trans-interaction of Dsg1 is required for clustering. Finally, a p38MAPK inhibitor blocked Dsg1 clustering. When pathogenic strength was measured by the dissociation assay, a mixture of pathogenic and non-pathogenic IgG mAbs disrupted keratinocyte adhesion more than a single pathogenic mAb. This pathogenic effect was only

  3. Non-pathogenic pemphigus foliaceus (PF) IgG acts synergistically with a directly pathogenic PF IgG to increase blistering by p38MAPK-dependent desmoglein 1 clustering.

    Science.gov (United States)

    Yoshida, Kenji; Ishii, Ken; Shimizu, Atsushi; Yokouchi, Mariko; Amagai, Masayuki; Shiraishi, Ken; Shirakata, Yuji; Stanley, John R; Ishiko, Akira

    2017-03-01

    Pemphigus foliaceus (PF) is an autoimmune blistering disease caused by autoantibodies (Abs) against desmoglein 1 (Dsg1). PF sera contain polyclonal Abs which are heterogeneous mixture of both pathogenic and non-pathogenic Abs, as shown by isolation of monoclonal Abs (mAbs). To investigate how pathogenic and non-pathogenic anti-Dsg1 Abs contribute to blister formation in PF. Using organ-cultured human skin, we compared the effect of a single pathogenic anti-Dsg1 IgG mAb, a single non-pathogenic anti-Dsg1 IgG mAb, and their mixture on blister formation as analyzed by histology, subcellular localization of IgG deposits and desmosomal proteins by confocal microscopy, and desmosomal structure by electron microscopy. In addition, we measured keratinocyte adhesion by an in vitro dissociation assay. 24h after injection, a single pathogenic anti-Dsg1 IgG caused a subcorneal blister with IgG and Dsg1 localized linearly on the cell surface of keratinocytes. A single non-pathogenic anti-Dsg1 IgG bound linearly on the keratinocytes but did not induce blisters. A pathogenic and a non-pathogenic IgG mAb injected together caused an aberrant granular pattern of IgG and Dsg1 in the lower epidermis with blister formation in the superficial epidermis. Electron microscopy demonstrated that the mixture of mAbs shortened desmosomal lengths more than a single mAb in the basal and spinous layers. Furthermore, although Dsg1 clustering required both cross-linking of Dsg1 molecules by the non-pathogenic IgG plus a pathogenic antibody, the latter could be in the form of a monovalent single chain variable fragment, suggesting that loss of trans-interaction of Dsg1 is required for clustering. Finally, a p38MAPK inhibitor blocked Dsg1 clustering. When pathogenic strength was measured by the dissociation assay, a mixture of pathogenic and non-pathogenic IgG mAbs disrupted keratinocyte adhesion more than a single pathogenic mAb. This pathogenic effect was only partially suppressed by the p38MAPK

  4. Immunofluorescence serration pattern analysis as a diagnostic criterion in antilaminin-332 mucous membrane pemphigoid : immunopathological findings and clinical experience in 10 Dutch patients

    NARCIS (Netherlands)

    Terra, J. B.; Pas, H. H.; Hertl, M.; Dikkers, F. G.; Kamminga, N.; Jonkman, M. F.

    2011-01-01

    Background Antilaminin-332 mucous membrane pemphigoid (anti-LN-332 MMP) is a chronic subepidermal blistering disease characterized by IgG anti-epidermal basement membrane zone (BMZ) autoantibodies against laminin-332 (LN-332). Patients with anti-LN-332 MMP have an increased relative risk of

  5. Sensitive and specific assays for routine serological diagnosis of epidermolysis bullosa acquisita

    NARCIS (Netherlands)

    Komorowski, Lars; Mueller, Ralf; Vorobyev, Artem; Probst, Christian; Recke, Andreas; Jonkman, Marcel F.; Hashimoto, Takashi; Kim, Soo-Chan; Groves, Richard; Ludwig, Ralf J.; Zillikens, Detlef; Stoecker, Winfried; Schmidt, Enno

    Background: Epidermolysis bullosa acquisita (EBA) is a severe autoimmune subepidermal blistering disease characterized by autoantibodies against the N-terminal collagenous domain (NC1) of type VII collagen (Col VII). Objective: Development of reliable assays for the detection of anti-Col VII-NC1

  6. Comparison of blistering of W bulk and film deposited by magnetron sputtering under helium irradiation

    Directory of Open Access Journals (Sweden)

    Jiangang Yu

    2017-08-01

    Full Text Available In this work, the W bulk prepared by powder sintering and W film deposited by magnetron sputtering were simultaneously exposed to the helium ions with the energy of 60keV and fluence of 1.0 × 1022 m−2 at room temperature. The surface modifications induced by the helium irradiation were studied by scanning electron microscopy. After helium ion irradiation, numerous blisters were observed on the surface of both samples, some of which burst in various degrees. The formation of blisters is attributed to the high gas pressure in the helium bubbles. In addition, the different structures between W bulk and W film lead to the differences in density and size of blisters.

  7. Gunther′s Disease

    Directory of Open Access Journals (Sweden)

    Gupta Somesh

    1998-01-01

    Full Text Available A three year old female child of Gunther’s disease with blistering over exposed parts of body, mutilation of nose and fingers, scanning alopecia and erythrodontia, is reported. Her urine was strongly positive for uropoprhyrinogen. The blistering could not be prevented by strict avoidance of sun, sunscreen with high SPF, chloroquine, high doses of activated charcoal and betacarotene, though there was significant symptomatic improvement by repeated packed erythrocyte transfusion.

  8. Disease: H01737 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available up of disorders that encompass dozens of clinically and genotypically distinct diseases. It is characterized... by mechanically fragile skin that readily blister. Most of the more severe subtypes are associated with clinica

  9. Comparative morphological analysis of apple blister mite, Eriophyes mali Nal., a new pest in Serbia

    Directory of Open Access Journals (Sweden)

    Biljana Vidović

    2014-06-01

    Full Text Available The apple blister mite, Eriophyes mali Nalepa, 1926 (Acari: Prostigmata: Eriophyoidea, has been recently found in Serbia as a new pest of apple. The history of its research, the results of a morphological analysis and degree of infestation are presented. A comparison of the main morphological features of mites from different populations of remote geographical origin has shown that the apple blister mite from Serbia is most similar to another European population (Bulgarian [or Austrian?] while it differs from E. mali originating from the USA and New Zealand. The percentage of infestation varied from 1.6% to 87.6%, with an average of 22.4%.

  10. White pine blister rust in Korea, Japan and other Asian regions: comparisons and implications for North America

    Science.gov (United States)

    M.-S. Kim; N. B. Klopfenstein; Y. Ota; S. K. Lee; K.-S. Woo; S. Kaneko

    2010-01-01

    This article briefly reviews the history of white pine blister rust, attributed to Cronartium ribicola, and addresses current research and management issues in South Korea, Japan and other regions of eastern Asia (China, Russia and Himalaya). For each region, the distribution, damage, aecial hosts, telial hosts and management of C. ribicola and other blister rust fungi...

  11. White pine blister rust resistance of 12 western white pine families at three field sites in the Pacific Northwest

    Science.gov (United States)

    Richard A. Sniezko; Robert Danchok; Jim Hamlin; Angelia Kegley; Sally Long; James Mayo

    2012-01-01

    Western white pine (Pinus monticola Douglas ex D. Don) is highly susceptible to the non-native, invasive pathogen Cronartium ribicola, the causative agent of white pine blister rust. The susceptibility of western white pine to blister rust has limited its use in restoration and reforestation throughout much of western North...

  12. Disease: H01650 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available e dermal-epidermal junction that clinically can manifest with urticarial lesions, tense blisters, and erosio...ly protein BP230. Most of these antibodies belong to the immunoglobulin G class. Diagnosis of BP relies on the clinica...osis disease. It is primarily a disease of the elderly with an equal incidence in men and women. The clini...cal characteristics are the formation of tense blisters and pruritic urticarial ery

  13. Polymerase Chain Reaction (PCR) applications in white pine blister rust resistance screening

    Science.gov (United States)

    Sam Hendricks; Wendy Sutton; Jeffrey Stone; Richard Sniezko; Angelia Kegley; Anna Schoettle

    2011-01-01

    A goal of breeding programs for resistance to white pine blister rust is the development of multigenic resistance, even if the genetics and mechanisms of resistance may be imperfectly understood. The goal of multigenic resistance has prompted efforts to categorize host resistance reactions at increasingly finer scales, to identify heritable traits that may confer...

  14. Study of hydrogen implantation-induced blistering in GaSb for potential layer transfer applications

    Science.gov (United States)

    Pathak, Ravi; Dadwal, U.; Singh, R.

    2017-07-01

    GaSb samples were implanted by 100 keV hydrogen ions (H+) at room temperature with fluence values of 1  ×  1017 and 2  ×  1017 ions cm-2. Post-implantation annealing studies revealed that the samples implanted with a fluence of 2  ×  1017 ions cm-2 did not show blistering/exfoliation. For the lower fluence, the samples showed the formation of surface blisters/craters along with the large area exfoliation of the top H-implanted surface. Topographical investigations of the samples were carried out using Nomarski optical microscopy, atomic force microscopy and stylus surface profilometry. The lateral sizes and heights of the blisters varied between 2-5 µm and 5-20 nm respectively. The root mean square roughness of the exfoliated region was about 12 nm while the exfoliation depth was found to be 730 nm. The exfoliation depth in the H-implanted GaSb is close to the damage concentration peak as found from SRIM calculations. The Föppl-von Karman theory of thin plates has been used to understand the effect of internal pressure and stress on the surface blistering. Using the above mentioned implantation and annealing parameters, potential layer transfer of GaSb could be enabled.

  15. Options for the management of white pine blister rust in the Rocky Mountain Region

    Science.gov (United States)

    Kelly S. Burns; Anna W. Schoettle; William R. Jacobi; Mary F. Mahalovich

    2008-01-01

    This publication synthesizes current information on the biology, distribution, and management of white pine blister rust (WPBR) in the Rocky Mountain Region. In this Region, WPBR occurs within the range of Rocky Mountain bristlecone pine (Pinus aristata), limber pine (P. flexilis), and whitebark pine (P. albicaulis...

  16. Monitoring white pine blister rust infection and mortality in whitebark pine in the Greater Yellowstone ecosystem

    Science.gov (United States)

    Cathie Jean; Erin Shanahan; Rob Daley; Gregg DeNitto; Dan Reinhart; Chuck Schwartz

    2011-01-01

    There is a critical need for information on the status and trend of whitebark pine (Pinus albicaulis) in the Greater Yellowstone Ecosystem (GYE). Concerns over the combined effects of white pine blister rust (WPBR, Cronartium ribicola), mountain pine beetle (MPB, Dendroctonus ponderosae), and climate change prompted an interagency working group to design and implement...

  17. Distribution of Ribes, an alternate host of white pine blister rust, in Colorado and Wyoming

    Science.gov (United States)

    Holly S. J. Kearns; William R. Jacobi; Kelly S. Burns; Brian W. Geils

    2008-01-01

    Ribes (currants and gooseberries) are alternate hosts for Cronartium ribicola, the invasive fungus that causes blister rust of white pines (Pinus, subgenus Strobus) in the Rocky Mountain region of Colorado and Wyoming. The location, species, and density of Ribes can affect...

  18. Resistance to white pine blister rust in Pinus flexilis and P

    Science.gov (United States)

    Anna W. Schoettle; Richard A. Sniezko; Angelia Kegley; Jerry Hill; Kelly S. Burns

    2010-01-01

    The non-native fungus Cronartium ribicola, that causes white pine blister rust (WPBR), is impacting or threatening limber pine, Pinus flexilis, and Rocky Mountain bristlecone pine, Pinus aristata. In the Southern Rockies, where the rust invasion is still expanding, we have the opportunity to be proactive and prepare the landscape for invasion. Genetic...

  19. The influence of white pine blister rust on seed dispersal in whitebark pine

    Science.gov (United States)

    Shawn T. McKinney; Diana F. Tomback

    2007-01-01

    We tested the hypotheses that white pine blister rust (Cronartium ribicola J.C. Fisch.) damage in whitebark pine (Pinus albicaulis Engelm.) stands leads to reduced (1) seed cone density, (2) predispersal seed survival, and (3) likelihood of Clark's Nutcracker (Nucifraga columbiana (Wilson, 1811)) seed...

  20. Non-destructive Quality control of tablets and blister packs by UV imaging

    DEFF Research Database (Denmark)

    Klukkert, Marten; Wu, Jian Xiong; Rantanen, Jukka

    2016-01-01

    Quality control of tablets and its primary packing material within the manufacturing line requires analytical routines that allow monitoring of the desired product attributes with high efficiency. The aim of this study was to evaluate the suitability of multispectral UV imaging combined with mult......Quality control of tablets and its primary packing material within the manufacturing line requires analytical routines that allow monitoring of the desired product attributes with high efficiency. The aim of this study was to evaluate the suitability of multispectral UV imaging combined...... with multivariate image analysis for verification of blister pack filling, differentiation of tablets of varying composition therein, as well as detection of imprint defects and surface cracks of bulk tablets. Moreover, the influence of polymer sealing foils on tablet characterization within blister cavities...... was investigated. Several tablets of different composition were imaged either as bulk, within unsealed blister packs, or within blister packs that were manually sealed with three different types of either PVC or PCTFE foils. It was demonstrated that UV imaging is a fast and reliable technique for counting...

  1. Conservation of biodiversity in sugar pine: effects of the blister rust epidemic on genetic diversity

    Science.gov (United States)

    Constance I. Millar; Bohun B. Kinloch; Robert D. Westfall

    1992-01-01

    Genetic diversity in sugar plne will be severely reduced by the blister rust pandemic predicted within the next 50 to 75 years. We model effects of the epidemic on genetic diversity at the stand and landscape levels for both natural and artificial regeneration. In natural stands, because natural frequencies of the dominant gene (R) for resistance are low, the most...

  2. Selection for resistance to white pine blister rust affects the abiotic stress tolerances of limber pine

    Science.gov (United States)

    Patrick J. Vogan; Anna W. Schoettle

    2015-01-01

    Limber pine (Pinus flexilis) mortality is increasing across the West as a result of the combined stresses of white pine blister rust (Cronartium ribicola; WPBR), mountain pine beetle (Dendroctonus ponderosae), and dwarf mistletoe (Arceuthobium cyanocarpum) in a changing climate. With the continued spread of WPBR, extensive mortality will continue with strong selection...

  3. Increased endothelin-1 and diminished nitric oxide levels in blister fluids of patients with intermediate cold type complex regional pain syndrome type 1

    Directory of Open Access Journals (Sweden)

    Niehof Sjoerd

    2006-11-01

    Full Text Available Abstract Background In complex regional pain syndrome type 1 (CRPS1 pro-inflammatory mediators and vascular changes play an important role in the sustained development and outcome of the disease. The aim of this study was to determine the involvement of vasoactive substances endothelin-1 (ET-1 and nitric oxide (NO during early chronic CRPS1. Methods Included were 29 patients with CRPS 1 who were diagnosed during the acute stage of their disease and observed during follow-up visits. Disease activity and impairment were determined and artificial suction blisters were made on the CRPS1 and the contralateral extremities for measurements of IL-6, TNF-α, ET-1 and nitrate/nitrite (NOx. Results The levels of IL-6, TNF-α and ET-1 in blister fluid in the CRPS1 extremity versus the contralateral extremity were significantly increased and correlated with each other, whereas NOx levels were decreased. Conclusion The NOx/ET-1 ratio appears to be disturbed in the intermediate stage of CRPS, resulting in vasoconstriction and consequently in a diminished tissue blood distribution.

  4. Reproduction in Laboratory and characterization of Blister of Hydride of zirconium in nuclear fuel pods; Reproduccion en laboratorio y caracterizacion de Blisters de hidroduro de circonio en muestras de vaina de combustible nuclear

    Energy Technology Data Exchange (ETDEWEB)

    Martin Rengel, M. A.; Ruiz-Hervias, J.; Munoz, P.

    2014-07-01

    This paper have replicated in laboratory blisters of different size in samples of pod of ZIRLO pre-hydrided evenly with 500 ppm of hydrogen. For these samples was used a technique of cathodic charging in basic medium. To produce the blister was heated up to about 350 degree centigrade in its outer surface sample. With the aim of producing a point cold on the surface of the sheath contacted the surface with a piece of aluminum water-cooled (cold finger). Was held a morphological characterization of the blisters by means of optical microscopy and found that the size of the produced blister is function of the contact time between fuel pod and cold finger. (Author)

  5. Effect of periodic deuterium ion irradiation on deuterium retention and blistering in Tungsten

    Directory of Open Access Journals (Sweden)

    M. Oya

    2017-08-01

    Full Text Available The effect of periodic irradiation on Deuterium (D retention and blistering in Tungsten (W was investigated. W samples were exposed to D plasma at a fixed fluence while varying the irradiation cycle number (1-shot, 2-shots and 3-shots. Exposure energy and flux were ∼50eV and ∼1 ×1022 D m−2 s−1, respectively. Sample temperatures were 537K and 643K. At 573K, D retention and blister density decreased with increasing number of irradiation cycle. In contrast at 643K, D retention showed no dependence on number of irradiation cycle. Therefore, sample temperature during irradiation is an important parameter in comparing the results of continuous and periodic irradiation, especially in studies involving extremely-high-flux (>1024 D m−2 s−1 irradiation and fluence dependency of D retention.

  6. Blister formation and hydrogen retention in aluminium and beryllium: A modeling and experimental approach

    Directory of Open Access Journals (Sweden)

    C. Quirós

    2017-08-01

    Full Text Available Experiments were performed in a low pressure-high density plasma reactor in order to study the impact of hydrogen retention in aluminium under plasma conditions. Microscopy scans of the surface were performed before and after 1h plasma exposure (fluence 6.1 ×1023ions/m2 where it is seen that blisters start to nucleate at the grain boundaries. Investigation on blister growth kinetics was performed for fluences ranging between 6 ×1023 and 3.7 ×1024ions/m2. The evolution of the characteristic size of the projected area was also analyzed. Finally, a macroscopic rate equations (MRE code was used to simulate hydrogen retention and diffusion in Al and bubble growth in the bulk was simulated using experimental results. This model was also used to simulate these phenomena in Be and compare its behavior with respect to Al.

  7. Blisters in BDF-type control rods: Engineering final summary report

    Energy Technology Data Exchange (ETDEWEB)

    Riedeman, G.W.

    1965-08-30

    The scope of this report defies a concise abstract. In lieu of an abstract, the following topical outline will both indicate the subjects covered and show how these subjects are organized. Part I, Engineering Aspects: Blister Formation, Temperature Effects, Cooling Effects, Drive Connections, Housing Effects, Housing Materials, Radiation Effects. Part II, The Behavior of Boron Carbide-Aluminum Poisons: Swelling, Swelling Rates, Restraint, Material Behavior.

  8. First report of the white pine blister rust pathogen, Cronartium ribicola, in Arizona

    Science.gov (United States)

    M. L. Fairweather; Brian Geils

    2011-01-01

    White pine blister rust, caused by Cronartium ribicola J.C. Fisch., was found on southwestern white pine (Pinus flexilis James var. reflexa Engelm., synonym P. strobiformis Engelm.) near Hawley Lake, Arizona (Apache County, White Mountains, 34.024°N, 109.776°W, elevation 2,357 m) in April 2009. Although white pines in the Southwest (Arizona and New Mexico) have been...

  9. Unruptured anterior communicating artery aneurysm with co-existing blister aneurysms: case report and review of literature

    Directory of Open Access Journals (Sweden)

    Karthikeyan Y. R.

    2017-06-01

    Full Text Available Blister aneurysms are a separate class of vascular malformations with a unique etiopathogenesis and clinical profile, elusive to radiological imaging and complex to manage. Unless identified and managed appropriately they often lead increased morbidity intra and post operatively. They are commonly reported in internal carotid artery. We are reporting a rare case of intraoperatively diagnosed blister aneurysm of the anterior cerebral artery, the management options and the importance of constant vigilance in cases where the aneurysm appears unruptured intraoperatively.

  10. Deuterium implantation into Y2O3-doped and pure tungsten: Deuterium retention and blistering behavior

    Science.gov (United States)

    Zhao, M.; Jacob, W.; Manhard, A.; Gao, L.; Balden, M.; von Toussaint, U.; Zhou, Z.

    2017-04-01

    The blistering and near-surface deuterium retention of a Y2O3-doped tungsten (W) and two different pure W grades were studied after exposure to deuterium (D) plasma at elevated temperatures (370, 450 and 570 K). Samples were exposed to a deuterium fluence of 6 × 1024 D m-2 applying a moderate ion flux of about 9 × 1019 D m-2 s-1 at an ion energy of 38 eV/D. Morphological modifications at the surface were analyzed by confocal laser scanning microscopy and scanning electron microscopy. The D depth profiles and the accumulated D inventories within the topmost 8 μm were determined by nuclear reaction analysis. Blistering and deuterium retention were strongly dependent on the implantation temperature. In addition, blistering was sensitively influenced by the used tungsten grade, although the total amount of retained D measured by nuclear reaction analysis was comparable. Among the three different investigated tungsten grades, Y2O3-doped W exhibited the lowest degree of surface modification despite a comparable total D retention.

  11. Evaluation of haemoglobin in blister fluid as an indicator of paediatric burn wound depth.

    Science.gov (United States)

    Tanzer, Catherine; Sampson, Dayle L; Broadbent, James A; Cuttle, Leila; Kempf, Margit; Kimble, Roy M; Upton, Zee; Parker, Tony J

    2015-08-01

    The early and accurate assessment of burns is essential to inform patient treatment regimens; however, this first critical step in clinical practice remains a challenge for specialist burns clinicians worldwide. In this regard, protein biomarkers are a potential adjunct diagnostic tool to assist experienced clinical judgement. Free circulating haemoglobin has previously shown some promise as an indicator of burn depth in a murine animal model. Using blister fluid collected from paediatric burn patients, haemoglobin abundance was measured using semi-quantitative Western blot and immunoassays. Although a trend was observed in which haemoglobin abundance increased with burn wound severity, several patient samples deviated significantly from this trend. Further, it was found that haemoglobin concentration decreased significantly when whole cells, cell debris and fibrinous matrix was removed from the blister fluid by centrifugation; although the relationship to depth was still present. Statistical analyses showed that haemoglobin abundance in the fluid was more strongly related to the time between injury and sample collection and the time taken for spontaneous re-epithelialisation. We hypothesise that prolonged exposure to the blister fluid microenvironment may result in an increased haemoglobin abundance due to erythrocyte lysis, and delayed wound healing. Copyright © 2015 Elsevier Ltd and ISBI. All rights reserved.

  12. Investigation of the Cause of Low Blister Threshold Temperatures in the RERTR-12 and AFIP-4 Experiments

    Energy Technology Data Exchange (ETDEWEB)

    Mitchell K Meyer

    2012-06-01

    Blister–threshold testing of fuel plates is a standard method through which the safety margin for operation of plate-type in research and test reactors is assessed. The blister-threshold temperature is indicative of the ability of fuel to operate at high temperatures for short periods of time (transient conditions) without failure. This method of testing was applied to the newly developed U-Mo monolithic fuel system. Blister annealing studies on the U-Mo monolithic fuel plates began in 2007, with the Reduced Enrichment for Research and Test Reactors (RERTR)-6 experiment, and they have continued as the U-Mo fuel system has evolved through the research and development process. Blister anneal threshold temperatures from early irradiation experiments (RERTR-6 through RERTR-10) ranged from 400 to 500°C. These temperatures were projected to be acceptable for NRC-licensed research reactors and the high-power Advanced Test Reactor (ATR) and the High Flux Isotope Reactor (HFIR) based on current safety-analysis reports (SARs). Initial blister testing results from the RERTR-12 experiment capsules X1 and X2 showed a decrease in the blister-threshold temperatures. Blister threshold temperatures from this experiment ranged from 300 to 400°C. Selected plates from the AFIP-4 experiment, which was fabricated using a process similar to that used to fabricate the RERTR-12 experiment, also underwent blister testing to determine whether results would be similar. The measured blister-threshold temperatures from the AFIP-4 plates fell within the same blister-threshold temperature range measured in the RERTR-12 plates. Investigation of the cause of this decrease in bister threshold temperature is being conducted under the guidance of Idaho National Laboratory PLN-4155, “Analysis of Low Blister Threshold Temperatures in the RERTR-12 and AFIP-4 Experiments,” and is driven by hypotheses. The main focus of the investigation is in the following areas: 1. Fabrication variables 2. Pre

  13. Hand, foot, and mouth disease on the soles (image)

    Science.gov (United States)

    Hand, foot, and mouth disease is cause by a coxsackie virus. It produces mouth ulcers and small blisters (vesicles) on the hands and feet. The vesicles often have a reddish border with a white or lighter colored area in ...

  14. Blister formation on 13Cr2MoNbVB ferritic-martensitic steel exposed to hydrogen plasma

    Science.gov (United States)

    Nikitin, A. V.; Tolstolutskaya, G. D.; Ruzhytskyi, V. V.; Voyevodin, V. N.; Kopanets, I. E.; Karpov, S. A.; Vasilenko, R. L.; Garner, F. A.

    2016-09-01

    The influence of pre-irradiation specimen deformation level on surface blister formation and sub-surface cracking of dual-phase 13Cr2MoNbVB ferritic-martensitic steel was studied using glow discharge hydrogen plasma with ion energy of 1 keV to fluences of 2 × 1025 H/m2. Protium was used for most studies, but deuterium was used for measuring the depth dependence of hydrogen diffusion. Formation of blisters was observed in the temperature range 230-340 K. It was found that pre-irradiation deformation caused changes in the threshold fluences of blister formation and also in blister size distribution. Subsurface cracks located on grain boundaries far beyond the implantation zone were formed concurrently with blisters, arising from hydrogen diffusion and trapping at defects. It was observed that cracks as long as 1 mm in length were formed in 95% deformed steel at depths up to 500 μm from surface.

  15. Foot-and-Mouth Disease

    DEFF Research Database (Denmark)

    Belsham, Graham; Charleston, Bryan; Jackson, Terry

    2015-01-01

    Foot‐and‐mouth disease (FMD) is an economically important, highly contagious disease of cloven‐hoofed animals characterised by the appearance of vesicles (blisters) on the feet and in, and around, the mouth. The causative agent, foot‐and‐mouth disease virus (FMDV), was the first mammalian virus...

  16. Foot-and-mouth disease

    DEFF Research Database (Denmark)

    Belsham, Graham; Charleston, Bryan; Jackson, Terry

    2009-01-01

    Foot-and-mouth disease is an economically important, highly contagious, disease of cloven-hoofed animals characterized by the appearance of vesicles (blisters) on the feet and in and around the mouth. The causative agent, foot-and-mouth disease virus, was the first mammalian virus to be discovered...

  17. Epidermolysis bullosa acquisita: current diagnosis and therapy

    Directory of Open Access Journals (Sweden)

    Christine R. Mehren

    2011-11-01

    Full Text Available Epidermolysis bullosa acquisita (EBA is an acquired, autoimmune subepidermal blistering disease with an approximate prevalence of 0,2/million people. The hallmark of EBA is the presence of autoantibodies (mainly IgG class to anchoring fibril collagen (type VII collagen located at the dermal-epidermal junction. Clinically EBA is subdivided into the inflammatory and the non-inflammatory phenotypes, depending on the level of the cleavage in the basal membrane. A recent addition to the diagnostic techniques is the analysis of the serration pattern of the autoantibody deposits at the basal membrane in the direct immunofluorescence. EBA and the closely related bullous systemic lupus erythematosus are the only diseases presenting with the so-called u-serration pattern which distinguishes them from many other autoimmune subepidermal blistering diseases. We also discuss the recent advances in therapy, including the experience with Rituximab.

  18. Diagnosis and clinical features of epidermolysis bullosa acquisita.

    Science.gov (United States)

    Caux, Frédéric

    2011-07-01

    Epidermolysis bullosa acquisita (EBA) is a rare autoimmune subepidermal blistering disease characterized by immune deposits on anchoring fibrils of cutaneous and mucosal basement membrane zones. It is due to circulating antibodies directed to type VII collagen. Clinical manifestations include a classical form with skin fragility, blisters and scars on trauma-prone surfaces, an inflammatory form, and a cicatricial pemphigoid-like form. Specialized tests available in only certain laboratories are necessary to confirm a diagnosis of EBA, such as immunoelectron microscopy, immunoblotting, or ELISA using recombinant proteins. A frequent association between EBA and Crohn disease has been observed. Copyright © 2011 Elsevier Inc. All rights reserved.

  19. Random amplified polymorphic DNA markers tightly linked to a gene for resistance to white pine blister rust in sugar pine

    Science.gov (United States)

    Michael E. Devey; Annette Delfino-Mix1; Bohun B. Kinloch; David B. NEALEt

    1995-01-01

    We have genetically mapped a gene for resistance to white pine blister rust (Cronartium ribicola Fisch.) in sugar pine (Pinus lambertiana Dougl.) by using an approach which relies on three factors: (i) the ability to assay for genetic markers in the haploid stage of the host's life cycle, using...

  20. Using landscape genetics simulations for planting blister rust resistant whitebark pine in the US northern Rocky Mountains

    Science.gov (United States)

    Erin L. Landguth; Zachary A. Holden; Mary F. Mahalovich; Samuel A. Cushman

    2017-01-01

    Recent population declines to the high elevation western North America foundation species whitebark pine, have been driven by the synergistic effects of the invasive blister rust pathogen, mountain pine beetle (MPB), fire exclusion, and climate change. This has led to consideration for listing whitebark pine (WBP) as a threatened or endangered species under the...

  1. Mitigated blistering and deuterium retention in tungsten exposed to high-flux deuterium–neon mixed plasmas

    NARCIS (Netherlands)

    Cheng, L.; De Temmerman, G.; Morgan, T. W.; Schwartz-Selinger, T.; Yuan, Y.; Zhou, H. B.; Wang, B.; Zhang, Y.; Lu, G. H.

    2017-01-01

    Surface morphology and deuterium retention in tungsten exposed at surface temperature of  550 K to mixed deuterium–neon plasmas of different neon concentrations are investigated. It is found that the addition of neon up to 20% mitigates blistering on the surface. Cross-section view of the surface

  2. Influence of Process Temperatures on Blister Creation in Micro Film Insert Molding of a Dual Layer Membrane

    DEFF Research Database (Denmark)

    Wöhner, Timo; R. Whiteside, Ben; Tosello, Guido

    2016-01-01

    In this work the suitability of a dual layer membrane, consisting of a non-woven Polypropylene (PP) support and a membrane layer made out of Polyethylene Terephthalate (PET) for Micro Film Insert Molding (μFIM) was investigated. The emergence of blisters at the surface of the PET-membrane layer...

  3. Using Landscape Genetics Simulations for Planting Blister Rust Resistant Whitebark Pine in the US Northern Rocky Mountains

    Science.gov (United States)

    Landguth, Erin L.; Holden, Zachary A.; Mahalovich, Mary F.; Cushman, Samuel A.

    2017-01-01

    Recent population declines to the high elevation western North America foundation species whitebark pine, have been driven by the synergistic effects of the invasive blister rust pathogen, mountain pine beetle (MPB), fire exclusion, and climate change. This has led to consideration for listing whitebark pine (WBP) as a threatened or endangered species under the Endangered Species Act, which has intensified interest in developing management strategies for maintaining and restoring the species. An important, but poorly studied, aspect of WBP restoration is the spatial variation in adaptive genetic variation and the potential of blister rust resistant strains to maintain viable populations in the future. Here, we present a simulation modeling framework to improve understanding of the long-term genetic consequences of the blister rust pathogen, the evolution of rust resistance, and scenarios of planting rust resistant genotypes of whitebark pine. We combine climate niche modeling and eco-evolutionary landscape genetics modeling to evaluate the effects of different scenarios of planting rust-resistant genotypes and impacts of wind field direction on patterns of gene flow. Planting scenarios showed different levels for local extirpation of WBP and increased population-wide blister rust resistance, suggesting that the spatial arrangement and choice of planting locations can greatly affect survival rates of whitebark pine. This study presents a preliminary, but potentially important, framework for facilitating the conservation of whitebark pine. PMID:28239390

  4. A paradigm shift for white pine blister rust: Non-Ribes alternate hosts for Cronartium ribicola in North America

    Science.gov (United States)

    Paul J. Zambino; Bryce A. Richardson; Geral I. McDonald; Ned B. Klopfenstein; Mee-Sook. Kim

    2007-01-01

    Naturally occurring Cronartium ribicola infections were discovered in August and September, 2004 on Pedicularis racemosa and Castilleja miniata in a mixed stand of white pine blister rust-infected whitebark pine (Pinus albicaulis) and western white pine (P. monticola) in northern Idaho, at Roman Nose Lakes, ca 30 km west of Bonners Ferry. Infections were confirmed by...

  5. Non-Ribes alternate hosts of white pine blister rust: What this discovery means to whitebark pine

    Science.gov (United States)

    Paul J. Zambino; Bryce A. Richardson; Geral I. McDonald; Ned B. Klopfenstein; Mee-Sook. Kim

    2006-01-01

    From early to present-day outbreaks, white pine blister rust caused by the fungus Cronartium ribicola, in combination with mountain pine beetle outbreaks and fire exclusion has caused ecosystem-wide effects for all five-needled pines (McDonald and Hoff 2001). To be successful, efforts to restore whitebark pine will require sound management decisions that incorporate an...

  6. A new genus and species of Cecidomyiidae (Diptera) from leaf blister galls on Ribes (Grosulariaceae)in North America

    Science.gov (United States)

    Ribesia sarae Gagné, new genus, new species(Diptera: Cecidomyiidae), is described from simple leaf blister galls on Ribes aureum(Grossulariaceae) from Montana. The female abdomen is superficially similar to that of CystiphoraKieffer and SackenomyiaFelt. The three genera are compared. Because of stro...

  7. Status of white pine blister rust and seed collections in california's high-elevation white pine species

    Science.gov (United States)

    J. Dunlap

    2011-01-01

    White pine blister rust (caused by the non-native pathogen Cronartium ribicola) reached northern California about 80 years ago. Over the years its spread southward had been primarily recorded on sugar pine. However, observations on its occurrence had also been reported in several of the higher elevation five-needled white pine species in California. Since the late...

  8. A kinetic stochastic model of blistering and nanofilm islands deposition: self-organization problem

    Energy Technology Data Exchange (ETDEWEB)

    Zmievskaya, G I; Bondareva, A L; Levchenko, V D; Levchenko, T V [M V Keldysh Institute of Applied Mathematics, Russian Academy of Sciences, Moscow (Russian Federation)

    2007-08-21

    First-order phase transition at a fluctuation stage into non-linear dissipative plasma-like media is considered. The clustering of new phase germs (or nucleation) is represented by stochastic Wiener processes. Brownian motion of clusters induced by a long-range potential of indirect (through acoustic phonons and Friedel's oscillation of electron density) interaction between one another is taken into account. Kinetic models for blistering materials in a controlled thermonuclear reactor and for melted metal thin film islands deposition during surface CVD modification are both put forward. The non-steady-state distribution of clusters versus their size and position in space is calculated using Ito-Stratonovich stochastic differential equations. Formation of radiation stimulated porosity layers in a lattice as well as liquid island chains on the surface are to be discussed as characteristics of phase transition at fluctuation stages as well as a new kind of self-organization phenomenon.

  9. Bullous Pemphigoid Induced by Vildagliptin

    OpenAIRE

    Bengür Taşkıran Bahattin; Erdoğan Canan Solak; Şişman Güven; Barış Cansu

    2016-01-01

    Bullous pemphigoid (BP) is an uncommon chronic, autoimmune, and subepidermal disease. Tense blisters occur on normal or erythematous skin. It can be induced by medications. There is a number of reports on BP induced by dipeptidyl peptidase 4 (DPP-4) inhibitors (vildagliptin, sitagliptin, saxagliptin). DPP-4 (CD26), present as a cell surface molecule on immune cells, also plays an important costimulatory role in immune activation. BP more commonly affects elderly men. We present a case of BP i...

  10. Bullous skin diseases: classical types of autoimmune diseases.

    Science.gov (United States)

    Damoiseaux, Jan

    2013-01-01

    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 (hemi)desmosomes. 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.

  11. Bullous pemphigoid associated with prostate adenocarcinoma

    Directory of Open Access Journals (Sweden)

    Öztürkcan Serap

    2004-01-01

    Full Text Available Bullous pemphigoid is a common autoimmune skin disease characterized by the presence of subepidermal blisters. It has been associated with underlying neoplasia in isolated reports. A 78-year-old man with generalized blisters was diagnosed as bullous pemphigoid on clinical, histopathological and direct immunofluorescence grounds. His free and total prostate specific antigen (PSA levels were high and histopathological examination of a prostate specimen revealed prostate adenocarcinoma. We present this rare case to discuss the possible association between bullous pemphigoid and prostate adenocarcinoma.

  12. Approaches to blister beetle control on millet: Botanical and biological agents, associational resistance, varietal resistance, and light and pheromone traps

    OpenAIRE

    Touré, G.K.; Edwards, R.C.; Traoré, S.H.

    1999-01-01

    Metadata only record Several approaches to management of insect pests of pearl millet (Pennisetum americum) were compared in on-farm trials in four villages in north central Mali. Light traps were used for assessment of insect pest populations, installed in millet and fallow fields, and illuminated each night from 5 August to 14 October 1997. The blister beetles Psalydolytta spp. and Mylabris spp. reached their highest numbers in late August and early September, the same time period as in ...

  13. Metamodeling and Optimization of a Blister Copper Two-Stage Production Process

    Science.gov (United States)

    Jarosz, Piotr; Kusiak, Jan; Małecki, Stanisław; Morkisz, Paweł; Oprocha, Piotr; Pietrucha, Wojciech; Sztangret, Łukasz

    2016-06-01

    It is often difficult to estimate parameters for a two-stage production process of blister copper (containing 99.4 wt.% of Cu metal) as well as those for most industrial processes with high accuracy, which leads to problems related to process modeling and control. The first objective of this study was to model flash smelting and converting of Cu matte stages using three different techniques: artificial neural networks, support vector machines, and random forests, which utilized noisy technological data. Subsequently, more advanced models were applied to optimize the entire process (which was the second goal of this research). The obtained optimal solution was a Pareto-optimal one because the process consisted of two stages, making the optimization problem a multi-criteria one. A sequential optimization strategy was employed, which aimed for optimal control parameters consecutively for both stages. The obtained optimal output parameters for the first smelting stage were used as input parameters for the second converting stage. Finally, a search for another optimal set of control parameters for the second stage of a Kennecott-Outokumpu process was performed. The optimization process was modeled using a Monte-Carlo method, and both modeling parameters and computed optimal solutions are discussed.

  14. Degradation of the blister agent sulfur mustard, bis(2-chloroethyl) sulfide, on concrete.

    Science.gov (United States)

    Brevett, Carol A S; Sumpter, Kenneth B; Wagner, George W; Rice, Jeffrey S

    2007-02-09

    The products formed from the degradation of the blister agent sulfur mustard [bis(2-chloroethyl) sulfide] on concrete were identified using gas chromatography with mass spectrometry detection (GC/MSD), (1)H NMR, 2D (1)H-(13)C NMR and (13)C solid state magic angle spinning (SSMAS) NMR. In situ and extraction experiments were performed. Sulfur mustard was detected in the in situ (13)C SSMAS samples for 12 weeks, whereas less than 5% of the sulfur mustard was detected in extracts from the concrete monoliths after 8 days. Sulfonium ions and (2-chloroethylthio)ethyl ether (T) were observed on the in situ samples after a period of 12 weeks, whereas vinyl species and bis(2-chloroethyl) sulfoxide were observed in the extracts of the concrete monoliths within 24h. The differences between the extraction and the SSMAS data indicated that the sulfur mustard existed in the concrete in a non-extractable form prior to its degradation. Extraction methods alone were not sufficient to identify the products; methods to identify the presence of non-extractable degradation products were also required.

  15. Degradation of the blister agent sulfur mustard, bis(2-chloroethyl) sulfide, on concrete

    Energy Technology Data Exchange (ETDEWEB)

    Brevett, Carol A.S. [GEO-CENTERS Operations, SAIC, Gunpowder Branch, P.O. Box 68, APG, MD 21010-0068 (United States)]. E-mail: carol.brevett@us.army.mil; Sumpter, Kenneth B. [CDR USA RDECOM, ATTN: AMSRD-ECB-RT-PD, 5183 Blackhawk Road, Aberdeen Proving Ground, MD 21010-5424 (United States)]. E-mail: kenneth.sumpter@us.army.mil; Wagner, George W. [CDR USA RDECOM, ATTN: AMSRD-ECB-RT-PD, 5183 Blackhawk Road, Aberdeen Proving Ground, MD 21010-5424 (United States)]. E-mail: george.wagner@us.army.mil; Rice, Jeffrey S. [CDR USA RDECOM, ATTN: AMSRD-ECB-RT-PD, 5183 Blackhawk Road, Aberdeen Proving Ground, MD 21010-5424 (United States)]. E-mail: jeffrey.rice@us.army.mil

    2007-02-09

    The products formed from the degradation of the blister agent sulfur mustard [bis(2-chloroethyl) sulfide] on concrete were identified using gas chromatography with mass spectrometry detection (GC/MSD), {sup 1}H NMR, 2D {sup 1}H-{sup 13}C NMR and {sup 13}C solid state magic angle spinning (SSMAS) NMR. In situ and extraction experiments were performed. Sulfur mustard was detected in the in situ {sup 13}C SSMAS samples for 12 weeks, whereas less than 5% of the sulfur mustard was detected in extracts from the concrete monoliths after 8 days. Sulfonium ions and (2-chloroethylthio)ethyl ether (T) were observed on the in situ samples after a period of 12 weeks, whereas vinyl species and bis(2-chloroethyl) sulfoxide were observed in the extracts of the concrete monoliths within 24 h. The differences between the extraction and the SSMAS data indicated that the sulfur mustard existed in the concrete in a non-extractable form prior to its degradation. Extraction methods alone were not sufficient to identify the products; methods to identify the presence of non-extractable degradation products were also required.

  16. Elevated homocysteine levels in suction-induced blister fluid of active vitiligo lesions.

    Science.gov (United States)

    Anbar, Tag; Zuel-Fakkar, Nehal Mohamed; Matta, Mary Fikry; Arbab, Mai Mohammed Ibrahim

    2016-01-01

    Vitiligo is the most prevalent acquired pigmentary disorder as a result of destruction of melanocytes. Several studies have reported increased serum levels of homocysteine (Hcy) in vitiligo patients which may be the result of decreased Vitamin B12 and folic acid levels. In addition, homocystinuria is associated with pigmentary dilution. On the other hand, other studies reported normal serum homocysteine levels. Our aim was to study the Hcy level in active vitiligo patients both in serum and in suction blister fluid obtained from the lesional skin. A total of 30 patients with active vitiligo of both sexes and 30 healthy volunteers were enrolled in this study. Sera from the blood and from lesional induced bullae were obtained from the patients and controls and were assayed for Hcy by enzyme-linked immunosorbent assay (ELISA). The collected data were analyzed by SPSS version 17. There were no significant differences in the serum levels of Hcy between patients and healthy controls, however, the increase in Hcy level was highly statistically significant in the patients' lesional induced bulla compared to the healthy controls. There was no significant difference in Hcy levels between males and females and between patients with negative or positive family histories of vitiligo. The presence of a high homocysteine level in active vitiligo lesions points to a local event occurring in this lesion, which is not reflected as an increase in the patient's serum level.

  17. A coupling model to simulate the dynamic process of blister-actuated nanosecond laser-induced forward transfer

    Science.gov (United States)

    Hu, Yongxiang; Cheng, Han; Xu, Jiaxi; Yao, Zhenqiang

    2017-08-01

    The modeling of laser-induced forward transfer process (LIFT) is helpful to understand and optimize its complex transfer process. In this work, a coupling model is developed to investigate the dynamic response of a thin polymer layer used as the release layer in the blister-actuated LIFT. In this model, the vapor pressure generated by nanosecond laser irradiation is computed through coupling with the transient vapor volume obtained from different step durations to simulate the dynamic blister formation. And the model is validated by experiments on polyimide film irradiated with different laser fluences, which is found to be capable of providing a consistent prediction of blister profiles under several laser conditions. The calibrated energy conversion ratios imply that laser pulse energy is mainly allocated for the heating and vaporizing of polymers, but increasing laser fluence can make this expense gradually saturated to allow more pulse energy to increase the vapor pressure. Transient pressure development from the coupling model is observed to increase rapidly within the pulse duration, but then to decrease because of vapor expansion. Forward velocity in axial direction is also observed to increase with laser fluence. The maximum velocity is possible to exceed the sound velocity under a high laser fluence. And the thin polymer layer is more preferred to obtain a high transfer velocity.

  18. Feasibility and safety of pipeline embolization device in patients with ruptured carotid blister aneurysms.

    Science.gov (United States)

    Yoon, Jang W; Siddiqui, Adnan H; Dumont, Travis M; Levy, Elad I; Hopkins, L Nelson; Lanzino, Giuseppe; Lopes, Demetrius K; Moftakhar, Roham; Billingsley, Joshua T; Welch, Babu G; Boulos, Alan S; Yamamoto, Junichi; Tawk, Rabih G; Ringer, Andrew J; Hanel, Ricardo A

    2014-10-01

    Treatment of internal carotid ruptured blister aneurysms (IC-RBA) presents many challenges to neurosurgeons because of the high propensity for rebleeding during intervention. The role of a Pipeline Embolization Device (PED) in the treatment of this challenging aneurysm subtype remains undefined despite theoretical advantages. To present a series of 11 patients treated with a PED and to discuss the management and results of this novel application of flow diverters. Medical records of patients who presented with IC-RBA from May 2011 to March 2013 were retrospectively reviewed at 6 institutions in the United States. All relevant data were independently compiled. A total of 12 IC-RBAs in 11 patients were treated during the study period. Nine (75%) were treated with a single PED; 1 was treated with 2 PEDs; 1 was treated with coils and 1 PED; and 1 was treated with coils and 2 PEDs. Three (27%) had major perioperative complications: middle cerebral artery territory infarction, vision loss, and death. Seven patients demonstrated complete obliteration of the aneurysm in postoperative imaging. Early clinical outcomes were favorable (modified Rankin Scale score, 0-2) in all 10 survivors. This study demonstrates the feasibility and safety of using the PED to treat IC-RBA with fair initial results. The proper introduction and management of antiplatelet regimen are key for successful results. Bleeding complications related to dual antiplatelet therapy were similar to those in previous studies of stent-assisted coiling for the same population. Larger cohort analysis is needed to define the precise role of flow diverters in the treatment of IC-RBA.

  19. False Blister Beetles and the Expansion of Gymnosperm-Insect Pollination Modes before Angiosperm Dominance.

    Science.gov (United States)

    Peris, David; Pérez-de la Fuente, Ricardo; Peñalver, Enrique; Delclòs, Xavier; Barrón, Eduardo; Labandeira, Conrad C

    2017-03-20

    During the mid-Cretaceous, angiosperms diversified from several nondiverse lineages to their current global domination [1], replacing earlier gymnosperm lineages [2]. Several hypotheses explain this extensive radiation [3], one of which involves proliferation of insect pollinator associations in the transition from gymnosperm to angiosperm dominance. However, most evidence supports gymnosperm-insect pollinator associations, buttressed by direct evidence of pollen on insect bodies, currently established for four groups: Thysanoptera (thrips), Neuroptera (lacewings), Diptera (flies), and now Coleoptera (beetles). Each group represents a distinctive pollination mode linked to a unique mouthpart type and feeding guild [4-9]. Extensive indirect evidence, based on specialized head and mouthpart morphology, is present for one of these pollinator types, the long-proboscid pollination mode [10], representing minimally ten family-level lineages of Neuroptera, Mecoptera (scorpionflies), and Diptera [8, 10, 11]. A recurring feature uniting these pollinator modes is host associations with ginkgoalean, cycad, conifer, and bennettitalean gymnosperms. Pollinator lineages bearing these pollination modes were categorized into four evolutionary cohorts during the 35-million-year-long angiosperm radiation, each defined by its host-plant associations (gymnosperm or angiosperm) and evolutionary pattern (extinction, continuation, or origination) during this interval [12]. Here, we provide the first direct evidence for one cohort, exemplified by the beetle Darwinylus marcosi, family Oedemeridae (false blister beetles), that had an earlier gymnosperm (most likely cycad) host association, later transitioning onto angiosperms [13]. This association constitutes one of four patterns explaining the plateau of family-level plant lineages generally and pollinating insects specifically during the mid-Cretaceous angiosperm radiation [12]. Published by Elsevier Ltd.

  20. Enhanced resistance to blister blight in transgenic tea (Camellia sinensis [L.] O. Kuntze) by overexpression of class I chitinase gene from potato (Solanum tuberosum).

    Science.gov (United States)

    Singh, H Ranjit; Deka, Manab; Das, Sudripta

    2015-07-01

    Tea is the second most consumed beverage in the world. A crop loss of up to 43 % has been reported due to blister blight disease of tea caused by a fungus, Exobasidium vexans. Thus, it directly affects the tea industry qualitatively and quantitatively. Solanum tuberosum class I chitinase gene (AF153195) is a plant pathogenesis-related gene. It was introduced into tea genome via Agrobacterium-mediated transformation with hygromycin phosphotransferase (hpt) gene conferring hygromycin resistance as plant selectable marker. A total of 41 hygromycin resistant plantlets were obtained, and PCR analysis established 12 plantlets confirming about the stable integration of transgene in the plant genome. Real-time PCR detected transgene expression in four transgenic plantlets (T28, C57, C9, and T31). Resistance to biotrophic fungal pathogen, E. vexans, was tested by detached leaf infection assay of greenhouse acclimated plantlets. An inhibitory activity against the fungal pathogen was evident from the detached leaves from the transformants compared with the control. Fungal lesion formed on control plantlet whereas the transgenic plantlets showed resistance to inoculated fungal pathogen by the formation of hypersensitivity reaction area. This result suggests that constitutive expression of the potato class I chitinase gene can be exploited to improve resistance to fungal pathogen, E. vexans, in economical perennial plantation crop like tea.

  1. Epidermolysis bullosa acquisita and anti-p200 pemphigoid as major subepidermal autoimmune bullous diseases diagnosed by floor binding on indirect immunofluorescence microscopy using human salt-split skin

    Directory of Open Access Journals (Sweden)

    Nupur Goyal

    2017-01-01

    Conclusion: In this study, we report three cases of anti-p200 pemphigoid from India. These cases, though indistinguishable clinically from bullous pemphigoid, revealed a floor-binding pattern on indirect immunofluorescence using salt-split skin.

  2. Reduced fibulin-2 contributes to loss of basement membrane integrity and skin blistering in mice lacking integrin α3β1 in the epidermis.

    Science.gov (United States)

    Longmate, Whitney M; Monichan, Ruby; Chu, Mon-Li; Tsuda, Takeshi; Mahoney, My G; DiPersio, C Michael

    2014-06-01

    Deficient epidermal adhesion is a hallmark of blistering skin disorders and chronic wounds, implicating integrins as potential therapeutic targets. Integrin α3β1, a major receptor in the epidermis for adhesion to laminin-332 (LN-332), has critical roles in basement membrane (BM) organization during skin development. In the current study we identify a role for α3β1 in promoting stability of nascent epidermal BMs through induction of fibulin-2, a matrix-associated protein that binds LN-332. We demonstrate that mice lacking α3β1 in the epidermis display ruptured BM beneath neo-epidermis of wounds, characterized by extensive blistering. This junctional blistering phenocopies defects reported in newborn α3-null mice, as well as in human patients with α3 gene mutations, indicating that the developmental role of α3β1 in BM organization is recapitulated during wound healing. Mice lacking epidermal α3β1 also have reduced fibulin-2 expression, and fibulin-2-null mice display perinatal skin blisters similar to those in α3β1-deficient mice. Interestingly, α3-null wound epidermis or keratinocytes also show impaired processing of the LN-332 γ2 chain, although this defect was independent of reduced fibulin-2 and did not appear to cause blistering. Our findings indicate a role for integrin α3β1 in BM stability through fibulin-2 induction, both in neonatal skin and in adult wounds.

  3. Theoretical Study on Synchronous Characterization of Surface and Interfacial Mechanical Properties of Thin-Film/Substrate Systems with Residual Stress Based on Pressure Blister Test Technique

    Directory of Open Access Journals (Sweden)

    Zhi-xin Yang

    2018-01-01

    Full Text Available In this study, based on the pressure blister test technique, a theoretical study on the synchronous characterization of surface and interfacial mechanical properties of thin-film/substrate systems with residual stress was presented, where the problem of axisymmetric deformation of a blistering film with initial stress was analytically solved and its closed-form solution was presented. The expressions to determine Poisson’s ratios, Young’s modulus, and residual stress of surface thin films were derived; the work done by the applied external load and the elastic energy stored in the blistering thin film were analyzed in detail and their expressions were derived; and the interfacial adhesion energy released per unit delamination area of thin-film/substrate (i.e., energy release rate was finally presented. The synchronous characterization technique presented here has theoretically made a big step forward, due to the consideration for the residual stress in surface thin films.

  4. Triglyceride blisters in lipid bilayers: implications for lipid droplet biogenesis and the mobile lipid signal in cancer cell membranes.

    Directory of Open Access Journals (Sweden)

    Himanshu Khandelia

    Full Text Available Triglycerides have a limited solubility, around 3%, in phosphatidylcholine lipid bilayers. Using millisecond-scale course grained molecular dynamics simulations, we show that the model lipid bilayer can accommodate a higher concentration of triolein (TO than earlier anticipated, by sequestering triolein molecules to the bilayer center in the form of a disordered, isotropic, mobile neutral lipid aggregate, at least 17 nm in diameter, which forms spontaneously, and remains stable on at least the microsecond time scale. The results give credence to the hotly debated existence of mobile neutral lipid aggregates of unknown function present in malignant cells, and to the early biogenesis of lipid droplets accommodated between the two leaflets of the endoplasmic reticulum membrane. The TO aggregates give the bilayer a blister-like appearance, and will hinder the formation of multi-lamellar phases in model, and possibly living membranes. The blisters will result in anomalous membrane probe partitioning, which should be accounted for in the interpretation of probe-related measurements.

  5. Recovery plan for Scots pine blister rust caused by Cronartium flaccidum (Alb. & Schwein.) G. Winter and Peridermium pini (Pers.) Lév. [syn. C. asclepiadeum (Willd.) Fr., Endocronartium pini (Pers.) Y. Hiratsuka

    Science.gov (United States)

    Brian W. Geils; Ned B. Klopfenstein; Mee-Sook Kim; Pauline Spaine; Bryce A. Richardson; Paul J. Zambino; Charles G. Shaw; James Walla; Russ Bulluck; Laura Redmond; Kent. Smith

    2009-01-01

    The sexually reproducing form of Scots pine blister rust, C. flaccidum, completes its life cycle alternating between pines of the subgenus Pinus and seed-plants of various families. Scots pine blister rust is also caused by a form of the rust that spreads directly from pine to pine and is named, Peridermium pini...

  6. A randomised controlled trial to compare the safety, effectiveness and cost-effectiveness of doxycycline (200 mg/day) with that of oral prednisolone (0.5 mg/kg/day) for initial treatment of bullous pemphigoid: the Bullous Pemphigoid Steroids and Tetracyclines (BLISTER) trial.

    Science.gov (United States)

    Chalmers, Joanne R; Wojnarowska, Fenella; Kirtschig, Gudula; Mason, James; Childs, Margaret; Whitham, Diane; Harman, Karen; Chapman, Anna; Walton, Shernaz; Schmidt, Enno; Godec, Thomas R; Nunn, Andrew J; Williams, Hywel C

    2017-03-01

    Bullous pemphigoid (BP) is an autoimmune blistering skin disorder with increased morbidity and mortality in the elderly. To evaluate the effectiveness, safety and cost-effectiveness of a strategy of initiating BP treatment with oral doxycycline or oral prednisolone. We hypothesised that starting treatment with doxycycline gives acceptable short-term blister control while conferring long-term safety advantages over starting treatment with oral prednisolone. Pragmatic multicentre two-armed parallel-group randomised controlled trial with an economic evaluation. A total of 54 dermatology secondary care centres in the UK and seven in Germany. Adults with BP [three or more blisters at two sites and positive direct and/or indirect immunofluorescence (immunoglobulin G and/or complement component 3 immunofluorescence at the dermal-epidermal junction)] and able to give informed consent. Participants were allocated using online randomisation to initial doxycycline treatment (200 mg/day) or prednisolone (0.5 mg/kg/day). Up to 30 g/week of potent topical corticosteroids was permitted for weeks 1-3. After 6 weeks, clinicians could switch treatments or alter the prednisolone dose as per normal practice. Primary outcomes: (1) the proportion of participants with three or fewer blisters at 6 weeks (investigator blinded) and (2) the proportion with severe, life-threatening and fatal treatment-related events at 52 weeks. A regression model was used in the analysis adjusting for baseline disease severity, age and Karnofsky score, with missing data imputed. Secondary outcomes included the effectiveness of blister control after 6 weeks, relapses, related adverse events and quality of life. The economic evaluation involved bivariate regression of costs and quality-adjusted life-years (QALYs) from a NHS perspective. In total, 132 patients were randomised to doxycycline and 121 to prednisolone. The mean patient age was 77.7 years and baseline severity was as follows: mild 31.6% (three

  7. Breakdown of major gene resistance to white pine blister rust in sugar pine at Mountain Home Demonstration State Forest: what are the implications?

    Science.gov (United States)

    Jr. Bohun B. Kinloch

    1996-01-01

    A virulent race of blister rust capable of neutralizing major gene resistance (MGR) in sugar pine made its first appearance nearly two decades ago at a test plantation of resistant sugar pines near Happy Camp, in northern California. Until this year (1996), it had not been found outside the very close neighborhood of this site. Its discovery last summer at Mountain...

  8. Ecology of whitebark pine populations in relation to white pine blister rust infection in subalpine forests of the Lake Tahoe Basin: Implications for restoration

    Science.gov (United States)

    Patricia E. Maloney; Detlev R. Vogler; Camille E. Jensen; Annette. Delfino Mix

    2012-01-01

    For over a century, white pine blister rust (WPBR), caused by the introduced fungal pathogen, Cronartium ribicola J.C. Fisch., has affected white pine (Subgenus Strobus) individuals, populations, and associated forest communities in North America. We surveyed eight populations of whitebark pine (Pinus albicaulis Engelm.) across a range of environmental conditions in...

  9. White pine blister rust at mountain home demonstration state forest: a case study of the epidemic and prospects for genetic control.

    Science.gov (United States)

    Bohun B. Kinloch; Dulitz Jr.

    1990-01-01

    The behavior of white pine blister rust at Mountain Home State Demonstration Forest and surrounding areas in the southern Sierra Nevada of California indicates that the epidemic has not yet stabilized and that the most likely prognosis is a pandemic on white pines in this region within the next few decades. The impact on sugar pines, from young regeneration to old...

  10. Effect of white pine blister rust (Cronartium ribicola) and rust-resistance breeding on genetic variation in western white pine Pinus monticola)

    Science.gov (United States)

    M. -S. Kim; S. J. Brunsfeld; G. I. McDonald; N. B. Klopfenstein

    2003-01-01

    Western white pine (Pinus monticola) is an economically and ecologically important species from western North America that has declined over the past several decades mainly due to the introduction of blister rust (Cronartium ribicola) and reduced opportunities for regeneration. Amplified fragment length polymorphism (AFLP) was used...

  11. Synoptic climatology of the long-distance dispersal of white pine blister rust II. Combination of surface and upper level conditions

    Science.gov (United States)

    K. L. Frank; B. W. Geils; L. S. Kalkstein; H. W. Thistle

    2008-01-01

    An invasive forest pathogen, Cronartium ribicola, white pine blister rust (WPBR), is believed to have arrived in the Sacramento Mountains of south-central New Mexico about 1970. Epidemiological and genetic evidence supports the hypothesis that introduction was the result of long-distance dispersal (LDD) by atmospheric transport from California. This...

  12. Antitumor effect of blister beetles: an ethno-medicinal practice in Karbi community and its experimental evaluation against a murine malignant tumor model.

    Science.gov (United States)

    Verma, Akalesh Kumar; Prasad, Surya Bali

    2013-07-30

    The blister beetles Epicauta hirticornis and Mylabris cichorii are used as a folk medicine by the Karbi tribe in Karbi Anglong district of Assam, India for the treatment of different human ailments, including cancer cases. It includes field survey related to zoo-therapeutic aspects of two blister beetles in Karbi community, isolation of bio-active compound and evaluation of its antitumor potential with possible mode of action against murine Ehrlich ascites carcinoma (EAC). The main bio-active compound of blister beetles was isolated from ethyl acetate extract and the structure was confirmed as cantharidin using NMR, IR, Mass and X-ray diffractometer. The effect of cantharidin on apoptosis, necrosis, autophagy and the apoptosis related signaling pathways were determined using different bioassays, including cell cycle analysis, mitochondrial membrane potential, western blot analysis of cytochrome c, caspases 9, 3/7 assays, and lactate dehydrogenase (LDH) assay. Cantharidin induced apoptosis, necrosis and autophagy cell death in EAC cells. The decrease in mitochondrial membrane potential was observed, which may help to release cytochrome c from mitochondria to cytosol. Cantharidin treatment caused up-regulation of caspases 9 and -3/7 and a decrease in LDH activity in EAC cells. The major bioactive compound of these blister beetles is cantharidin which induces severe apoptosis in EAC cells involving mitochondrial intrinsic pathway. Cantharidin-mediated inhibition of LDH activity may lead to short supply of NAD(+) and cut off energy and anabolic supply to cancer cells. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  13. Bullous Skin Diseases: Classical Types of Autoimmune Diseases

    Directory of Open Access Journals (Sweden)

    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.

  14. Pharmacological properties of blister beetles (Coleoptera: Meloidae) promoted their integration into the cultural heritage of native rural Spain as inferred by vernacular names diversity, traditions, and mitochondrial DNA.

    Science.gov (United States)

    Percino-Daniel, Nohemí; Buckley, David; García-París, Mario

    2013-06-03

    Beetles of the family Meloidae (blister beetles) are often reported in pharmacological literature because of their content of cantharidin. Cantharidin has a long history in human medicine and was commonly applied in the 19th and the early 20th centuries, although its use has been progressively abandoned since then. Contrary to most, even common, large species of Coleoptera, blister beetles of the genera Berberomeloe, Physomeloe and to a lesser extent Meloe, are usually recognized and often incorporated into local folk taxonomy by inhabitants of rural areas in Spain. To demonstrate the role that pharmacological properties of blister beetles must have played in their integration in the culture of early Iberian human societies, but also in the preservation of their identity until today, a rare case for Spanish insects. To achieve this purpose we document the diversity of vernacular names applied in rural areas of Spain, and we determine, using molecular data, the antiquity of the presence of two species of the better-known blister beetle in rural Spain, Berberomeloe majalis and Berberomeloe insignis. We try to document the extent of traditional knowledge of meloid beetles in rural areas by interviewing about 120 people from villages in central and southern Spain. We also use mitochondrial DNA sequences (Cytochrome Oxidase I and 16SrRNA) obtained from several populations of two species of the better known blister beetle in rural Spain, Berberomeloe majalis and Berberomeloe insignis, to determine whether these beetles were already present in the Iberian Peninsula when earlier ancient cultures were developing. Our results show that, based on mitochondrial DNA, blister beetles of the genus Berberomeloe were present in the Iberian Peninsula long before humans arrived, so ancient Iberian cultures were in contact with the same beetle species occurring now in rural areas. On the other hand, people interviewed in rural communities provided us with more than 28 different

  15. False-negative results in immunoblot assay of serum IgA antibodies reactive with the 180-kDa bullous pemphigoid antigen : the importance of primary incubation temperature

    NARCIS (Netherlands)

    Pas, HH; Kloosterhuis, GJ; De Jong, MCJM; Jonkman, MF

    2001-01-01

    Background Different subepidermal autoimmune blistering skin disorders are characterized by linear deposition of IgA, sometimes accompanied by linear IgG, along the epidermal basement membrane zone. Identification of the targeted autoantigen is usually attempted by immunoblotting. Although

  16. Effect of litter quality on foot pad dermatitis, hock burns and breast blisters in broiler breeders during the production period.

    Science.gov (United States)

    Kaukonen, Eija; Norring, Marianna; Valros, Anna

    2016-12-01

    Foot pad dermatitis and hock burn lesions are a form of contact dermatitis, a condition affecting skin areas in contact with unsuitable or irritating material. Contact dermatitis is a common problem, reducing the welfare of broilers, and is believed to also affect broiler breeders. However, there is very little research on contact dermatitis in breeders. This study followed the severity of foot pad lesions in broiler breeders throughout the production period. At slaughter the presence of hock burns and breast blisters was also determined. In addition, changes in litter condition over time and the impact of litter quality on foot pads were evaluated. The study was performed on 10 broiler breeder farms, including altogether 18 flocks. Foot pads of 100 hens per flock were assessed at the end of rearing period, three times during the production period, and at slaughter. Foot pad and hock lesions, as well as litter condition were scored on a 5-point scale. Litter quality was evaluated as pH, moisture and ammonia content. The condition of foot pads deteriorated towards slaughter age, with the occurrence of severe lesions reaching a maximum of 64% on average at slaughter. Hock lesions and breast blisters were rare. The litter layer became drier over time. Although poorer litter condition and wetness influenced foot pad health negatively, the effect on severe lesions was not significant. We also observed a negative effect on foot pad condition of larger slat areas. In conclusion, maintaining good litter quality alone is not enough to ensure healthy foot pads in broiler breeders.

  17. Cultured cells of white pine show genetic resistance to axenic blister rust hyphae.

    Science.gov (United States)

    Diner, A M; Mott, R L; Amerson, H V

    1984-04-27

    Hypersensitive resistance to axenically cultured Cronartium ribicola was displayed by subcultured callus of Pinus lambertiana. Cellular resistance to a destructive rust disease can now be studied at the macromolecular level through use of cloned cells of both host and pathogen in a system amenable to emerging recombinant DNA technology.

  18. Laminin isoforms in development and disease

    DEFF Research Database (Denmark)

    Schéele, Susanne; Nyström, Alexander; Durbeej, Madeleine

    2007-01-01

    . This provides for the specific basement membrane functions to stabilize cellular structures, to serve as effective physical barriers, and furthermore, to govern cell fate by inducing intracellular signalling cascades. Many different types of diseases involve basement membrances and laminins. Metastasizing solid...... blistering and kidney defects, respectively. This review summarizes recent progress concerning the molecular mechanisms of laminins in development and disease. The current knowledge may lead to clinical treatment of lamininopathies and may include stem-cell approaches as well as gene therapy....

  19. Study of the mechanical behavior of the hydride blister/rim structure in Zircaloy-4 using in-situ synchrotron X-ray diffraction

    Science.gov (United States)

    Lin, Jun-li; Han, Xiaochun; Heuser, Brent J.; Almer, Jonathan D.

    2016-04-01

    High-energy synchrotron X-ray diffraction was utilized to study the mechanical response of the f.c.c δ hydride phase, the intermetallic precipitation with hexagonal C14 lave phase and the α-Zr phase in the Zircaloy-4 materials with a hydride rim/blister structure near one surface of the material during in-situ uniaxial tension experiment at 200 °C. The f.c.c δ was the only hydride phase observed in the rim/blister structure. The conventional Rietveld refinement was applied to measure the macro-strain equivalent response of the three phases. Two regions were delineated in the applied load versus lattice strain measurement: a linear elastic strain region and region that exhibited load partitioning. Load partitioning was quantified by von Mises analysis. The three phases were observed to have similar elastic modulus at 200 °C.

  20. Flow cytometric analysis of skin blister fluid induced by mosquito bites in a patient with chronic active Epstein-Barr virus infection

    OpenAIRE

    Wada, Taizo; Yokoyama, Tadafumi; Nakagawa, Hiroyasu; Asai, Erika; Taga, Akiko; Sakakibara, Yasuhisa; Shibata, Fumie; Tone, Yumi; Shimizu, Masaki; Toma, Tomoko; Yachie, Akihiro

    2009-01-01

    In chronic active Epstein-Barr virus (EBV) infection (CAEBV), ectopic EBV infection has been described in T or natural killer (NK) cells. NK cell-type infection (NK-CAEBV) is characterized by large granular lymphocytosis, high IgE levels and unusual reactions to mosquito bites, including severe local skin reactions, fever and liver dysfunction. However, the mechanisms underlying these reactions remain undetermined. Herein, we describe a patient with NK-CAEBV whose blister fluid after mosquito...

  1. Non-invasive measurement of reepithelialization and microvascularity of suction-blister wounds with benchmarking to histology

    DEFF Research Database (Denmark)

    Larsen, Heidi Fhaer; Ahlström, Malin Glindvad; Gjerdrum, Lise Mette Rahbek

    2018-01-01

    blister (10 mm) was induced on each buttock in 30 healthy volunteers (15 females:15 males) and de-roofed on day 0. The wounds were randomized to daily treatment with 1.4% zinc sulfate shower gel (n = 20), placebo (n = 20) or control (n = 20). Digital photography coupled with planimetry, transepidermal...... groups but increased more with the placebo than with the zinc shower gel (p = 0.003) or the control treatment (p = 0.002) and correlated (rS = 0.313, p = 0.015) with the inflammatory response on day 4, as determined by histology. Coagulase-negative staphylococci were more common in wounds compared...... with skin (p = 0.002) and was reduced (p = 0.030) with zinc sulfate treatment. Planimetric analysis of digital wound images was not biased (p = 0.234) compared with histology, and TEWL measurements showed no correlation (rS = 0.052, p = 0.691) with epithelialization. Neoepidermal formation, determined...

  2. Successful treatment of a blood blister-like aneurysm of the internal carotid artery by trapping with a high-flow bypass.

    Science.gov (United States)

    Kawashima, Akitsugu; Okada, Yoshikazu; Kawamata, Takakazu; Onda, Hideaki; Kubo, Osami; Hori, Tomokatsu

    2008-07-01

    Treatment of blood blister-like aneurysms of the internal carotid artery (ICA) is difficult because the wall of the aneurysm is fragile and there is a high risk of rebleeding. There has been no consensus on the best way to treat these aneurysms. A 32-year-old woman presented with subarachnoid hemorrhage (SAH) caused by a ruptured blood blister-like aneurysm of the ICA. The site of the aneursym was clipped. Although angiography 1week after the operation showed that the aneurysm had been treated successfully, 3 weeks after the initial operation, the aneurysm was found to have recurred to the distal side. In a second operation, the aneurysm was successfully treated by trapping with a high-flow bypass. This case shows that clipping of the rupture site can be insufficient to treat blood blister-like aneurysm of the ICA causing SAH, even if the aneurysm seems to have resolved in follow-up angiographic studies. Trapping of the ICA with a bypass, if necessary a high-flow bypass, is recommended.

  3. Dystrophic epidermolysis bullosa: a review

    Directory of Open Access Journals (Sweden)

    Shinkuma S

    2015-05-01

    Full Text Available Satoru Shinkuma Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan Abstract: Dystrophic epidermolysis bullosa is a rare inherited blistering disorder caused by mutations in the COL7A1 gene encoding type VII collagen. The deficiency and/or dysfunction of type VII collagen leads to subepidermal blistering immediately below the lamina densa, resulting in mucocutaneous fragility and disease complications such as intractable ulcers, extensive scarring, malnutrition, and malignancy. The disease is usually diagnosed by immunofluorescence mapping and/or transmission electron microscopy and subsequently subclassified into one of 14 subtypes. This review provides practical knowledge on the disease, including new therapeutic strategies. Keywords: type VII collagen, anchoring fibril, subtypes, revertant mosaicism, treatment, gene therapy 

  4. Blister Test for Measurements of Adhesion and Adhesion Degradation of Organic Polymers on AA2024-T3

    Science.gov (United States)

    Rincon Troconis, Brendy Carolina

    A key parameter for the performance of corrosion protective coatings applied to metals is adhesion. Surface preparation prior to coating application is known to be critical, but there is a lack of understanding of what controls adhesion. Numerous techniques have been developed in the last decades to measure the adhesion strength of coatings to metals. Nonetheless, they are generally non-quantitative, non-reproducible, performed in dry conditions, or overestimate adhesion. In this study, a quantitative and reproducible technique, the Blister Test (BT), is used. The BT offers the ability to study the effects of a range of parameters, including the presence or absence of a wetting liquid, and simulates the stress situation in the coating/substrate interface. The effects of roughness and surface topography were studied by the BT and Optical Profilometry, using AA2024-T3 substrates coated with polyvinyl butyral (PVB). Random abrasion generated a surface with lower average roughness than aligned abrasion due to the continual cross abrasion of the grooves. The BT could discern the effects of different mechanical treatments. An adhesion strength indicator was defined and found to be a useful parameter. The effectiveness of standard adhesion techniques such as ASTM D4541 (Pull-off Test) and ASTM D3359 (Tape Test) was compared to the BT. Also, different attempts to measure adhesion and adhesion degradation of organic polymers to AA2024-T3 were tested. The pull-off test does not produce adhesive failure across the entire interface, while the tape test is a very qualitative technique and does not discern between the effects of different coating systems on the adhesion performance. The BT produces adhesive failure of the primer studied, is very reproducible, and is able to rank different coating systems. Therefore, it was found to be superior to the others. The approaches tested for adhesion degradation were not aggressive enough to have a measurable effect. The effects of

  5. A Novel Approach to the Quantitation of Coeluting Cantharidin and Deuterium Labelled Cantharidin in Blister Beetles (Coleop-tera: Meloidae

    Directory of Open Access Journals (Sweden)

    MR Nikbakhtzadeh

    2007-12-01

    Full Text Available Blister beetles (Coleoptera: Meloidae are the main natural source of cantharidin, but the compound titre is depended on several factors including, age, sex and mating status of the insects. In order to eliminate such uncertainty factors in physio¬logical and chemical studies deuterium labelled cantharidin (D2C with no natural abundance is normally introduced into the beetles' body to use it as a model for studying the cantharidin behaviour in vivo. Experiments were achieved on Mylabris quadripunctata (Col.: Meloidae from Southern France and the beetles were exposed to an artificial diet containing a de¬fined amount of D2C. On the other hand, because of the high similarity between the two compounds they cannot be well quantified by gas chromatography. In order to remove the burden, MRM technique was used for the first time which could successfully create well-defined cantharidin and D2C peaks and hence a precise measurement. MRM technique was exam¬ined using a GC-MS Varian Saturn which collected MS/MS data of more than one compound in the same time window of the chromatogram. It is especially useful when coeluting compounds have different parent ions, i.e. m/z 84 for D2C (coelut¬ing isotopically-labelled compound and m/z 82 for cantharidin (beetle-originated compound. Using the routine GC-MS runs, measurement accuracy may be significantly reduced because the D2C peak is covered by the cantharidin huge peak while MRM could reveal the two coincided peaks of cantharidin and D2C. Therefore MRM is hereby introduced as the method of choice to separate cantharidin from D2C with high sensitivity and thus provide a precise base of quantitation.

  6. Composição, proteólise, capacidade de derretimento e formação de "blisters" do queijo mussarela obtido pelos métodos tradicional e de ultrafiltração: composition, proteolysis, melting capacity and blisters formation Mozzarella by ultrafiltration

    Directory of Open Access Journals (Sweden)

    Patrícia D. Pizaia

    2003-12-01

    Full Text Available O objetivo deste trabalho foi comparar a composição, a proteólise, a capacidade de derretimento e a formação de "blisters" (bolhas em queijos tipo Mussarela fabricados com retentado de leite (MR de fator de concentração volumétrica (FCV de 2,34:1, com um queijo Mussarela padrão (MP fabricado com leite não ultrafiltrado. Foi realizado um ensaio de produção com 3 lotes de MR e um lote de MP. Determinou-se a composição do leite, retentado, soro, água de filagem e queijos e a proteólise, a capacidade de derretimento e a formação de "blisters" nos queijos com 7, 15, 30 e 60 dias de armazenamento refrigerado. MRs apresentaram maiores valores de pH e de porcentagem de cinzas e de proteína total e menores porcentagens de acidez titulável, gordura, gordura no extrato seco e sal quando comparadas a MP. Durante o tempo de estocagem, as MRs apresentaram menor proteólise e capacidade de derretimento, em todas as datas analisadas. A porcentagem de área coberta por 'blisters" na pizza e o diâmetro médio dos mesmos foram maiores na MP durante o primeiro mês de estocagem e depois ambos os tipos de queijos apresentaram comportamentos similares para estes 2 parâmetros.The objective of this research was to compare the composition, proteolysis, melting capacity and blisters formation in Mozzarella cheese manufactured with milk retentate (MR of a volumetric concentration factor (FCV of 2.34:1, with a standard Mozzarella cheese (MP manufactured with non ultrafiltrated milk. It was realized one production assay with 3 batches of MRs and one of MP. It was evaluated the milk, retentate, whey, stretching water and cheeses composition and the proteolysis, melting capacity and the blisters formation on cheeses with 7, 15, 30 and 60 days of refrigerates storage. MRs presented larger pH, ash and total protein contents and lower titratable acidity and fat, fat on dry matter and salt contents when compared to MP. Along the storage time the MRs

  7. Heat shock protein 90 inhibition: A potential double- or triple-edged sword in the treatment of mucous membrane pemphigoid.

    Science.gov (United States)

    Kasperkiewicz, Michael; Płatkowska, Anna; Zalewska, Anna; Zillikens, Detlef

    2015-10-01

    Mucous membrane pemphigoid (MMP) is a subtype of autoimmune subepidermal blistering diseases characterized by autoantibodies to structural components of the hemidesmosome primarily affecting mucous membranes. Inflammation-related progressive scarring can lead to serious complications, including blindness, and the disease may be associated with malignancy. Conventional immunosuppressive treatment is often insufficiently effective and limited due to side effects, warranting new therapeutic options ideally targeting both inflammation and extensively recalcitrant cicatrization. Heat shock protein 90 (Hsp90) is a cell stress-inducible chaperone required for the function of a large number of client proteins, and its pharmacological inhibition has proven to be effective and relatively safe in patients with cancer. Recent observations also suggest a promising role of Hsp90 as drug target in preclinical in vivo murine models of autoimmune diseases such as subepidermal bullous and fibrotic autoimmune disorders comprising epidermolysis bullosa acquisita and systemic sclerosis, respectively, which exhibit some pathophysiological features reminiscent of MMP. This article thus hypothesizes that Hsp90 blockade could represent a double-edged sword in MMP treatment by targeting pathogenic factors of inflammatory blister and fibrosis formation. Moreover, Hsp90 inhibitors could even be proclaimed as a triple-edged sword in case of an underlying malignancy. Future studies investigating the role of Hsp90 in MMP are needed to clarify whether Hsp90 inhibition could become a novel treatment approach for patients with this potentially devastating disease. Copyright © 2015 Elsevier Ltd. All rights reserved.

  8. Blisters, Calluses, and Corns

    Science.gov (United States)

    ... all the time. They usually form on the ball of the foot. (The ball is the roundish part on the bottom of ... special doughnut-shaped pads that let the corn fit right into the hole in the middle to ...

  9. Differentiating antiepiligrin cicatricial pemphigoid from epidermolysis bullosa acquisita by indirect immunofluorescence of skin substrates lacking Type VII collagen or laminin 332: a case report and review of literature

    Directory of Open Access Journals (Sweden)

    Chih-Pin Chen

    2011-06-01

    Full Text Available Antiepiligrin cicatricial pemphigoid (AECP is a chronic autoimmune subepidermal blistering disease characterized by clinical features of cicatricial pemphigoid and circulating IgG antibasement membrane autoantibodies directed against laminin 332. There is growing evidence of an increased relative risk for solid cancers and lymphomas in AECP patients, especially in the 1st year after the onset of blisters. However, it is difficult to distinguish patients with initially skin-predominant AECP from similar findings of epidermolysis bullosa acquisita merely based on clinical, histopathologic, and immuno-pathologic examinations. This is a report on a case of AECP confirmed by indirect immunofluorescence of type VII collagen- and laminin 332-deficient skin as substrates to differentiate it from epidermolysis bullosa acquisita.

  10. Interventional treatments for Hailey-Hailey disease.

    Science.gov (United States)

    Farahnik, Benjamin; Blattner, Collin M; Mortazie, Michael B; Perry, Benjamin M; Lear, William; Elston, Dirk M

    2017-03-01

    Hailey-Hailey disease or familial benign chronic pemphigus is a rare blistering dermatosis that is characterized by recurrent erythematous plaques with a predilection for the skin folds. For extensive Hailey-Hailey disease that is recalcitrant to conventional therapy, laser ablation, photodynamic therapy, electron beam radiotherapy, botulinum toxin type A, dermabrasion, glycopyrrolate, and afamelanotide have been reported as useful treatments, but comparative trials are lacking. This review discusses the various treatment modalities for Hailey-Hailey disease and a summary of the evidence for the most recommended treatments. Copyright © 2016 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  11. High-flow bypass and wrap-clipping for ruptured blood blister-like aneurysm of the internal carotid artery using intraoperative monitoring of cerebral hemodynamics.

    Science.gov (United States)

    Kubo, Yoshitaka; Koji, Takahiro; Yoshida, Kenji; Saito, Hideo; Ogawa, Akira; Ogasawara, Kuniaki

    2015-01-01

    Aneurysms at non-branching sites in the supraclinoid internal carotid artery (ICA) can be classified as "blood blister-like aneurysms" (BBAs), which have blood blister-like configurations and fragile walls. While surgical treatment for the BBA in the acute stage is recommended, the optimal surgical procedure remains controversial. In the study reported here, we describe the case of a 37-year-old woman with a ruptured BBA in the ophthalmic segment of the right ICA who underwent wrap-clipping with external carotid artery-internal carotid artery bypass by intraoperative estimation of the measurement of cortical cerebral blood flow (CoBF) using a thermal diffusion flow probe. Trapping of the ICA in the acute stage of subarachnoid hemorrhage may result in ischemic complications secondary to hemodynamic hypoperfusion or occlusion of the perforating artery, and/or delayed vasospasm, even with concomitant bypass surgery. We believe that it is important to perform scheduled external carotid artery-internal carotid artery bypass before trapping of the ICA in patients with a ruptured BBA in the acute stage of subarachnoid hemorrhage and to perform wrap-clipping rather than trapping. This would provide much more CoBF if a reduction of CoBF occurs after trapping occlusion of the ICA including a ruptured BBA according to intraoperative CoBF monitoring. As far as we are aware, the case reported here is the first report on high-flow bypass and wrap-clipping for a ruptured BBA of the ICA using intraoperative monitoring of cerebral hemodynamics.

  12. High-flow bypass and wrap-clipping for ruptured blood blister-like aneurysm of the internal carotid artery using intraoperative monitoring of cerebral hemodynamics

    Directory of Open Access Journals (Sweden)

    Kubo Y

    2015-06-01

    Full Text Available Yoshitaka Kubo, Takahiro Koji, Kenji Yoshida, Hideo Saito, Akira Ogawa, Kuniaki Ogasawara Department of Neurosurgery, Iwate Medical University, Morioka, Japan Abstract: Aneurysms at non-branching sites in the supraclinoid internal carotid artery (ICA can be classified as “blood blister-like aneurysms” (BBAs, which have blood blister-like configurations and fragile walls. While surgical treatment for the BBA in the acute stage is recommended, the optimal surgical procedure remains controversial. In the study reported here, we describe the case of a 37-year-old woman with a ruptured BBA in the ophthalmic segment of the right ICA who underwent wrap-clipping with external carotid artery–internal carotid artery bypass by intraoperative estimation of the measurement of cortical cerebral blood flow (CoBF using a thermal diffusion flow probe. Trapping of the ICA in the acute stage of subarachnoid hemorrhage may result in ischemic complications secondary to hemodynamic hypoperfusion or occlusion of the perforating artery, and/or delayed vasospasm, even with concomitant bypass surgery. We believe that it is important to perform scheduled external carotid artery–internal carotid artery bypass before trapping of the ICA in patients with a ruptured BBA in the acute stage of subarachnoid hemorrhage and to perform wrap-clipping rather than trapping. This would provide much more CoBF if a reduction of CoBF occurs after trapping occlusion of the ICA including a ruptured BBA according to intraoperative CoBF monitoring. As far as we are aware, the case reported here is the first report on high-flow bypass and wrap-clipping for a ruptured BBA of the ICA using intraoperative monitoring of cerebral hemodynamics. Keywords: surgery, cortical blood flow, external carotid artery–internal carotid artery bypass, subarachnoid hemorrhage

  13. Is Dental Implantation Indicated in Patients with Oral Mucosal Diseases

    OpenAIRE

    Kalogirou Eleni-Marina; Sklavounou Alexandra

    2017-01-01

    Background/Aim: Dental implants are a reliable treatment choice for rehabilitation of healthy patients as well as subjects with several systemic conditions. Patients with oral mucosal diseases often exhibit oral mucosal fragility and dryness, erosions, blisters, ulcers or microstomia that complicate the use of removable dentures and emphasize the need for dental implants. The aim of the current study is to review the pertinent literature regarding the dental implantation prospects for patient...

  14. STUDY OF THE MOLECULAR ALTERATIONS AND TREATMENT OF HAILEY-HAILEY DISEASE

    OpenAIRE

    Manca, Sonia

    2014-01-01

    Hailey-Hailey Disease (HHD) is a chronic, recurrent blistering rare disorder, clinically characterized by erosions occurring primarily in intertriginous regions and histologically by suprabasal cutaneous cells separation (acantholysis). Despite a strong relationship between mutations in ATP2C1 gene, encoding for hSPCA1 calcium pump, manifestations of the disease occurs with high variability between patients. In our previous studies we used primary keratinocytes derived from les...

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

    OpenAIRE

    Licarete Emilia; Ganz Susanne; Recknagel Martin J; Di Zenzo Giovanni; Hashimoto Takashi; Hertl Michael; Zambruno Giovanna; Hundorfean Gheorghe; Mudter Jonas; Neurath Markus F; Bruckner-Tuderman Leena; Sitaru Cassian

    2012-01-01

    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 wetl...

  16. DISEASES

    DEFF Research Database (Denmark)

    Pletscher-Frankild, Sune; Pallejà, Albert; Tsafou, Kalliopi

    2015-01-01

    Text mining is a flexible technology that can be applied to numerous different tasks in biology and medicine. We present a system for extracting disease-gene associations from biomedical abstracts. The system consists of a highly efficient dictionary-based tagger for named entity recognition...... of human genes and diseases, which we combine with a scoring scheme that takes into account co-occurrences both within and between sentences. We show that this approach is able to extract half of all manually curated associations with a false positive rate of only 0.16%. Nonetheless, text mining should...... not stand alone, but be combined with other types of evidence. For this reason, we have developed the DISEASES resource, which integrates the results from text mining with manually curated disease-gene associations, cancer mutation data, and genome-wide association studies from existing databases...

  17. Investigations on a Flaps-Like Disease of Cattle (’Benign Flaps,’ Stomatitis Papulosa Bovis Specifica) (Untersuchungen uber eine Maulseucheahnliche Erkrankung des Rindes (’Gutartige Maulseuche,’ Stomatitis Papulosa Bovis Specifica))

    Science.gov (United States)

    Stomatitis papulosa infections of cattle can be differentiated from so-called sporadic aphtha and from aphthous fever. Stomatitis papulosa can be...distinguished from both diseases by the absence of blisters and pustules. Moreover, it can also be differentiated from aphthous fever in that it involves only the oral cavity and not the skin and hooves.

  18. Exfoliation of oxide in bar: estimation of the geometry of HZ{sub r} blister; Exfoliacion de oxido en barra: estimacion de la geometria de la ampolla de HZ{sub r}

    Energy Technology Data Exchange (ETDEWEB)

    Ramos Calvo, A.; Munoz-Reja, C.; Nicolas Garcia, E.

    2013-07-01

    This paper presents a method to model the formation of a blister of HZ{sub r}. The method consists of two distinct parts. First is a study of the temperature distribution in the pod different configurations of loss of thickness of oxide fuel to define the likely distribution of hydrides geometry. Second is an engineering approach to solving the equation of precipitation of hydrides in the sheath.

  19. Variable levels of apoptotic signal-associated cytokines in the disease course of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis.

    Science.gov (United States)

    Yang, Yongsheng; Li, Feng; Du, Juan; Shen, Yanyun; Lin, Jinran; Zhu, Xiaohua; Luo, Xiaoqun; Liang, Jun; Xu, Jinhua

    2017-08-01

    Keratinocyte death is a hallmark of Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). Apoptotic signal-associated cytokines, such as TNF-α, sFasL, granulysin, sTRAIL and IFN-γ have been reported to participate in keratinocyte apoptosis. However, their levels are variable, which hampers the elucidation of the role of these cytokines. We sought to determine whether cytokine levels vary with disease course. The serum cytokine levels of 24 patients and blister fluid of 10 were analysed by enzyme-linked immunosorbent assay on the first day of their admission to hospital and were evaluated at different time points in the disease course. Meanwhile, surface markers (CD3, CD4, CD8, CD1a, CD14, CD16+56 and CD68) of blister fluid cells were measured by flow cytometry. The concentrations of all cytokines in the serum and blister fluid were higher than those in the controls and were more elevated in the blister fluid than in the serum. Moreover, sTRAIL, IFN-γ and TNF-α quantities were relatively stable, while those of sFasL and granulysin decreased rapidly in the disease course. On the first day, CD8+ T and natural killer cells were predominant in the blister fluid but their relative percentage diminished gradually, while that of CD14+ cells increased. Our study confirmed there are high but variable levels of these cytokines in SJS/TEN, especially in the early phase and different tendencies are manifested in the disease course. © 2016 The Australasian College of Dermatologists.

  20. Flow cytometric analysis of skin blister fluid induced by mosquito bites in a patient with chronic active Epstein-Barr virus infection.

    Science.gov (United States)

    Wada, Taizo; Yokoyama, Tadafumi; Nakagawa, Hiroyasu; Asai, Erika; Toga, Akiko; Sakakibara, Yasuhisa; Shibata, Fumie; Tone, Yumi; Shimizu, Masaki; Toma, Tomoko; Yachie, Akihiro

    2009-12-01

    In chronic active Epstein-Barr virus (EBV) infection (CAEBV), ectopic EBV infection has been described in T or natural killer (NK) cells. NK cell-type infection (NK-CAEBV) is characterized by large granular lymphocytosis, high IgE levels and unusual reactions to mosquito bites, including severe local skin reactions, fever and liver dysfunction. However, the mechanisms underlying these reactions remain undetermined. Herein, we describe a patient with NK-CAEBV whose blister fluid after mosquito bites was analyzed. The patient exhibited significant increases in the percentage of CD56(+) NK cells in the fluid compared with a simple mosquito allergy, in which the majority of infiltrated cells were CD203c(+) cells, indicating basophils and/or mast cells. His fluid also contained CD203c(+) cells, and his circulating basophils were activated by mosquito extracts in vitro. These results suggest that CD203c(+) cells as well as NK cells may play pathogenic roles in the severe skin reactions to mosquito bites in NK-CAEBV.

  1. High-separation efficiency micro-fabricated multi-capillary gas chromatographic columns for simulants of the nerve agents and blister agents

    Science.gov (United States)

    Li, Yi; Du, Xiaosong; Wang, Yang; Tai, Huiling; Qiu, Dong; Lin, Qinghao; Jiang, Yadong

    2014-05-01

    To achieve both high speed and separation efficiency in the separation of a mixture of nerve and blister agent simulants, a high-aspect-ratio micro-fabricated multi-capillary column (MCC, a 50-cm-long, 450-μm-deep, and 60-μm-wide four-capillary column) was fabricated by the application of the microelectromechanical system (MEMS) techniques. Mixtures of chemical warfare agent (CWA) simulants - dimethyl methylphosphonate (DMMP), triethyl phosphate (TEP), and methyl salicylate - were used as samples. The fabricated MCC allowed for the separation of all the components of the gaseous mixture within 24 s, even when the difference in boiling point was 4°C, as in the case of TEP and methyl salicylate. Furthermore, interfering agents - dichloromethane, ethanol, and toluene - were also included in the subsequent gaseous mixture samples. The boiling point of these six components ranged from 78°C to 219°C. All six components were clearly separated within 70 s. This study is the first to report the clear separation of gas mixtures of components with close boiling points. The column efficiency was experimentally determined to be 12,810 plates/m.

  2. De Novo Transcriptome and Expression Profile Analysis to Reveal Genes and Pathways Potentially Involved in Cantharidin Biosynthesis in the Blister Beetle Mylabris cichorii.

    Directory of Open Access Journals (Sweden)

    Yi Huang

    Full Text Available The dried body of Mylabris cichorii is well-known Chinese traditional medicine. The sesquiterpenoid cantharidin, which is secreted mostly by adult male beetles, has recently been used as an anti-cancer drug. However, little is known about the mechanisms of cantharidin biosynthesis. Furthermore, there is currently no genomic or transcriptomic information for M. cichorii. In this study, we performed de novo assembly transcriptome of M. cichorii using the Illumina Hiseq2000. A single run produced 9.19 Gb of clean nucleotides comprising 29,247 sequences, including 23,739 annotated sequences (about 81%. We also constructed two expression profile libraries (20-25 day-old adult males and 20-25 day-old adult females and discovered 2,465 significantly differentially-expressed genes. Putative genes and pathways involved in the biosynthesis of cantharidin were then characterized. We also found that cantharidin biosynthesis in M. cichorii might only occur via the mevalonate (MVA pathway, not via the methylerythritol 4-phosphate/deoxyxylulose 5-phosphate (MEP/DOXP pathway or a mixture of these. Besides, we considered that cantharidin biosynthesis might be related to the juvenile hormone (JH biosynthesis or degradation. The results of transcriptome and expression profiling analysis provide a comprehensive sequence resource for M. cichorii that could facilitate the in-depth study of candidate genes and pathways involved in cantharidin biosynthesis, and may thus help to improve our understanding of the mechanisms of cantharidin biosynthesis in blister beetles.

  3. Cross-reactivity of autoantibodies from patients with epidermolysis bullosa acquisita with murine collagen VII.

    Science.gov (United States)

    Csorba, Kinga; Sesarman, Alina; Oswald, Eva; Feldrihan, Vasile; Fritsch, Anja; Hashimoto, Takashi; Sitaru, Cassian

    2010-04-01

    The pathomechanism of antibody-mediated tissue damage in autoimmune diseases can be best studied in experimental models by passively transferring specific autoantibodies into animals. The reproduction of the disease in animals depends on several factors, including the cross-reactivity of patient autoantibodies with the animal tissue. Here, we show that autoantibodies from patients with epidermolysis bullosa acquisita (EBA), a subepidermal autoimmune blistering disease, recognize multiple epitopes on murine collagen VII. Indirect immunofluorescence microscopy revealed that EBA patients' IgG cross-reacts with mouse skin. Overlapping, recombinant fragments of murine collagen VII were used to characterize the reactivity of EBA sera and to map the epitopes on the murine antigen by ELISA and immunoblotting. The patients' autoantibody binding to murine collagen VII triggered pathogenic events as demonstrated by a complement fixing and an ex vivo granulocyte-dependent dermal-epidermal separation assay. These findings should greatly facilitate the development of improved disease models and novel therapeutic strategies.

  4. Gene editing for skin diseases: designer nucleases as tools for gene therapy of skin fragility disorders.

    Science.gov (United States)

    March, Oliver P; Reichelt, Julia; Koller, Ulrich

    2017-03-07

    What is the topic of this review? This review concerns current gene editing strategies for blistering skin diseases with respect to individual genetic constellations and distinct conditions. What advances does it highlight? Specificity and safety dominate the discussion of gene editing applications for gene therapy, where a number of tools are implemented. Recent developments in this rapidly progressing field pose further questions regarding which tool is best suited for each particular use. The current treatment of inherited blistering skin diseases, such as epidermolysis bullosa (EB), is largely restricted to wound care and pain management. More effective therapeutic strategies are urgently required, and targeting the genetic basis of these severe diseases is now within reach. Here, we describe current gene editing tools and their potential to correct gene function in monogenetic blistering skin diseases. We present the features of the most frequently used gene editing techniques, transcription activator-like effector nuclease (TALEN) and clustered regularly interspaced short palindromic repeats/CRISPR-associated protein 9 (CRISPR/Cas9), determining their preferential application for specific genetic conditions, including the type of mutational inheritance, the targeting site within the gene or the possibility to target the mutation specifically. Both tools have traits beneficial in specific situations. Promising developments in the field engender gene editing as a potentially powerful therapeutic option for future clinical applications. © 2017 The Authors. Experimental Physiology © 2017 The Physiological Society.

  5. Autoimmune bullous skin diseases. Part 1: Clinical manifestations.

    Science.gov (United States)

    Kneisel, Andrea; Hertl, Michael

    2011-10-01

    Autoimmune bullous skin diseases are characterized by autoantibodies against adhesion molecules of the skin. Pemphigus is a disorder with an intraepidermal loss of adhesion and is characterized by fragile blisters and erosions. Pemphigus vulgaris often shows extensive lesions of the oral mucosa, while pemphigus foliaceus is commonly restricted to cutaneous involvement with puff pastry-like scale formation. Paraneoplastic pemphigus is obligatorily associated with malignancies and often presents as hemorrhagic stomatitis with multiforme-like exanthems. IgA pemphigus typically presents with pustules and annular plaques but not with mucosal involvement. The clinical spectrum of the pemphigoids 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 and is more heterogeneous in adulthood. Classical epidermolysis bullosa acquisita shows extensive skin fragility. Dermatitis herpetiformis is associated with gluten-sensitive enteropathy and manifests clinically with severe itching and papulovesicles on the extensor surfaces of the extremities and the lumbosacral area. The intention of the review is to demonstrate the heterogeneous clinical spectrum of autoimmune bullous disorders. © The Authors • Journal compilation © Blackwell Verlag GmbH, Berlin.

  6. Oral mucosal manifestations of autoimmune skin diseases.

    Science.gov (United States)

    Mustafa, Mayson B; Porter, Stephen R; Smoller, Bruce R; Sitaru, Cassian

    2015-10-01

    A group of autoimmune diseases is characterised by autoantibodies against epithelial adhesion structures and/or tissue-tropic lymphocytes driving inflammatory processes resulting in specific pathology at the mucosal surfaces and the skin. The most frequent site of mucosal involvement in autoimmune diseases is the oral cavity. Broadly, these diseases include conditions affecting the cell-cell adhesion causing intra-epithelial blistering and those where autoantibodies or infiltration lymphocytes cause a loss of cell-matrix adhesion or interface inflammation. Clinically, patients present with blistering, erosions and ulcers that may affect the skin as well as further mucosal surfaces of the eyes, nose and genitalia. While the autoimmune disease may be suspected based on clinical manifestations, demonstration of tissue-bound and circulating autoantibodies, or lymphocytic infiltrates, by various methods including histological examination, direct and indirect immunofluorescence microscopy, immunoblotting and quantitative immunoassay is a prerequisite for definitive diagnosis. Given the frequency of oral involvement and the fact that oral mucosa is the initially affected site in many cases, the informed practitioner should be well acquainted with diagnostic and therapeutic aspects of autoimmune dermatosis with oral involvement. This paper reviews the pathogenesis and clinical presentation of these conditions in the oral cavity with a specific emphasis on their differential diagnosis and current management approaches. Copyright © 2015 Elsevier B.V. All rights reserved.

  7. disease

    African Journals Online (AJOL)

    Objective. To undertake an economic evaluation of the administration and monitoring costs of the two different forms of heparin in patients with unstable coronary artery disease (DCAD). Study design. Equivalent efficacy was found for low- molecular-weight heparin (LMWH) and for unfraction- ated heparin (UFH) in the ...

  8. Keratinocyte-targeted expression of human laminin γ2 rescues skin blistering and early lethality of laminin γ2 deficient mice.

    Directory of Open Access Journals (Sweden)

    Tracy L Adair-Kirk

    Full Text Available Laminin-332 is a heterotrimeric basement membrane component comprised of the α3, ß3, and γ2 laminin chains. Laminin-332 modulates epithelial cell processes, such as adhesion, migration, and differentiation and is prominent in many embryonic and adult tissues. In skin, laminin-332 is secreted by keratinocytes and is a key component of hemidesmosomes connecting the keratinocytes to the underlying dermis. In mice, lack of expression of any of the three Laminin-332 chains result in impaired anchorage and detachment of the epidermis, similar to that seen in human junctional epidermolysis bullosa, and death occurs within a few days after birth. To bypass the early lethality of laminin-332 deficiency caused by the knockout of the mouse laminin γ2 chain, we expressed a dox-controllable human laminin γ2 transgene under a keratinocyte-specific promoter on the laminin γ2 (Lamc2 knockout background. These mice appear similar to their wild-type littermates, do not develop skin blisters, are fertile, and survive >1.5 years. Immunofluorescence analyses of the skin showed that human laminin γ2 colocalized with mouse laminin α3 and ß3 in the basement membrane zone underlying the epidermis. Furthermore, the presence of "humanized" laminin-332 in the epidermal basement membrane zone rescued the alterations in the deposition of hemidesmosomal components, such as plectin, collagen type XVII/BP180, and integrin α6 and ß4 chains, seen in conventional Lamc2 knockout mice, leading to restored formation of hemidesmosomes. These mice will be a valuable tool for studies of organs deficient in laminin-332 and the role of laminin-332 in skin, including wound healing.

  9. Clinical evaluation of a multiparametric ELISA as a rapid tool for routinely diagnosing IgG-mediated autoimmune blistering dermatoses in ethnic Slavs.

    Science.gov (United States)

    Gornowicz-Porowska, Justyna; Seraszek-Jaros, Agnieszka; Bowszyc-Dmochowska, Monika; Bartkiewicz, Paweł; Kaczmarek, Elżbieta; Dmochowski, Marian

    2017-09-26

    Technical innovation of autoimmune blistering dermatoses (ABDs) diagnosis aimed at multiplex approach. Two multiparametric ELISA tests are commercially available for ABDs serology. The aim was to compare diagnostic accuracy of multiparametric and monospecific ELISAs and to examine the diagnostic value/agreement of multivariant ELISA in compliance with traditional diagnostic setup for ABDs. In total, 128 sera from suspected ABDs patients were studied (27 sera in order to compare ELISAs). Multivariant ELISA (detection of IgG against desmoglein 1 and 3 - DSG1/3; BP180, BP230, envoplakin, type VII collagen), monovariant ELISA, and statistical analysis were performed. With the use of sera from patients with suspected ABDs, the multiparametric ELISA yield an agreement of 84% with traditional stepwise diagnostics. Multivariant ELISA with BP180 and BP230 showed 87.5% and 80% sensitivity, 87.5% and 91% specificity, 87.5% reliability as well as 87.5% and 80% positive predictive value, 87.5% and 91% negative predictive value, respectively, in relation to monospecific ELISA. Multivariant ELISA with DSG1 and DSG3 showed 50% and 80% sensitivity, 100% and 80% specificity, 85% and 80% reliability as well as 100% and 57% positive predictive value, 82% and 92% negative predictive value, respectively, in relation to monospecific ELISA. A better rate of agreement was observed among ELISA systems with BP180 and BP230, than with ELISA systems with DSG1 and DSG3. Multivariant ELISA test combined with clinical examinations and DIF is recommended as a minimal approach to diagnosing ABDs in ethnic Slavs. © 2017 Wiley Periodicals, Inc.

  10. Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Quentin Binet

    2017-01-01

    Full Text Available Background. Acquired hemophilia A (AHA is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening. Case Study. We report a case of AHA associated with bullous pemphigoid (BP, a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association. Following treatment for less than 3 months consisting of methylprednisolone at decreasing dose levels along with four courses of rituximab (monoclonal antibody directed against the CD20 protein, AHA was completely cured and BP well-controlled. Conclusions. This report illustrates a rare association of AHA and BP, supporting the possibility of eradicating the inhibitor with a well-conducted short-term treatment.

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

    Science.gov (United States)

    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.

  12. Adherence to a flexible extended regimen for oral hormonal contraception provided in a blister packaging compared with an adherence-supporting digital tablet dispenser: historical comparison of data from two clinical studies

    Directory of Open Access Journals (Sweden)

    Elliesen J

    2016-08-01

    Full Text Available Jörg Elliesen, Dietmar Trummer Bayer Pharma AG, Berlin, Germany Background: The Clyk™ digital pill dispenser helps ensure correct and consistent administration of a flexible extended regimen of the combined oral contraceptive, ethinylestradiol (EE 20 µg/drospirenone 3 mg (EE/drospirenoneFlex; YAZ® Flex, guiding users through the intake cycle and 4-day pill break and providing visible and acoustic daily reminders when pill intake is due. A study showed that the audible alarm function of the dispenser could help reduce the number of missed pills, but it lacked an appropriate “non-dispenser” group for a meaningful assessment of the impact of the dispenser on adherence. This study indirectly assessed the overall effect of the digital dispenser on adherence by comparing data from a treatment with standard blister packaging.Materials and methods: One-year adherence data were compared from two similarly designed, Phase III, open-label, randomized trials of EE/drospirenoneFlex. In study 1, women used diary cards to record adherence with EE/drospirenoneFlex dispensed in blister packs (n=640, and in study 2 the dispenser was used with the alarm activated (n=250 or deactivated (n=248 in addition to using diary cards.Results: A mean (±SD of 4.3 (±4.24 missed pills over 1 year were recorded in diary cards among women who dispensed their pills from the blister packages (study 1 compared with 1.0 (±2.4 recorded by the alarm-activated dispenser (study 2. In study 2, a mean of 1.9 (±4.2 missed pills were reported in the diaries over 1 year compared with 4.4 (±9.1 from automatic recording by the dispenser (both arms of study 2, indicating underreporting of missed pills in diary cards vs the digital dispenser. Adjusting for this rate of underreporting, an estimated mean of ten pills were missed over 1 year by women using EE/drospirenoneFlex in blister packs, or ten times more than with the digital dispenser with activated acoustic alarm

  13. Adherence to a flexible extended regimen for oral hormonal contraception provided in blister packaging compared with an adherence-supporting digital tablet dispenser: historical comparison of data from two clinical studies.

    Science.gov (United States)

    Elliesen, Jörg; Trummer, Dietmar

    2016-01-01

    The Clyk™ digital pill dispenser helps ensure correct and consistent administration of a flexible extended regimen of the combined oral contraceptive, ethinylestradiol (EE) 20 μg/drospirenone 3 mg (EE/drospirenone ; YAZ(®) Flex Flex), guiding users through the intake cycle and 4-day pill break and providing visible and acoustic daily reminders when pill intake is due. A study showed that the audible alarm function of the dispenser could help reduce the number of missed pills, but it lacked an appropriate "non-dispenser" group for a meaningful assessment of the impact of the dispenser on adherence. This study indirectly assessed the overall effect of the digital dispenser on adherence by comparing data from a treatment with standard blister packaging. One-year adherence data were compared from two similarly designed, Phase III, open-label, randomized trials of EE/drospirenoneFlex. In study 1, women used diary cards to record adherence with EE/drospirenoneFlex dispensed in blister packs (n=640), and in study 2 the dispenser was used with the alarm activated (n=250) or deactivated (n=248) in addition to using diary cards. A mean (±SD) of 4.3 (±4.24) missed pills over 1 year were recorded in diary cards among women who dispensed their pills from the blister packages (study 1) compared with 1.0 (±2.4) recorded by the alarm-activated dispenser (study 2). In study 2, a mean of 1.9 (±4.2) missed pills were reported in the diaries over 1 year compared with 4.4 (±9.1) from automatic recording by the dispenser (both arms of study 2), indicating underreporting of missed pills in diary cards vs the digital dispenser. Adjusting for this rate of underreporting, an estimated mean of ten pills were missed over 1 year by women using EE/drospirenoneFlex in blister packs, or ten times more than with the digital dispenser with activated acoustic alarm. The digital dispenser helps reduce the number of missed pills and increases adherence.

  14. Overlapped Stenting Combined with Coiling for Blood Blister-Like Aneurysms: Comparison of Low-Profile Visualized Intraluminal Support (LVIS) Stent and Non-LVIS Stent.

    Science.gov (United States)

    Zhu, Deyuan; Fang, Yibin; Yang, Pengfei; Zhang, Ping; Chen, Lei; Xu, Yi; Hong, Bo; Huang, Qinghai; Liu, Jian-Min

    2017-08-01

    To evaluate the safety and efficacy of overlapped stenting for blood blister-like aneurysms (BBAs) and to compare the outcomes between Low-profile Visualized Intraluminal Support (LVIS) and non-LVIS stents. A retrospective review of the aneurysm database identified 37 patients with intracranial carotid artery BBAs treated by overlapped stenting in our institution from June 2013 to June 2016. The clinical characteristics and angiographic results were reviewed. Overlapped stenting combined with coiling were applied in 37 BBAs, including LVIS stents in 18 cases and non-LVIS stents in 19. For the LVIS group, angiographic results at 3-24 months were complete occlusion in 15 cases (83.3%), improved in 2 cases (11.1%), and recanalized in 1 case (5.6%). The modified Rankin Scale scores at 3-36 months' follow-up were 0-2 in 15 cases (83.3%) and 3-6 in 3 cases (16.7%). For the non-LVIS group, angiographic results at 3-46 months were complete occlusion in 12 cases (63.2%) and recanalized in 7 cases (36.8%). Clinical outcomes at 6-58 months were modified Rankin Scale scores of 0-2 in 17 cases (89.5%) and 3-6 in 2 cases (10.5%). Use of the LVIS stent was less likely to result in recanalization (odds ratio 0.10, 95% confidence interval 0.01-0.93, P = 0.042) than the non-LVIS stent. The LVIS group had a lower average number of stents than did the non-LVIS group (2.2 vs. 2.6, P = 0.016). In terms of complication rate (11.1% vs. 5.3%, P = 0.604), good outcome rate (83.3% vs. 89.5%, P = 0.660), and immediate angiographic result (P = 0.424), no statistically significant difference between the 2 groups was found. Overlapped LVIS stenting combined with coiling is feasible and safe for BBAs. Overall, the LVIS stent provided less risk of BBA recurrence compared with the non-LVIS stent and did not increase the risk of procedure-related complications. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. Epidermolysis bullosa pruriginosa

    Directory of Open Access Journals (Sweden)

    Yesudia P

    2000-01-01

    Full Text Available Epidermolysis bullosa pruriginosa is a recently described variant of epidermolysis bullosa dystrophica. It is characterised by pruritic nodular prurigo like lesions, milia and with a histopathology of a subepidermal blister. We report 3 cases of this new variant.

  16. Forest insects and diseases in Fundy National Park in 1992. Technical note No. 276. Annual publication

    Energy Technology Data Exchange (ETDEWEB)

    Cormier, J.R.; McPhee, D.A.

    1993-01-01

    Personnel of the Forest Insect and Disease Survey regularly survey national parks for forest insect and disease conditions. This document discusses briefly some of the conditions encountered in Fundy National Park in 1992, including insects and diseases found throughout the Park that are likely to recur: Balsam gall midge, balsam twig aphid, birch casebearer, gypsy moth, porcupines, sirococcus shoot blight, white pine weevil, whitespotted sawyer beetle, yellowheaded spruce sawfly, leaf blister of yellow birch, snow damage, yellow witches' broom of balsam fir, and fall webworm.

  17. Anti-type VII collagen autoantibodies, detected by enzyme-linked immunosorbent assay, fluctuate in parallel with clinical severity in patients with epidermolysis bullosa acquisita.

    Science.gov (United States)

    Ito, Yoshihiro; Kasai, Hiroko; Yoshida, Tetsuya; Saleh, Marwah A; Amagai, Masayuki; Yamagami, Jun

    2013-11-01

    Epidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal blistering disease caused by autoantibodies against type VII collagen. An enzyme-linked immunosorbent assay (ELISA) is currently available to detect autoantibodies in EBA. There have been reports suggesting generically that ELISA indices reflect EBA disease severity; however, there is, as yet, no conclusion as to whether ELISA indices fluctuate with disease activity over time in each EBA patient. This study aimed to investigate whether ELISA titers fluctuate with EBA disease activity and to validate the clinical significance of checking ELISA values in EBA by monitoring type VII collagen ELISA titers and disease severity, evaluated in terms of numbers of blisters and erosions as a clinical score, over time in three Japanese patients with EBA. All three cases in this study, which were treated successfully, showed titers of anti-type VII collagen autoantibodies detected by ELISA that fluctuated in parallel with disease activity. Especially in case 1, we could determine that the expanding erosions were not due to flare-ups of EBA because the ELISA indices stayed low, although new lesions continued to appear. In fact, control of infection and nutrition helped the lesions to become epithelialized. In conclusion, we found that repeated ELISA measurements are useful in monitoring disease activity and making decisions in EBA treatment plans. © 2013 Japanese Dermatological Association.

  18. Thermal evolution of surface blistering and exfoliation due to ion-implanted hydrogen monomers into Si Left-Pointing-Angle-Bracket 1 1 1 Right-Pointing-Angle-Bracket

    Energy Technology Data Exchange (ETDEWEB)

    Liang, J.H., E-mail: jhliang@ess.nthu.edu.tw [Institute of Nuclear Engineering and Science, National Tsing Hua University, 101, Section 2, Kuang-Fu Road, Hsinchu 300, Taiwan (China); Department of Engineering and System Science, National Tsing Hua University, Hsinchu 300, Taiwan (China); Hu, C.H.; Bai, C.Y. [Department of Engineering and System Science, National Tsing Hua University, Hsinchu 300, Taiwan (China); Chao, D.S. [Nuclear Science and Technology Development Center, National Tsing Hua University, Hsinchu 300, Taiwan (China); Lin, C.M. [Department of Applied Science, National Hsinchu University of Education, Hsinchu 300, Taiwan (China)

    2012-08-01

    This study investigated the dependence of surface blistering and exfoliation phenomena on post-annealing time in H{sup +}-implanted Si Left-Pointing-Angle-Bracket 1 1 1 Right-Pointing-Angle-Bracket . Czochralski-grown n-type Si Left-Pointing-Angle-Bracket 1 1 1 Right-Pointing-Angle-Bracket wafers were room-temperature ion-implanted with 40 keV hydrogen monomers to a fluence of 5 Multiplication-Sign 10{sup 16} cm{sup -2}, and followed by furnace annealing treatments at 400 and 500 Degree-Sign C for various durations ranging from 0.25 to 3 h. The corresponding analysis results for Si Left-Pointing-Angle-Bracket 1 0 0 Right-Pointing-Angle-Bracket (Liang et al., 2008); (Bai, 2007) were adopted in order to make comparisons. The evolution of blister formation and growth for Si Left-Pointing-Angle-Bracket 1 1 1 Right-Pointing-Angle-Bracket at 400 Degree-Sign C has a shorter characteristic time compared to Si Left-Pointing-Angle-Bracket 1 0 0 Right-Pointing-Angle-Bracket . However, there is a longer characteristic time when annealing takes place at 500 Degree-Sign C. In addition, no craters were observed for Si Left-Pointing-Angle-Bracket 1 1 1 Right-Pointing-Angle-Bracket annealed at 400 Degree-Sign C while the opposite is true for Si Left-Pointing-Angle-Bracket 1 0 0 Right-Pointing-Angle-Bracket . The evolution of crater development for Si Left-Pointing-Angle-Bracket 1 1 1 Right-Pointing-Angle-Bracket annealed at 500 Degree-Sign C has a longer characteristic time compared to Si Left-Pointing-Angle-Bracket 1 0 0 Right-Pointing-Angle-Bracket . These results are attributed to the fact that compared to Si Left-Pointing-Angle-Bracket 1 0 0 Right-Pointing-Angle-Bracket , Si Left-Pointing-Angle-Bracket 1 1 1 Right-Pointing-Angle-Bracket has a smaller surface binding energy of silicon atoms and a larger areal number density of silicon atoms on the plane perpendicular to the incident-ion axis. Furthermore, Si Left-Pointing-Angle-Bracket 1 1 1 Right-Pointing-Angle-Bracket has a

  19. Efficacy of the melanocortin analogue Nle4-D-Phe7-α-melanocyte-stimulating hormone in the treatment of patients with Hailey–Hailey disease

    OpenAIRE

    Biolcati, G; Aurizi, C; Barbieri, L.; Cialfi, S; Screpanti, I.; Talora, C

    2013-01-01

    Background Hailey–Hailey disease (HHD) is a rare, chronic and recurrent blistering disorder, which is characterized clinically by erosions occurring primarily in intertriginous regions, and histologically by suprabasal acantholysis. Oxidative stress plays a specific role in the pathogenesis of HHD, by regulating the expression of factors playing an important role in keratinocyte proliferation and differentiation. Aim Given the significance of oxidative stress in HHD, we investigated the poten...

  20. Spectrum of autoimmune bullous diseases in Kuwait.

    Science.gov (United States)

    Nanda, Arti; Dvorak, Richard; Al-Saeed, Khloud; Al-Sabah, Humoud; Alsaleh, Qasem A

    2004-12-01

    Autoimmune bullous diseases (ABDs) are a rare but significant group of dermatoses that pose great challenges to the treating dermatologist. Most epidemiological studies have focused on a single ABD. Few surveys have been carried out to describe the whole spectrum of ABDs in a region, and no such studies are available from the Arabian Peninsula. To determine the clinico-epidemiological features of various ABDs in Kuwait, and to compare the results with those reported elsewhere. A total of 128 cases of ABDs were studied over a span of 11.5 years. The diagnosis in all cases was confirmed by histopathology, and direct and indirect immunofluorescence (IMF). The diagnosis of various subepidermal ABDs was further confirmed by indirect IMF on salt-split skin (SSS) and that of pemphigus by desmoglein 1 and 3 enzyme-linked immunosorbent assay (ELISA). Eighty seven per cent of patients were of Arab ethnicity. Pemphigus was observed to be the commonest ABD (47%), followed by pemphigoid (22%), pemphigoid gestationis (PG) (19%), linear IgA bullous disease (LABD) (7%), lichen planus pemphigoides (LPP) (3%), and epidermolysis bullosa acquisita (EBA) (2.3%). The minimum estimated incidence in the local population was 4.6, 2.14, 1.83, 0.69, 0.30, and 0.23 cases per million per year, respectively. Pemphigus patients were observed to have a younger age of onset (36.50 +/- 11.36 years) than reported elsewhere. BP, although the second commonest ABD, was less prevalent than in Europe and Singapore, and BP patients were observed to have a striking female predominance (85%). The prevalence of PG was much higher than that reported elsewhere. LABD was the fourth commonest ABD, and 89% of patients were children. The study suggests that similar surveys from different regions would expand our understanding of ABD.

  1. Autoimmune bullous disease and Hashimoto's disease complicated by acquired hemophilia A.

    Science.gov (United States)

    Nishiura, Nobuko; Ujimoto, Daisuke; Fujita, Jiro; Maeda, Tetsuo; Nakagawa, Yukinobu; Kashiwagi, Hirokazu; Oritani, Kenji; Tomiyama, Yoshiaki; Kanakura, Yuzuru

    2017-01-01

    A 67-year-old man was admitted with a 1-month history of spontaneous hematoma in his right back and severe anemia. He had suffered from rashes with blisters involving both legs for 10 years, which had shown worsening and extended to his entire body concurrently with the hematoma. APTT was markedly prolonged to 119 seconds, and Factor VIII:C and FVIII inhibitor levels were less than 1% and 153.1 BU/ml, respectively, confirming the diagnosis of acquired hemophilia A (AHA). Skin biopsy revealed his rashes to be caused by autoimmune bullous disease (ABD), and laboratory and physical findings showed that he also had autoimmune hypothyroidism (Hashimoto's disease). Recombinant FVIIa was effective for management of his bleeding; in addition, FVIII inhibitor reduction and FVIII:C recovery, in parallel with improvement of the skin lesions, were achieved by administering prednisolone and cyclophosphamide. To our knowledge, this is the first report of AHA associated with ABD and Hashimoto's disease.

  2. Exanthema in Legionnaires' disease mimicking a severe cutaneous drug reaction.

    Science.gov (United States)

    Ziemer, M; Ebert, K; Schreiber, G; Voigt, R; Sayer, H G; Marx, G

    2009-07-01

    Legionnaires' disease is an acute bacterial infection, generally caused by Legionella pneumophila, which primarily involves the lower respiratory tract, although it is often associated with multisystemic extrapulmonary features. Cutaneous features are very uncommon and may include erythematous or petechial, macular or maculopapular lesions. We report a male patient who expressed all features of a severe lobular pneumonia. Over the course of the disease the patient developed a livid erythematous, maculopapular exanthem rapidly extending over the entire body. Given the rapid development and target-like appearance of the skin lesions with extensive skin involvement and blister formation, the initial diagnosis was that of a severe cutaneous drug reaction. However, histological examination of biopsy did not confirm this diagnosis, but instead was suspicious for a viral exanthem or a more aggressive inflammatory response due to sensitization to bacterial antigens. L. pneumophila infection was verified during the course of the disease.

  3. Junctional Epidermolysis Bullosa

    Directory of Open Access Journals (Sweden)

    Chuan-Hong Kao

    2006-10-01

    Full Text Available Epidermolysis bullosa (EB encompasses a heterogeneous group of genodermatoses, characterized by fragility and blistering of the skin, often associated with extracutaneous manifestations. The level of vesiculation within the skin defines 3 major subtypes of EB: EB simplex, junctional EB, and dystrophic EB. We present the case of a male neonate of 36 weeks' gestation, who was born with a few blisters with erosions and who rapidly developed extensive blistering of the skin. Histopathology revealed subepidermal blistering. Electron microscopy confirmed the cleavage of epidermis from dermis within the lamina lucida. Junctional EB was the diagnosis. The patient was discharged after hospitalization for 28 days. The development of new blisters with erosions were gradually improved after AQUACEL® Ag dressing, and the general condition was much better than at admission. The patient likely has a subtype of junctional EB termed generalized atrophic benign EB that clinically improves with age. He has the potential to father children and has a normal life expectancy.

  4. A skin disease, a blood disease or something in between? An exploratory focus group study of patients' experiences with porphyria cutanea tarda*

    Science.gov (United States)

    Andersen, J; Gjengedal, E; Sandberg, S; Råheim, M

    2015-01-01

    Background Porphyria cutanea tarda (PCT) is characterized by fragile skin with blistering on sun-exposed areas. Symptoms typically develop in late adulthood and can be triggered by iron overload, alcohol intake, oestrogens and various liver diseases. Treatment consists of phlebotomy to reduce iron, or increasing urinary porphyrin excretion by administering chlorochin. To optimize patient care, health personnel need to understand the subjective experiences of PCT. Objectives To explore the experiences of persons with PCT with regard to symptoms, treatment, follow-up and prevention of the disease. Methods Interpretive description was used as a qualitative approach. Twenty-one participants attended three focus groups. All participants had experienced PCT symptoms during the last 5 years. Results Participants' experiences varied from trivializing symptoms and fragile skin to what was described as a desperate situation, with huge blisters, skin falling off and feeling as if one was in a ‘horror movie’. For some, itching was very troublesome, preventing sleep and delaying skin healing. In managing PCT a shift in focus from skin to blood was described. PCT was perceived as a chronic and systemic disease causing a range of health problems. Strategies for preventing symptoms ranged from doing nothing to frequent controls and check-ups. Conclusions Participants had a systemic perception of PCT, and a tendency to attribute a range of health problems to the condition. This study adds insight into the experiences patients have with PCT. PMID:24958197

  5. Disease: H00586 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available osa, junctional, with pyloric atresia (JEB-PA) Inherited epidermolysis bullosa is a diverse group of disorders characterized by mecha...nically fragile skin that readily blister. The junctiona

  6. Disease: H00585 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available lysis bullosa simplex, Ogna type (EBS-Ogna) Inherited epidermolysis bullosa is a diverse group of disorders characterized by mechanic...ally fragile skin that readily blister. The conditions i

  7. Epidermólise bolhosa albopapulóide (variante de Pasini Albopapuloid epidermolysis bullosa (Pasini's variant

    Directory of Open Access Journals (Sweden)

    Letícia Pereira Spinelli

    2003-08-01

    Full Text Available A epidermólise bolhosa albopapulóide é doença rara, de caráter autossômico dominante, também conhecida como epidermólise bolhosa distrófica albopapulóide de Pasini. Os autores relatam o caso de uma paciente de 37 anos de idade, com início do quadro aos sete anos, com lesões bolhosas e milia nas áreas de traumatismo, além de máculas hipocrômicas e atróficas (lesões albopapulóides no corpo. A paciente também apresenta pele apergaminhada, xerótica, e distrofia ungueal dos pés. A histopatologia da lesão bolhosa revelou clivagem subepidérmica, compatível com o diagnóstico clínico.Albopapuloid epidermolysis bullosa (Pasini's variant is a rare, autosomal dominant inherited disorder. The authors report the case of a 37-year-old female patient with blisters on trauma areas, atrophic macules (albopapuloid lesions and milia, with onset of disease at age seven. The histopathological exam of the blister reveals a subepidermal cleavage.

  8. Bullous pemphigoid with prominent milium formation.

    Science.gov (United States)

    Tsuruta, Daisuke; Brzezinski, Piotr; Koga, Hiroshi; Ohata, Chika; Furumura, Minao; Hashimoto, Takashi

    2013-01-01

    Milia are very common superficial keratinous cysts, clinically seen as pearly white dome-shaped lesions with a diameter of 1-2 mm. Bullous pemphigoid (BP) is an autoimmune bullous disease characterized clinically by tense bullae on the extremities and trunk. The major target autoantigens of BP are BP180 and BP230. We report a 55-year-old Polish BP patient presenting prominent milium formation. Physical examination revealed multiple tense bullae on the erythemas scattered on the extremities and trunk. Histopathology revealed subepidermal blisters with infiltration of eosinophils in and around the blister. Direct immunofluorescence showed IgG and C3 depositions at basement membrane zone. Although indirect immunofluorescence of normal human skin sections was negative, indirect immunofluorescence of salt-split skin sections showed IgG reactivity with epidermal side. Immunoblotting showed that IgG antibodies in the serum reacted with recombinant protein of the BP180 NC16a domain. ELISA of BP180, but not BP230 and type VII collagen, showed positive results. Several months after oral prednisolone therapy, multiple large milia appeared on the healed BP lesions. Histopathology showed cysts with flaky keratinous inclusions in the mid-dermis. We diagnosed the patient as BP with milia. Since milia are occasionally found in BP, they are not a definite differential criterion from epidermolysis bullosa acquisita.

  9. Immune deposits in cutaneous lesions of Wegener′s granulomatosis: Predictor of an active disease

    Directory of Open Access Journals (Sweden)

    Seema Chhabra

    2011-01-01

    Full Text Available A retrospective analysis was conducted of eight cases of Wegener′s granulomatosis (WG, who presented with cutaneous lesions. The clinical, immunopathologic and histopathologic features of the cutaneous lesions were reviewed. Antineutrophil cytoplasmic antibody (ANCA status of the patients was established. When possible, a comparison of immunofluorescence findings of skin biopsies was made with those of renal biopsies taken at the same time. In all except one, systemic and cutaneous disease developed concurrently. On histopathology, leukocytoclastic vasculitis was noted in five patients and features of lupus erythematosus and pyoderma gangrenosum in one case each. Four patients showed immunoglobulin deposits in subepidermal blood vessel walls, while one patient showed granular immune deposits at dermo-epidermal junction only. Immunoglobulin G was the most common immunoreactant detected. C-ANCA/proteinase 3 (PR3-ANCA was positive in six patients, P-ANCA/myeloperoxidase (MPO-ANCA in one patient, while one patient did not show ANCA positivity on indirect immunofluorescence. All four renal biopsies showed pauci-immune glomerulonephritis, irrespective of the presence (n=3 or absence (n=1 of immune deposits in the skin biopsy. Skin manifestations are encountered in nearly half of the patients with WG, thus it is important to be familiar with cutaneous histopathologic as well as immunofluorescence findings in WG patients.

  10. The IL-8 release from cultured human keratinocytes, mediated by antibodies to bullous pemphigoid autoantigen 180, is inhibited by dapsone

    Science.gov (United States)

    Schmidt, E; Reimer, S; Kruse, N; Bröcker, E-B; Zillikens, D

    2001-01-01

    Bullous pemphigoid (BP) is a subepidermal blistering disease associated with autoantibodies to the hemidesmosomal 180 kD BP autoantigen (BP180). However, the binding of autoantibodies to BP180 alone is not sufficient for blister formation in this disease and the infiltration of neutrophils into the skin is required. Dapsone and nicotinamide inhibit neutrophil chemotaxis and are used effectively in treating BP. IL-8 is a known chemoattractant for neutrophils and has been implicated in the inflammatory process of both human and experimental murine BP. We have recently shown that antibodies to BP180 mediate a dose and time-dependent release of IL-6 and IL-8 from cultured normal human epidermal keratinocytes (NHEK). In the present study, we addressed the question whether dapsone or nicotinamide influence this cytokine release. We demonstrate that dapsone, but not nicotinamide, in its pharmacological range, inhibits the IL-8, but not the IL-6 release from NHEK, induced by anti-BP180 IgG, in a dose-dependent fashion as detected by ELISA. IL-8 mRNA levels, as determined by RT-PCR, were the same in cells treated with BP IgG alone compared to cells treated with BP IgG plus dapsone. This observation suggests that dapsone inhibits the BP IgG-induced IL-8 release from cultured NHEK by mechanisms at the post-transcriptional level. Our findings contribute to the understanding how dapsone leads to a reduced influx of neutrophils into BP lesions and, finally, to the cessation of blister formation in this disease. PMID:11359455

  11. Aspectos morfológicos da colonização de Colletotrichum gloeosporioides em órgãos de plantas de cafeeiros e com sintomas da mancha manteigosa Morphologic aspects of colonization of Colletotrichum gloeosporioides on organs of coffee plants with blister spot

    Directory of Open Access Journals (Sweden)

    Josimar Batista Ferreira

    2009-08-01

    Full Text Available Objetivou-se, no presente trabalho, analisar microscopicamente a colonização de C. gloeosporioides, agente da mancha manteigosa (MM, em condições naturais da doença, sobre os diferentes órgãos e tecidos da cultivar Catucaí Vermelho: folhas, pecíolos, nervuras, ramos, frutos e pedúnculos. Todas as amostras foram processadas, obtendo-se imagens em microscópico eletrônico de varredura. Os ramos e as nervuras de folhas de cafeeiros com mancha manteigosa apresentando morte descendente e hipocótilos oriundos de sementes têm os vasos do xilema, floema e células do córtex colonizados por C. gloeosporioides, já os frutos com sintoma da mancha manteigosa apresentaram colonização nos tecidos do exocarpo, mesocarpo, endocarpo e endosperma.The aim of this work was to study the colonization of leaves, petioles, veins, branches, fruits, and peduncles by C. gloeosporioides, the causal agent of blister spot, under natural infections of cultivar Catucaí Vermelho in field conditions. All materials were analyzed through scanning electron microscopy. The branches and veins of coffee leaves affected by blister spot with descending death as well as hypocotyls from seedlings had their xylem, phloem and cortical cells colonized by C. gloeosporioides while fruits showing blister spot symptoms had their exocarp, mesocarp, endocarp, and endosperm tissues colonized by C. gloeosporioides.

  12. Treatment of Intraepidermal Autoimmune Bullous Diseases Sürekli Eğitim

    Directory of Open Access Journals (Sweden)

    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

  13. Injection of Acanthaster planci with thiosulfate-citrate-bile-sucrose agar (TCBS). I. Disease induction.

    Science.gov (United States)

    Rivera-Posada, J A; Pratchett, M; Cano-Gómez, A; Arango-Gómez, J D; Owens, L

    2011-12-06

    This is the first report of the successful induction of a transmissible disease in the coral-eating crown-of-thorns starfish Acanthaster planci (COTS). Injection of thiosulfate-citrate-bile-sucrose agar (TCBS) culture medium into COTS induced a disease characterized by discoloured and necrotic skin, ulcerations, loss of body turgor, accumulation of colourless mucus on many spines especially at their tip, and loss of spines. Blisters on the dorsal integument broke through the skin surface and resulted in large, open sores that exposed the internal organs. Oedema and reddened digestive tissues and destruction of connective fibers were common. Moreover, healthy COTS in contact with these infected animals also displayed signs of disease and died within 24 h. TCBS induced 100% mortality in injected starfish. There was no introduction of new pathogens into the marine environment. TCBS promoted the growth of COTS' naturally occurring Vibrionales to high densities with subsequent symbiont imbalance followed by disease and death.

  14. Rituximab therapy in pemphigus and other autoantibody-mediated diseases [version 1; referees: 3 approved

    Directory of Open Access Journals (Sweden)

    Nina A. Ran

    2017-01-01

    Full Text Available Rituximab, a monoclonal antibody targeting the B cell marker CD20, was initially approved in 1997 by the United States Food and Drug Administration (FDA for the treatment of non-Hodgkin lymphoma. Since that time, rituximab has been FDA-approved for rheumatoid arthritis and vasculitides, such as granulomatosis with polyangiitis and microscopic polyangiitis. Additionally, rituximab has been used off-label in the treatment of numerous other autoimmune diseases, with notable success in pemphigus, an autoantibody-mediated skin blistering disease. The efficacy of rituximab therapy in pemphigus has spurred interest in its potential to treat other autoantibody-mediated diseases. This review summarizes the efficacy of rituximab in pemphigus and examines its off-label use in other select autoantibody-mediated diseases.

  15. The role of neutrophils in autoimmune diseases.

    Science.gov (United States)

    Németh, Tamás; Mócsai, Attila

    2012-03-30

    Though chronic autoimmune disorders such as rheumatoid arthritis or systemic lupus erythematosus affect a significant percentage of the human population and strongly diminish the quality of life and life expectancy in Western societies, the molecular pathomechanisms of those diseases are still poorly understood, hindering the development of novel treatment strategies. Autoimmune diseases are thought to be caused by disturbed recognition of foreign and self antigens, leading to the emergence of autoreactive T-cells (so-called immunization phase). Those autoreactive T-cells then trigger the second (so-called effector) phase of the disease which is characterized by immune-mediated damage to host tissues. For a long time, neutrophils have mainly been neglected as potential players of the development of autoimmune diseases. However, a significant amount of new experimental data now indicates that neutrophils likely play an important role in both the immunization and the effector phase of autoimmune diseases. Here we review the current literature on the role of neutrophils in autoimmune diseases with special emphasis on rheumatoid arthritis, systemic lupus erythematosus, autoimmune vasculitides and blistering skin diseases. We also discuss the role of neutrophil cell surface receptors (e.g. integrins, Fc-receptors or chemokine receptors) and intracellular signal transduction pathways (e.g. Syk and other tyrosine kinases) in the pathogenesis of autoimmune inflammation. Though many of the results discussed in this review were obtained using animal models, additional data indicate that those mechanisms likely also contribute to human pathology. Taken together, neutrophils should be considered as one of the important cell types in autoimmune disease pathogenesis and they may also prove to be suitable targets of the pharmacological control of those diseases in the future. Copyright © 2012 Elsevier B.V. All rights reserved.

  16. Childhood Epidermolysis Bullosa Acquisita: Confirmation of Diagnosis by Skin Deficient in Type VII Collagen, Enzyme-linked Immunosorbent Assay, and Immunoblotting

    OpenAIRE

    Nupur Goyal; Raghavendra Rao; Balachandran, C.; Sathish Pai; Balbir S Bhogal; Enno Schmidt; Detlef Zillikens

    2016-01-01

    Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disorder characterized by autoantibodies against Type VII collagen. It usually affects adults; childhood EBA is rare. We describe a 10-year-old girl presenting with recurrent tense blisters predominantly on legs, dorsa of hands and feet accompanied by oral erosions since the age of 5 years. Direct immunofluorescence (IF) microscopy showed linear deposition of IgG and C3 along the basement membrane zone (BMZ); indirect I...

  17. Immunopathogenic Oral Diseases: An Overview 
Focusing on Pemphigus Vulgaris and Mucous Membrane Pemphigoid.

    Science.gov (United States)

    Feller, Liviu; Ballyram, Raoul; Khammissa, Razia Ag; Altini, Mario; Lemmer, Johan

    Pemphigus vulgaris, mucosal pemphigoid (mucous membrane pemphigoid), lichen planus, discoid lupus erythematosus and erythema multiforme are a group of immune-mediated mucocutaneous disorders characterised clinically by the formation of blisters, erosions or ulcers. The oral mucosa is often affected, and sometimes the disease is limited to the mouth. The target antigens, autoreactive immune responses, microscopic features, treatment and prognosis vary from one disease to the other. Treatment aims to eliminate exogenous risk factors, suppress the pathogenic immuno-inflammatory reactions, promote healing and prevent infection. The aim of this article is to provide the general dental practitioner with a succinct overview of the diagnostic, clinical, aetiopathogenic features and characteristics of, as well as treatment guidelines for oral pemphigus vulgaris and oral mucosal pemphigoid. Early diagnosis and treatment could prevent severe consequences of the disease in their full-blown forms.

  18. Coma blisters after poisoning caused by central nervous system depressants: case report including histopathological findings Bolhas do coma pós-intoxicação por depressores do sistema nervoso central: relato de caso incluindo achados histopatológicos

    Directory of Open Access Journals (Sweden)

    Maira Migliari Branco

    2012-08-01

    Full Text Available Blister formation and eccrine sweat gland necrosis is a cutaneous manifestation associated with states of impaired consciousness, most frequently reported after overdoses of central nervous system depressants, particularly phenobarbital. The case of a 45-year-old woman who developed "coma blisters" at six distinct anatomic sites after confirmed (laboratory phenobarbital poisoning, associated with other central nervous system depressants (clonazepam, promethazine, oxcarbazepine and quetiapine, is presented. A biopsy from the left thumb blister taken on day 4 revealed focal necrosis of the epidermis and necrosis of sweat gland epithelial cells; direct immunofluorescence was strongly positive for IgG in superficial blood vessel walls but negative for IgM, IgA, C3 and C1q. The patient was discharged on day 21 with no sequelae.Formação de bolhas e necrose de glândula sudoríparas écrinas é uma manifestação cutânea associada com estados de diminuição da consciência, mais frequentemente relatada após superdosagens de depressores do sistema nervoso central, particularmente fenobabital. Relatamos o caso de uma paciente de 45 anos que desenvolveu "bolhas do coma" após tentativa de suicídio por fenobarbital (confirmada laboratorialmente, associada a outros depressores do sistema nervoso central (clonazepam, prometazina, oxcarbazepina e quetiapina. Biópsia da bolha do 1o quirodáctilo esquerdo no 4o dia de internação revelou necrose focal da epiderme e necrose de células epiteliais de glândula sudorípara; a imunofluorescência direta foi fortemente positiva para IgG na parede superficial dos vasos sanguíneos, mas negativa para IgM, IgA, C3 e C1q. A paciente teve alta no 21o dia, sem seqüelas.

  19. Bullous Mastocytosis Mimicking Congenital Epidermolysis Bullosa

    Directory of Open Access Journals (Sweden)

    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.

  20. Autoimmune bullous skin diseases. Part 2: diagnosis and therapy.

    Science.gov (United States)

    Kneisel, Andrea; Hertl, Michael

    2011-11-01

    Autoimmune bullous skin diseases represent a heterogenous group of disorders of skin and mucosa which are commonly associated with IgG or IgA autoantibodies against distinct adhesion molecules of the skin. The antibodyinduced loss of adhesion between epidermis and dermis results in blister formation and extensive erosions. There is a great need for rapidly establishing the diagnosis of these disorders since they may run a severe and potentially life-threatening course. In addition, because of their rarity and heterogeneous symptoms, autoimmune bullous skin diseases often pose a major diagnostic challenge. While histopathological examinations provide evidence for the level of blister formation, immunofluorescence microscopy has been established to identify tissue-bound and circulating autoantibodies. Direct immunofluorescence microscopy represents the gold standard for detecting tissue-bound autoantibodies. Indirect immunofluorescence microscopy with defined tissue substrates is considered the first step in detecting circulating autoantibodies. Confirmatory tests such as ELISA, immunoblot or immunoprecipitation analyses are performed utilizing recombinant proteins or keratinocyte extracts. The later assays can be used for primary diagnosis as well as for immunoserological follow-up. Systemic immunosuppressive drugs usually represent the main therapeutic regimen. Initially, systemic corticosteroids are commonly administered in combination with steroid-sparing, immunosuppressive agents. Novel targeted treatments such as immunoadsorption, rituximab or high-dose intravenous immunoglobulins have proven to be highly effective in severe and refractory pemphigus. This review presents a state-of-the-art algorithm for making the diagnosis of autoimmune bullous disorders and provides an overview on currently available therapeutic options. © The Authors • Journal compilation © Blackwell Verlag GmbH, Berlin.

  1. Chemical Blistering: Cellular and Macromolecular Components

    Science.gov (United States)

    1987-01-30

    nucleoids derived from monolayers of keratinocytes imiediately after exposure for 1 hour to 0.1 to 10 uM BCES ..................... 11 Figure 2...Sedimentation rate of nucleoids derived from monolayers of ke- ratinocytes exposed for 1 hour to 0.1 to 10 u. BCES, washed and incubated for 22 hours in fresh...Effect of a topical exposure to BCES on the DNA of basal cells within a lifted stratified culture ........................ 47 4 Figure 41. Nucleoid

  2. Chemical Blistering: Cellular and Macromolecular Components.

    Science.gov (United States)

    1987-11-15

    Ulex europeus lectin. In tissue sections of skin, this lectin binds to the epidermal spinous cells (cf Brabec, et al., 1980). The methodology of...the surface of basal cell and the cells of the lower two spinous layers. On the other hand, Ulex europeus Agglutinin I (UEA) was seen to bind to the

  3. Identification of novel elements of the Drosophila blisterome sheds light on potential pathological mechanisms of several human diseases.

    Directory of Open Access Journals (Sweden)

    Oleksii Bilousov

    Full Text Available Main developmental programs are highly conserved among species of the animal kingdom. Improper execution of these programs often leads to progression of various diseases and disorders. Here we focused on Drosophila wing tissue morphogenesis, a fairly complex developmental program, one of the steps of which--apposition of the dorsal and ventral wing sheets during metamorphosis--is mediated by integrins. Disruption of this apposition leads to wing blistering which serves as an easily screenable phenotype for components regulating this process. By means of RNAi-silencing technique and the blister phenotype as readout, we identify numerous novel proteins potentially involved in wing sheet adhesion. Remarkably, our results reveal not only participants of the integrin-mediated machinery, but also components of other cellular processes, e.g. cell cycle, RNA splicing, and vesicular trafficking. With the use of bioinformatics tools, these data are assembled into a large blisterome network. Analysis of human orthologues of the Drosophila blisterome components shows that many disease-related genes may contribute to cell adhesion implementation, providing hints on possible mechanisms of these human pathologies.

  4. A sudden paediatric death due to hand, foot and mouth disease: the importance of vigilance.

    Science.gov (United States)

    Rahimi, R; Omar, E; Tuan Soh, T S; Mohd Nawi, S F A; Md Noor, S

    2017-08-01

    Hand, foot and mouth disease (HFMD) is caused by enteroviruses such as Coxsackie virus A16 (CVA16) and Enterovirus 71 (EV71). The diagnostic hallmarks are oral ulcers and maculo-papular or vesicular rash on the hands and feet. Severe form of this disease can lead to death due to neurological and cardiopulmonary complications. This case report aims to describe a fatal case of HFMD with minimal oral and skin manifestations. A four-year-old girl was brought to a hospital after suddenly becoming unresponsive at home. She had a history of fever and lethargy for three days prior to her demise. The patient, and f ive other children in her neighbourhood had been diagnosed to have HFMD at a local health clinic; the other children had recovered without complications. Autopsy revealed a few punctate, sub-epidermal vesicles measuring 1 to 2 mm on the palm of her right hand and sole of the right foot, visible only with a magnifying glass. Internal examination revealed prominent nodularity at the oro- and hypopharynxes. The lungs were markedly congested and oedematous. Histopathology of the lung showed marked oedema and haemorrhage with mild pneumonic changes. Oedema with increase in macroglia and astrocytic proliferation were seen in the cerebral tissue, but no lymphocytic infiltration was evident. Enterovirus EV71 was detected by polymerase chain reaction in samples from the lung, cerebrospinal fluid and serum. The cause of death was given as HFMD complicated by pneumonia. Fatal HFMD may have minimal signs. A complete history, careful physical examination and relevant investigations lead to a diagnosis at post mortem examination. Awareness of the subtle signs and rapid deterioration associated with a fatal case of HFMD is a challenge to clinicians who encounter these cases.

  5. Bullous pemphigoid associated with acquired hemophilia a: a rare association of autoimmune disease.

    Science.gov (United States)

    Aljasser, Mohammed I; Sladden, Chris; Crawford, Richard I; Au, Sheila

    2014-01-01

    Acquired hemophilia (AH) is a rare autoimmune disease with an annual incidence of one per million and has a mortality rate of up to 22%. It is caused by the development of autoantibodies against factor VIII. Approximately half of the reported cases are associated with autoimmune disorders, pregnancy, malignancies, and adverse drug reactions. Autoimmune diseases are the most frequently associated disorders and include rheumatoid arthritis, systemic lupus erythematosus, cryoglobulinemia, pemphigus vulgaris, and bullous pemphigoid. There are a few reports of acquired hemophilia and bullous pemphigoid in the literature. We report a 73-year-old male who presented with cutaneous blistering, upper gastrointestinal bleeding, and hemoptysis. He later developed right flank pain secondary to a retroperitoneal hematoma. He had a prolonged partial thromboplastin time, a low factor VIII level, and a high factor VIII inhibitor level, all consistent with acquired hemophilia. Skin biopsies were diagnostic for bullous pemphigoid. He was treated successfully with prednisone, cyclophosphamide, rituximab, and intravenous immunoglobulin.

  6. 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.

  7. [Epidermolysis bullosa acquisita].

    Science.gov (United States)

    Caux, Frédéric

    2010-10-01

    Epidermolysis bullosa acquisita (EBA) is a rare autoimmune subepidermal bullous disease with autoimmunity to the type VII collagen which is the major component of anchoring fibrils. Clinical manifestations of the classical EBA include skin fragility, blisters over the trauma-prone surfaces and milium cysts. Other presentations of EBA have been reported: mucosal predominant appearance reminiscent of cicatricial pemphigoid, bullous pemphigoid-like presentation and IgA-EBA. Making a definitive diagnosis of EBA could be difficult because specialized tests available in only some laboratories are necessary to confirm the clinical suspicion: immunoelectron microscopy demonstrating immune deposits on anchoring fibrils and immunoblotting or enzyme-linked immunosorbent assay (Elisa) detecting autoantibodies recognizing the type VII collagen. EBA frequently is associated with Crohn's disease and an inflammatory bowel disease must be ruled out in patients with EBA and abdominal manifestations. EBA potentially is serious, has usually a chronical evolution and is difficult to treat.There are no guidelines for treatment of EBA, which is adapted to clinical severity and include dapsone, cyclosporine and rituximab. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

  8. Disease: H00644 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00644 Ectodermal dysplasia/skin fragility syndrome Ectodermal dysplasia/skin fragility... syndrome is a very rare genodermatosis that develops skin fragility with tearing and blisters and cong...ophilin 1 gene result in ectodermal dysplasia/skin fragility syndrome. ... JOURNAL ... Nat Genet 17:240-4 (1997) DOI:10.1038/ng1097-240 ...

  9. Dermatoses bolhosas auto-imunes Autoimmune bullous dermatoses

    Directory of Open Access Journals (Sweden)

    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.

  10. Imunoglobulina intravenosa para tratamento de epidermólise bolhosa adquirida grave refratária a terapia imunossupressora convencional Intravenous immunoglobulin for treatment of severe acquired bullous epidermolysis refractory to conventional immunosuppressive therapy

    Directory of Open Access Journals (Sweden)

    Carolina Balbi Mosqueira

    2010-08-01

    Full Text Available A epidermólise bolhosa adquirida é doença bolhosa subepidérmica crônica e rara. Geralmente, inicia-se na fase adulta, sendo a etiologia desconhecida, embora vinculada à presença de anticorpos contra o colágeno tipo VII. Há formação de bolhas, espontaneamente ou após trauma, podendo causar complicações graves. O tratamento é desapontador e difícil. Além da terapia convencional com corticoides sistêmicos, recentemente, novas modalidades terapêuticas promissoras estão sendo utilizadas, dentre elas, a imunoglobulina intravenosa. Destaca-se, neste relato, o difícil manejo clínico desta doença, e a melhora importante com a imunoglobulina intravenosa.Acquired bullous epidermolysis is a chronic and rare bullous subepidermal disease. It usually begins in adulthood and its etiology is unknown although it is associated with antibodies against type VII collagen. There are spontaneous and trauma induced formation of blisters that may cause serious complications. Treatment is disappointing and difficult. Apart from conventional therapy with systemic corticosteroid, new therapeutic modalities such as intravenous immunoglobulin are currently being used. This report highlights the extremely difficult clinical management of this rare disease and the important improvement provided by intravenous immunoglobulin.

  11. Anti-p200 pemphigoid responding to dapsone

    Directory of Open Access Journals (Sweden)

    Laura Van Lerberghe

    2014-03-01

    Full Text Available Anti-p200 pemphigoid is a rare autoimmune subepidermal blistering disease. Clinical presentation is similar to standard bullous pemphigoid (BP but mucous membranes and cephalic lesions are more frequent. Histology and direct immunofluorescence (IF are identical to BP but indirect IF discloses linear deposits of immunoglobulin G (IgG on the dermal side of artificial salt-split skin. Specific diagnosis is based on western immunoblotting that shows circulating IgG recognizing a 200-kDa protein localized on the dermal extract. The 200-kDa antigen was recently identified as laminin γ1. Anti-p200 pemphigoid should be considered before all atypical or topical steroid-resistant bullous disease, as well as mucous membranes pemphigoid or epidermolysis bullosa acquisita. Dapsone appears to be the most effective treatment and should be used as the first option in combination with topical steroids. In this report, we describe the case of a patient with a typical clinical and immunopathological anti-p200 pemphigoid, responding to a combination of topical steroids and dapsone.

  12. Munchausen by proxy syndrome mimicking systemic autoinflammatory disease: case report and review of the literature.

    Science.gov (United States)

    Wittkowski, Helmut; Hinze, Claas; Häfner-Harms, Sigrid; Oji, Vinzenz; Masjosthusmann, Katja; Monninger, Martina; Grenzebach, Ulrike; Foell, Dirk

    2017-04-05

    Systemic autoinflammatory diseases (SAIDs) represent a growing number of monogenic, polygenic or multifactorial disorders that are often difficult to diagnose. Here we report a patient who was initially erroneously diagnosed and treated for SAID. Symptoms consisted of recurrent fever, erythematous and/or blistering skin lesions, angioedema, susceptibility to bleeding, external ear infections and reversible anisocoria in the absence of laboratory evidence of systemic inflammation. After two and a half years of extensive diagnostic work-up and multiple empirical therapies, a final diagnosis of Munchausen by proxy syndrome (MBPS) was established. The diagnosis of SAID needs to be carefully reassessed if measurable systemic inflammation is missing, and MBPS should be included in the differential diagnosis.

  13. 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.

  14. The Ellipta®in asthma and chronic obstructive pulmonary disease: device characteristics and patient acceptability.

    Science.gov (United States)

    Jones, Thomas L; Neville, Daniel M; Chauhan, Anoop J

    2018-02-01

    Asthma and chronic obstructive pulmonary disease are primarily treated with inhaled medication, but delivery of that medication to its site of action is problematic; patients' ability to use inhalers will affect therapeutic response. Multiple inhaler devices are available but they are variably easy to use with consequent effects on compliance, intentional or otherwise. The Ellipta ® device is a novel blister strip dry powder inhaler with medium resistance and a consistent delivered dose across a range of inspiratory flow rates. The Ellipta has proven easy to use and is preferred by patients across several evaluations and compared with other inhaler devices. The Ellipta is used to administer multiple inhaled medications, all in single daily-dose regimens, making it ideal for patients who struggle with complex inhaled therapy regimens.

  15. Update on hand-foot-and-mouth disease.

    Science.gov (United States)

    Ventarola, Daniel; Bordone, Lindsey; Silverberg, Nanette

    2015-01-01

    Hand-foot-and-mouth disease is a viral exanthem caused, primarily by Coxsackie A16 and enterovirus 71 with typical clinical features of fever, painful papules and blisters over the extremities and genitalia and an enanthem involving ulceration of the mouth, palate, and pharynx. Other enteroviruses have recently been noted to cause severe neurologic illness and paralysis (enterovirus 68) with variable cutaneous features. A recent outbreak of Coxsackie A6 infection has been seen worldwide with cases reported in the United States, Japan, Southeast Asia, and Europe. These cases have caused extensive cutaneous disease variants, some of which are not previously recognized in Coxsackie infection, namely vesicobullous and erosive eruptions, extensive cutaneous involvement, periorificial lesions, localization in areas of atopic dermatitis or in children with atopic dermatitis (the so-called eczema coxsackium), Gianotti-Crosti-like lesions, petechial/purpuric eruptions, delayed onychomadesis, and palmoplantar desquamation. Finally, adult cases appear to occur with this form of hand-foot-and-mouth disease, likely due to fecal-oral transmission in a household setting. Copyright © 2015 Elsevier Inc. All rights reserved.

  16. Endocrine Diseases

    Science.gov (United States)

    ... to. Featured Topics Adrenal Insufficiency and Addison's Disease Pregnancy & Thyroid Disease Hypothyroidism (Underactive Thyroid) Hashimoto's Disease Hyperthyroidism (Overactive Thyroid) Graves' ...

  17. An unusual case of Sweet syndrome in a child: overlapping presentation with erythema elevatum diutinum.

    Science.gov (United States)

    Wang, Tingting; Liu, Hongjie; Wang, Lin; Guo, Zaipei; Li, Li

    2014-06-01

    In 1964, Sweet described an acute febrile neutrophilic dermatosis. It is now widely accepted that Sweet syndrome is one of the associated neutrophilic dermatoses. Herein, we describe an unusual case of Sweet syndrome in a 5-year-old child who suffered from recurrent papules, plaques, and blisters on his face and trunk after the initial onset of fever without an obvious cause. Two skin biopsies were performed. The histopathological findings of the left arm biopsy showed a subepidermal blister with dense infiltrating neutrophils within the blister that were superficial to the middle layer of the dermis. The biopsy from a plaque on the left leg showed a dense infiltrate of neutrophils and a large number of infiltrating histiocytes superficial to the middle layer of the dermis. Direct immunofluorescence of a skin biopsy from the left arm confirmed that immunoglobulin M (IgM), IgG, IgA, and C3 expression were negative.

  18. 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.

  19. Heart Disease

    Science.gov (United States)

    ... type of heart disease you have. Symptoms of heart disease in your blood vessels (atherosclerotic disease) Cardiovascular disease ... can sometimes be found early with regular evaluations. Heart disease symptoms caused by abnormal heartbeats (heart arrhythmias) A ...

  20. TETANUS—Prophylaxis and Treatment of the Disease

    Science.gov (United States)

    Ross, Donald E.; Kraut, J. J.

    1959-01-01

    Cleansing and debridement is paramount in dealing with tetanus-prone wounds (severe crushing injuries, piercing wounds, blisters and burns are outstanding examples, particularly if contaminated with dirt, grass or other debris). Prophylaxis then is relatively easy in persons who have been actively immunized by toxoid injections. For them, a “booster” injection is indicated. Use of antitoxin, however, is hazardous, whether for prophylaxis or for treatment of the disease. Since it may in itself cause severe disease, including anaphylactic reaction and serum sickness, decision to use it must be weighed against the possibility of the development of tetanus in each case. To prepare for use of it, careful history should be taken, with particular reference to sensitivity to horse dander. Dermal tests, and perhaps ophthalmic tests, for sensitivity to the serum should be carried out. Even the tests may be hazardous and precautions should be taken accordingly. If it is decided that the use of antitoxin is necessary even though the patient is sensitive to the material, desensitization must be carried out promptly, with adequate preparation for severe reaction. There is experimental evidence that antibiotics of the tetracycline group, given soon after injury, may have prophylactic effect against tetanus. PMID:13651954

  1. Central serous chorioretinopathy secondary to corticosteroids in patients with atopic disease.

    Science.gov (United States)

    Ricketti, Peter A; Unkle, David W; Cleri, Dennis J; Prenner, Jonathan L; Coluccielo, Michael; Ricketti, Anthony J

    2015-01-01

    Central serous chorioretinopathy (CSCR) is of unknown etiology and is the most common cause of retinopathy after age-related macular degeneration, diabetic retinopathy, and retinal vein occlusion. Vision loss results from fluid leakage and serous detachment in the macula. Five percent of patients develop chronic CSCR. It is predominantly found in middle-aged men (age-adjusted rates per 100,000: 9.9 for men and 1.7 for women) and is usually unilateral and reversible. Three-quarters of CSCR patients resolve within 3 months but 45% have recurrences, usually with only minor visual acuity changes. Risk factors include type A personality, emotional stress, elevated catecholamines, hypertension, pregnancy, organ transplantation, increased levels of endogenous cortisol, psychopharmacologic medication, use of phosphodiesterase 5 inhibitors, obstructive sleep apnea, Helicobacter pylori infection, or treatment with corticosteroids. Five percent of patients develop chronic disease as a result of subretinal fibrin formation within the blister. CSCR is often bilateral, multifocal, and recurrent, and may be associated with subretinal fibrin formation within the blister. Permanent loss of vision may result from subretinal fibrin-fibrosis with scarring of the macula. Corticosteroid-associated CSCR occurs bilaterally in 20% of patients. Steroid-associated therapy may begin days to years after therapy with any form of drug delivery. We present three atopic patients who presented at various times after oral, inhaled, intranasal, and topical corticosteroid therapy. One patient developed CSCR after three separate types of administration of corticosteroids, which, to our knowledge, has not been observed in the literature.

  2. Outbreak of variant hand-foot-and-mouth disease caused by coxsackievirus A6 in Auckland, New Zealand.

    Science.gov (United States)

    Hayman, Rebecca; Shepherd, Michael; Tarring, Claire; Best, Emma

    2014-10-01

    Hand-foot-and-mouth disease is a common, usually mild childhood illness caused by enteroviruses. Over the last five years, coxsackievirus A6 has been identified as a causative agent in outbreaks in Europe, South-East Asia and America. It has an atypical presentation compared with other enteroviruses, with more widespread rash, larger blisters and subsequent skin peeling and/or nail shedding. We give the first description of an outbreak of coxsackievirus A6 in New Zealand and how health-care communication networks enabled detection of and dissemination of information about this emergent strain. © 2014 The Authors. Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  3. Steroid sparing regimens for management of oral immune-mediated diseases

    Directory of Open Access Journals (Sweden)

    Arti Agrawal

    2014-01-01

    Full Text Available Immune-mediated mucocutaneous disease may present oral symptoms as a first sign of the disease. The primary etiology could be the cellular and/or humoral immune responses directed against epithelial or connective tissue, in a chronic and recurrent pattern. Lichen planus, pemphigus vulgaris and bullous pemphigoid are the most frequent immunologically mediated mucocutaneous diseases. More often than not, patients present with complaints of blisters, oral ulcers, pain, burning sensation, and bleeding from the various oral sites. Steroids, whether topical or systemic, are the treatment of choice as they have both anti-inflammatory and immune-suppressant properties; however, challenges in the treatment of autoimmune diseases are the complexity of symptoms, the need to manage long-term medications for preserving organ function, and the long-term adverse effects of steroids. In such situations steroid sparing agents, such as, tacrolimus, dapsone, azathioprine, cyclosporine, and so on, may be helpful. Here an attempt is made to review various treatment regimens that could be used as alternatives to steroids for management of such diseases.

  4. Persistent release of IL-1s from skin is associated with systemic cardio-vascular disease, emaciation and systemic amyloidosis: the potential of anti-IL-1 therapy for systemic inflammatory diseases.

    Directory of Open Access Journals (Sweden)

    Keiichi Yamanaka

    Full Text Available The skin is an immune organ that contains innate and acquired immune systems and thus is able to respond to exogenous stimuli producing large amount of proinflammatory cytokines including IL-1 and IL-1 family members. The role of the epidermal IL-1 is not limited to initiation of local inflammatory responses, but also to induction of systemic inflammation. However, association of persistent release of IL-1 family members from severe skin inflammatory diseases such as psoriasis, epidermolysis bullosa, atopic dermatitis, blistering diseases and desmoglein-1 deficiency syndrome with diseases in systemic organs have not been so far assessed. Here, we showed the occurrence of severe systemic cardiovascular diseases and metabolic abnormalities including aberrant vascular wall remodeling with aortic stenosis, cardiomegaly, impaired limb and tail circulation, fatty tissue loss and systemic amyloid deposition in multiple organs with liver and kidney dysfunction in mouse models with severe dermatitis caused by persistent release of IL-1s from the skin. These morbid conditions were ameliorated by simultaneous administration of anti-IL-1α and IL-1β antibodies. These findings may explain the morbid association of arteriosclerosis, heart involvement, amyloidosis and cachexia in severe systemic skin diseases and systemic autoinflammatory diseases, and support the value of anti-IL-1 therapy for systemic inflammatory diseases.

  5. Is Dental Implantation Indicated in Patients with Oral Mucosal Diseases

    Directory of Open Access Journals (Sweden)

    Kalogirou Eleni-Marina

    2017-07-01

    Full Text Available Background/Aim: Dental implants are a reliable treatment choice for rehabilitation of healthy patients as well as subjects with several systemic conditions. Patients with oral mucosal diseases often exhibit oral mucosal fragility and dryness, erosions, blisters, ulcers or microstomia that complicate the use of removable dentures and emphasize the need for dental implants. The aim of the current study is to review the pertinent literature regarding the dental implantation prospects for patients with oral mucosal diseases. Material and Method: The English literature was searched through PubMed and Google Scholar electronic databases with key words: dental implants, oral mucosal diseases, oral lichen planus (OLP, epidermolysis bullosa (EB, Sjögren’s syndrome (SS, cicatricial pemphigoid, bullous pemphigoid, pemphigus vulgaris, scleroderma/systemic sclerosis, lupus erythematosus, leukoplakia, oral potentially malignant disorders, oral premalignant lesions, oral cancer and oral squamous cell carcinoma (SCC. Results: Literature review revealed dental implantation in patients with OLP (14 articles, EB (11 articles, pemphigus vulgaris (1 article, SS (14 articles, systemic sclerosis (11 articles, systemic lupus erythematosus (3 articles and oral SCC development associated with leukoplakia (5 articles. No articles regarding dental implants in patients with pemphigoid or leukoplakia without SCC development were identified. Most articles were case-reports, while only a few retrospective, prospective or observational studies were identified. Conclusions: Dental implants represent an acceptable treatment option with a high success rate in patients with chronic mucocutaneous and autoimmune diseases with oral manifestations, such as OLP, SS, EB and systemic sclerosis. Patients with oral possibly malignant disorders should be closely monitored to rule out the development of periimplant malignancy. Further studies with long follow-up, clinical and radiographic

  6. Pneumococcal Disease

    Science.gov (United States)

    ... Pneumococcal disease is a very serious infection that causes pneumonia, meningitis, and bloodstream infection (sepsis). About one million ... when someone gets pneumococcal disease? Pneumococcal disease can cause pneumonia, meningitis, or bloodstream infection. People with pneumococcal disease ...

  7. Autoimmune Diseases

    Science.gov (United States)

    ... autoimmune diseases are rare, while others, such as Hashimoto's disease, affect many people. Who gets autoimmune diseases? ... often occur on both sides of the body. Hashimoto's (hah-shee-MOH-tohz) disease (underactive thyroid) A ...

  8. Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease.

    Science.gov (United States)

    Collin, Pekka; Salmi, Teea T; Hervonen, Kaisa; Kaukinen, Katri; Reunala, Timo

    2017-02-01

    Dermatitis herpetiformis (DH) is an itchy blistering skin disease with predilection sites on elbows, knees, and buttocks. Diagnosis is confirmed by showing granular immunoglobulin A deposits in perilesional skin. DH is one manifestation of coeliac disease; the skin symptoms heal with gluten free diet (GFD) and relapse on gluten challenge. Of the first-degree relatives, 5% may be affected by either condition. Tissue transglutaminase (TG2) is the autoantigen in coeliac disease and epidermal transglutaminase (TG3) in DH. Both diseases conditions exhibit TG2-specific autoantibodies in serum and small bowel mucosa; patients with DH have IgA-TG3 in the skin. There are some divergencies between these two phenotypes. One-fourth of DH patients do not have small bowel mucosal villous atrophy, but virtually all have coeliac-type inflammatory changes. The skin symptoms respond slowly to GFD. The incidence of coeliac disease is increasing, whereas the opposite is true for DH. A female predominance is evident in coeliac disease, while DH may be more common in males. Coeliac disease carries the risk of small intestinal T-cell lymphoma; in DH B-cell lymphomas at any site may prevail. Adult coeliac disease carries a slightly increased elevated mortality risk, whereas in DH, the relative mortality rate is significantly decreased. Key messages Dermatitis herpetiformis is a cutaneous manifestation of coeliac disease; both conditions are genetically determined and gluten-dependent. Gastrointestinal symptoms and the degree of villous atrophy are less obvious in dermatitis herpetiformis than in coeliac disease. Both show tissue transglutaminase (TG2) specific autoantibodies in serum and small bowel mucosa. In addition, TG3-targeted IgA antibodies are found in the skin of DH patients Both conditions carry an increased elevated risk of lymphoma, in coeliac disease small intestinal T-cell lymphoma, in dermatitis herpetiformis mainly B-cell lymphoma at various sites. Coeliac disease is

  9. Behcet's Disease

    Science.gov (United States)

    ... are here: Home / Types of Vasculitis / Behcet’s Disease Behcet’s Disease First Description Who gets Behcet’s Disease (the “typical” ... for Behcet’s Disease is Behcet’s syndrome . Who gets Behcet’s Disease (the “typical” patient)? Behcet’s disease is most common ...

  10. Frequency of some acropodium diseases in dairy cows in Serbia

    Directory of Open Access Journals (Sweden)

    Milosavljević P.

    2013-01-01

    Full Text Available The research included 520 dairy cows on 64 mini-farms in mountainous Serbian areas, with the goal to acquire an insight on acropodium diseases frequency in dairy cows, as well as distribution of hoof alterations. In 56.25% of farms, animals were kept tied in the stable, while 43.75% were grazing regularly or occasionally. Only 10.94% of farms were free of hoof acropodium alterations. Extremely bad hygienic conditions were noted in 18.75% of farms, where 74.01% of animals had acropodium lesions. Based on clinical analysis of 520 dairy cows, 388 of them were diagnosed with acropodium alternations: overgrown hooves (in 21.91% of animals, shoe-like hooves (7.22%, scissors-like (17.27% and spiral hooves (8.76%. Wounded acropodium skin was diagnosed in 1.8%, interdigital phlegmon in 9.02%, aseptic pododermatitis in 3.61%, digital dermatitis in 4.89%, interdigital dermatitis in 3.09%, hoof ulcer in 3.35%, interdigital blister in 4.89%, hollow hoof wall in 6.18%, horn wall rupture in 0.51%, heel abscess in 3.09%, tendovaginitis in 3.85% and crown joint displacement in 0.26% of animals. We found no pathological lesions of the acropodium in 132 animals (25.38%.

  11. Valve Disease

    Science.gov (United States)

    ... See also on this site: Diseases of the Mitral Valve Diseases of the Aortic Valve Diseases of the Tricuspid ... most invasive option for the treatment of valve disease. During surgery, ... defects of the mitral valve. Replacement is used to treat any diseased ...

  12. Kawasaki Disease

    Science.gov (United States)

    Kawasaki disease is a rare childhood disease. It makes the walls of the blood vessels in the ... veins, and capillaries. No one knows what causes Kawasaki disease. Symptoms include High fever that lasts longer ...

  13. Whipple's Disease

    Science.gov (United States)

    ... way to prevent Whipple disease. Eating, Diet, and Nutrition A person with Whipple disease and malabsorption may need a diet high in calories and protein vitamins nutritional supplements People with Whipple disease should discuss their nutritional ...

  14. Celiac Disease

    Science.gov (United States)

    ... diabetes, rheumatoid arthritis, autoimmune thyroid or liver disease, Addison’s disease, or Sjogren’s syndrome.Have a genetic disorder such ... results will confirm that you have celiac disease. Diagnosis of dermatitis herpetiformis with a positive blood test ...

  15. Alzheimer's Disease

    Science.gov (United States)

    Alzheimer's disease (AD) is the most common form of dementia among older people. Dementia is a brain disorder that ... higher if a family member has had the disease. No treatment can stop the disease. However, some ...

  16. Huntington's Disease

    Science.gov (United States)

    Huntington's disease (HD) is an inherited disease that causes certain nerve cells in the brain to waste ... express emotions. If one of your parents has Huntington's disease, you have a 50 percent chance of ...

  17. Alzheimer Disease

    Science.gov (United States)

    ... CPR: A Real Lifesaver Kids Talk About: Coaches Alzheimer Disease KidsHealth > For Kids > Alzheimer Disease Print A A ... slow it down. When Someone You Love Has Alzheimer Disease You might feel sad or angry — or both — ...

  18. Moyamoya Disease

    Science.gov (United States)

    ... Page You are here Home » Disorders » All Disorders Moyamoya Disease Information Page Moyamoya Disease Information Page What research is being done? ... Definition Treatment Prognosis Clinical Trials ... Publications Definition Moyamoya disease is a rare, progressive cerebrovascular disorder caused ...

  19. Crohn's Disease

    Science.gov (United States)

    Crohn's disease causes inflammation of the digestive system. It is one of a group of diseases called inflammatory ... small intestine called the ileum. The cause of Crohn's disease is unknown. It may be due to an ...

  20. Wilson Disease

    Science.gov (United States)

    ... Liver Foundation March of Dimes National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) Wilson Disease Association See all related organizations Publications Order NINDS Publications Definition Wilson disease (WD) is a rare inherited disorder ...

  1. Ribbing disease

    Directory of Open Access Journals (Sweden)

    Mukkada Philson

    2010-01-01

    Full Text Available Ribbing disease is a rare sclerosing dysplasia that involves long tubular bones, especially the tibia and femur. It occurs after puberty and is reported to be more common in women. In this article we describe how Ribbing disease can be differentiated from diseases like Engelmann-Camurati disease, van Buchem disease, Erdheim-Chester disease, osteoid osteoma, chronic osteomyelitis, stress fracture, etc.

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

    Directory of Open Access Journals (Sweden)

    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. Epidermolysis bullosa pruriginosa a clinico-pathological study in an index case, highlighting its affliction in 11 of 27 member of the family

    Directory of Open Access Journals (Sweden)

    Manisha Nijhawan

    2017-07-01

    Full Text Available Epidermolysis bullosa pruriginosa, a rare variant of dominant inheritance pattern, characterized by itchy, papules and/or nodules coalescing to form plaque(s, studded by blisters, is describe in a young man an index case. In all 12 afflicted individuals of the 27 family members was interesting, the diagnosis of which was made by its morphology and inflammatory changes in the epidermis, comprising hyperkeratosis, moderate acanthosis, presence of inflammatory cells, and above all inflammatory cell containing sub-epidermal cleavage or cleft.

  4. Reportable diseases

    Science.gov (United States)

    ... Listeriosis Lyme disease Malaria Measles Meningitis (meningococcal disease) Mumps Novel influenza A virus infections Pertussis Plague Poliomyelitis Poliovirus infection, nonparalytic Psittacosis ...

  5. Clinical and demographic overlaps among immunologically mediated oral diseases: a challenge for clinicians.

    Science.gov (United States)

    do Carmo, Maria Auxiliadora Vieira; Gleber-Netto, Frederico Omar; Romano, Maria Luisa de Freitas; Caldeira, Patricia Carlos; de Aguiar, Maria Cassia Ferreira

    2014-01-01

    This study sought to assess and compare retrospective demographic and clinical data of oral lesions of lichen planus, pemphigus vulgaris, and mucous membrane pemphigoid from the records of the Department of Oral Pathology and Surgery, School of Dentistry, Universidade Federal de Minas Gerais, Brazil, covering a period of 55 years. Out of 25,435 specimens, these immunologically-mediated diseases accounted for 301 (1.18%) cases, of which 250 (0.98%) were lichen planus, 27 (0.11%) were pemphigus vulgaris, and 24 (0.09%) were mucous membrane pemphigoid. Lichen planus presented mainly as white asymptomatic plaques on buccal mucosa. Pemphigus vulgaris was usually characterized by multiple symptomatic erithematous ulcers on the buccal mucosa. Painful ulcers and/or blisters on the gingiva were the most common presentation for mucous membrane pemphigoid. Desquamative gingivitis was noted for all 3 diseases, but mainly for mucous membrane pemphigoid. Overall, lesions were more frequent in white women >50 years. Oral manifestations of immunologically-mediated diseases are relatively rare, and the correct diagnosis can be a challenge for dentists as the lesions often share similar clinical and demographic features.

  6. [Epidermolysis bullosa].

    Science.gov (United States)

    García Pérez, A

    1999-01-01

    The epidermolysis bullosa are a group of genodermatoses in which there is congenital fragility of the skin which produce blisters with the least of traumas. The group includes up to thirty clinical-genetical entities. They are classified according to the level where the blister is found into simplex or epidermolytic (intraepitelial blister), junctional (blister in the dermoepidermal junction) and dystrophic or dermolytic (subepidermal blister) epidermolysis bullosa. The symptoms, classic or Mendelian genetics and the recent findings in the most frequent forms of each one of these groups are reviewed. In most of them, the gene that produces the mutations and the chromosome in which its locus is found are known. In the simplex forms, the disorders lies in the genes that codify the different keratins. In the junctional ones, mutations are found in the laminin genes that act in the development of the anchoring filaments or in those of the other components of the basement membrane. In the dystrophic ones, which are the most serious, the mutations affect the collagen VII gene that codify the development of the anchoring fibrils. There is no treatment for any of the forms of epidermolysis bullosa. It is possible that the future advances in genetic engineering can contribute to their prophylaxis.

  7. Is it lucio phenomenon or necrotic erythema nodosum leprosum?

    Directory of Open Access Journals (Sweden)

    PSS Ranugha

    2013-01-01

    Full Text Available Lucio phenomenon (LP or erythema necroticans is a relatively rare, peculiar reaction pattern occurring in untreated lepromatous (LL or borderline lepromatous (BL leprosy cases. A 38-year-old male, a cook by occupation, was referred to the dermatology clinic from otolaryngology department with blistering over both the hands and feet of 2 days duration. He had been admitted 1 week back with epistaxis and nasopharyngeal myiasis in otolaryngology department. He was started on systemic antibiotics gentamycin, crystalline penicillin, and metronidazole with nasal instillation of turpentine oil 2 drops 6 times a day. Two days later, he had developed edema with painless hemorrhagic blistering over the dorsum of left hand followed by involvement of the right hand, dorsa of both feet, and both the earlobes within a day. Histopathology of the blister showed sub-epidermal blister, with necrotizing leukocytoclastic vasculitis of papillary dermal vessels with thrombosis, numerous acid-fast bacilli in macrophages, and macrophage granulomas extending up to subcutis. In view of the absent fever or constitutional symptoms, and the classical angular infarcts and hemorrhagic blisters evolving into ulcers with angulated margins, we considered LP as the most likely diagnosis. The patient was started on a combination of WHO recommended multibacillary anti-leprosy therapy and prednisolone (40 mg/day.

  8. Heart Diseases

    Science.gov (United States)

    ... you're like most people, you think that heart disease is a problem for others. But heart disease is the number one killer in the ... of disability. There are many different forms of heart disease. The most common cause of heart disease ...

  9. [Social diseases, civilization diseases or lifestyle diseases?].

    Science.gov (United States)

    Betlejewski, Stansław

    2007-01-01

    In general, the development of civilization is viewed as a positive step for the well-being of the human species, leading to an increased duration and quality of life. The accelerated progress of civilization (mainly industrialization, urbanization and nutrition) has lead to new possibilities for adverse effects on human health. In former high civilization--like old Egypt, Greece, Roman, Chinese, Indian, Maya civilizations--the "modem civilization diseases" were unknown. Modem science through improved sanitation, vaccination and antibiotics as well as improved social and economical conditions, has eliminated the threat of death from most infectious diseases. In the years after World War II the social, economic and health conditions changed. Most deaths have resulted from heart disease, stroke, cancer and other diseases as a result of an inappropriate relationship of people with their environment and changed lifestyle. Lifestyle diseases are different from other diseases because they are potentially preventable and can be lowered with changes in diet, lifestyle and environment.

  10. Misleading pustular plaques of the lower limbs during Crohn's disease: two case reports

    Directory of Open Access Journals (Sweden)

    Farhi David

    2007-10-01

    Full Text Available Abstract Background Extraintestinal manifestations of Crohn's disease may involve the skin, the eyes, the genital mucosa, and the joints. Dermatoses associated with Crohn's disease include neutrophilic dermatoses, erythema nodosum, granulomatous dermatitis, blistering dermatoses, and non-specific skin manifestations. Cutaneous Crohn's disease is characterized by skin non-caseating epithelioid granulomatas with giant cells, remote from the gastrointestinal tract. We report herein two new cases. Observations On both patients, differential diagnosis of neutrophilic dermatoses and infectious disease were evoked, and antimicrobial agents were introduced in one of them. Given the atypical presentation, the final diagnosis of cutaneous Crohn's disease could only be made with histological examination. In patient 1, the plaques decreased in size and infiltration by more than 75% after 3 weeks of treatment with bethametasone dipropionate 0.05% cream. In patient 2, the plaques decreased by more than 50% after 6 weeks of treatment with prednisolone (45 mg/day and azathioprine (100 mg/day. Discussion Cutaneous Crohn's disease may present as dusky, erythematous, infiltrated, and ulcerated plaques and nodules. Female-to-male sex ratio is about 2, and the mean age at onset is 35. Recurrently, the hypothesis of a skin mycobacterial or fungal infection greatly delays proper treatment. Rarity of cutaneous Crohn's disease hampers therapeutic assessment in controlled trials. Thus, available literature is limited to case reports and sparse small series, with contradictory results. These reports are subject to publication bias, and no definite evidence-based recommendations can be made on the most adequate therapeutic strategy.

  11. Kimura disease

    Science.gov (United States)

    AlGhamdi, Fares E.; Al-Khatib, Talal A.; Marzouki, Hani Z.; AlGarni, Mohammed A

    2016-01-01

    Kimura disease is a chronic inflammatory disease that mainly manifests as a lump in the cervical region. Although the underlying pathophysiology is not clear yet, the diagnosis can be established based on specific histopathological characteristics. The first case of this disease was described in China, as well as the majority of subsequent cases that were also described in the Far East countries made Kimura disease traditionally a disease of adult patients of Asian descent. This report describes the occurrence of Kimura disease in pediatric non-Asian patient with a similar clinicopathologic presentation. PMID:26905356

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

    Directory of Open Access Journals (Sweden)

    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

  13. Development of a simple enzyme-linked immunosorbent assay for the detection of autoantibodies in anti-p200 pemphigoid.

    Science.gov (United States)

    Groth, S; Recke, A; Vafia, K; Ludwig, R J; Hashimoto, T; Zillikens, D; Schmidt, E

    2011-01-01

    Anti-p200 pemphigoid is a subepidermal blistering skin disease characterized by autoantibodies against a 200-kDa protein (p200) of the dermal-epidermal junction. The laminin γ1 chain has recently been identified as target antigen in this disease and the C-terminus was described as an immunodominant region of laminin γ1. Diagnosis of anti-p200 pemphigoid requires detection of serum IgG at the dermal side of 1 mol L(-1) salt-split skin by indirect immunofluorescence microscopy and labelling of a 200-kDa protein by Western blotting of dermal extract. However, preparation of dermal extract is not widely available, limiting the possibility of diagnosing this disease to a few laboratories. To develop a simple, sensitive and specific diagnostic tool for anti-p200 pemphigoid. Sera from patients with anti-p200 pemphigoid (n = 35), bullous pemphigoid (BP, n = 101), epidermolysis bullosa acquisita (EBA, n = 10), antilaminin 332 mucous membrane pemphigoid (MMP, n = 14), pemphigus vulgaris (PV, n = 51) and healthy volunteers (HV, n = 131) were tested by a novel enzyme-linked immunosorbent assay (ELISA) that employed a recombinant monomeric C-terminal fragment of human laminin γ1 (hLAMC1-cterm) expressed in Escherichia coli. Serum reactivity with hLAMC1-cterm was detected in sera from 24 of 35 (69%) patients with anti-p200 pemphigoid, two of 101 (2%) with BP, 0 of 10 with EBA, two of 14 (14%) with anti-laminin 332 MMP, 0 of 51 with PV, and 0 of 131 HV. This novel ELISA will facilitate the diagnosis of anti-p200 pemphigoid. © 2010 The Authors. BJD © 2010 British Association of Dermatologists 2010.

  14. Digestive Diseases

    Science.gov (United States)

    ... Control Problems (Fecal Incontinence) Gas Lactose Intolerance Diarrhea Diverticulosis & Diverticulitis Acid Reflux (GER & GERD) More Digestive Disease ... Polyps Constipation Crohn's Disease Cyclic Vomiting ... and Diverticulitis Dumping Syndrome Foodborne Illnesses Gallstones Gas ...

  15. Heart Disease

    Science.gov (United States)

    ... Awareness Day National Women's Health Week Supporting Nursing Moms at Work Popular Topics Autoimmune diseases Breastfeeding Carpal tunnel syndrome ... Awareness Day National Women's Health Week Supporting Nursing Moms at Work Popular Topics Autoimmune diseases Breastfeeding Carpal tunnel syndrome ...

  16. Schilder's Disease

    Science.gov (United States)

    ... is not the same as Addison-Schilder disease (adrenoleukodystrophy). Symptoms may include dementia, aphasia, seizures, personality changes, ... is not the same as Addison-Schilder disease (adrenoleukodystrophy). Symptoms may include dementia, aphasia, seizures, personality changes, ...

  17. Coeliac disease

    African Journals Online (AJOL)

    2013-03-08

    . Coeliac disease, often called coeliac sprue, is an autoimmune disorder of the small intestine which occurs in genetically predisposed people. Coeliac disease is not an allergy or intolerance to gluten. It can present at all ages,.

  18. Parkinson's Disease

    Science.gov (United States)

    Parkinson's disease (PD) is a type of movement disorder. It happens when nerve cells in the brain don't ... coordination As symptoms get worse, people with the disease may have trouble walking, talking, or doing simple ...

  19. Addison Disease

    Science.gov (United States)

    ... blood pressure and water and salt balance. Addison disease happens if the adrenal glands don't make ... problem with your immune system usually causes Addison disease. The immune system mistakenly attacks your own tissues, ...

  20. Raynaud's Disease

    Science.gov (United States)

    Raynaud's disease is a rare disorder of the blood vessels, usually in the fingers and toes. It causes the ... secondary Raynaud's, which is caused by injuries, other diseases, or certain medicines. People in colder climates are ...

  1. Wilson Disease

    Science.gov (United States)

    Wilson disease is a rare inherited disorder that prevents your body from getting rid of extra copper. You need ... copper into bile, a digestive fluid. With Wilson disease, the copper builds up in your liver, and ...

  2. Fifth Disease

    Science.gov (United States)

    Fifth disease is a viral infection caused by parvovirus B19. The virus only infects humans; it's not the same parvovirus that dogs and cats can get. Fifth disease mostly affects children. Symptoms can include a low ...

  3. Meniere's Disease

    Science.gov (United States)

    Meniere's disease is a disorder of the inner ear. It can cause severe dizziness, a roaring sound in your ... together over several days. Some people with Meniere's disease have "drop attacks" during which the dizziness is ...

  4. Gaucher Disease

    Science.gov (United States)

    Gaucher disease is a rare, inherited disorder. It is a type of lipid metabolism disorder. If you have it, ... It usually starts in childhood or adolescence. Gaucher disease has no cure. Treatment options for types 1 ...

  5. Kidney Diseases

    Science.gov (United States)

    ... urine until you go to the bathroom. Most kidney diseases attack the nephrons. This damage may leave kidneys ... or medicines. You have a higher risk of kidney disease if you have diabetes, high blood pressure, or ...

  6. Parkinson disease

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000755.htm Parkinson disease To use the sharing features on this page, please enable JavaScript. Parkinson disease causes certain brain cells to die. These ...

  7. Legionnaire disease

    Science.gov (United States)

    ... disease is less severe. Risk factors include: Alcohol abuse Cigarette smoking Chronic illnesses, such as kidney failure ... Antibiotics are used to fight the infection. Treatment is started as soon as Legionnaire disease is suspected, ...

  8. Liver disease

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000205.htm Liver disease To use the sharing features on this page, please enable JavaScript. The term "liver disease" applies to many conditions that stop the ...

  9. Liver Diseases

    Science.gov (United States)

    Your liver is the largest organ inside your body. It helps your body digest food, store energy, and remove poisons. There are many kinds of liver diseases: Diseases caused by viruses, such as hepatitis ...

  10. Lyme Disease

    Science.gov (United States)

    ... can also spread to the nervous system, causing facial paralysis ( Bell's palsy ), or meningitis. The last stage of ... symptoms, joint pain or a swollen joint, or facial paralysis. How Is Lyme Disease Treated? Lyme disease is ...

  11. Liver Disease

    Science.gov (United States)

    ... from one or both of your parents can cause various substances to build up in your liver, resulting in liver damage. Genetic liver diseases include: Hemochromatosis Hyperoxaluria and oxalosis Wilson's disease Cancer and other growths Examples include: Liver cancer Bile ...

  12. Whipple Disease

    Science.gov (United States)

    ... ed. Sleisenger and Fordtran’s Gastrointestinal and Liver Disease: Pathophysiology/ Diagnosis/Management. 9th ed. Philadelphia: Saunders; 2010: 1833– ... treatment. Hope through Research The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) conducts and ...

  13. Alexander Disease

    Science.gov (United States)

    ... may be other genetic or perhaps even non-genetic causes of Alexander disease. Current research is aimed at understanding the mechanisms by which the mutations cause disease, developing better animal models for the disorder, and exploring potential strategies ...

  14. Alpers' Disease

    Science.gov (United States)

    ... Alpers-like" phenotype without liver disease have POLG mutations. × Definition Alpers' disease is a progressive, neurodevelopmental, mitochondrial DNA depletion syndrome characterized by three co-occurring clinical ...

  15. Farber's Disease

    Science.gov (United States)

    ... SEARCH Definition Treatment Prognosis Clinical Trials Organizations Publications Definition Farber’s disease, also known as Farber's lipogranulomatosis, describes a group of inherited metabolic disorders called lipid storage diseases, in which excess amounts of lipids ( ...

  16. Graves disease

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000358.htm Graves disease To use the sharing features on this page, please enable JavaScript. Graves disease is an autoimmune disorder that leads to an ...

  17. Buerger's Disease

    Science.gov (United States)

    ... is linked to the development of Buerger's disease. Sex Buerger's disease is far more common in males ... Clinic does not endorse any of the third party products and services advertised. Advertising and sponsorship policy ...

  18. Meniere's Disease

    Science.gov (United States)

    ... of Meniere's disease isn't understood. One popular theory that hasn't been proved is that Meniere's ... Medical Education and Research; 2015. Foster CA. Optimal management of Meniere's disease. Therapeutics and Clinical Risk Management. ...

  19. Fungal Diseases

    Science.gov (United States)

    ... Patients Medications that Weaken Your Immune System Outbreaks Rhizopus Investigation CDC at Work Global Fungal Diseases Cryptococcal ... September 6, 2017 Content source: Centers for Disease Control and Prevention National Center for Emerging and Zoonotic ...

  20. Chagas Disease

    Science.gov (United States)

    Chagas disease is caused by a parasite. It is common in Latin America but not in the United ... There are no vaccines or medicines to prevent Chagas disease. If you travel to areas where it occurs, ...

  1. Disease: H00644 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00644 Ectodermal dysplasia/skin fragility syndrome Ectodermal dysplasia/skin fragility... syndrome is a very rare genodermatosis that develops skin fragility with tearing and blisters and conge...945625 (gene, desription) McGrath JA, Mellerio JE Ectodermal dysplasia-skin fragility syndrome. Dermatol Cli...Garrod DR, Eady RA Mutations in the plakophilin 1 gene result in ectodermal dysplasia/skin fragility...n G, Fassihi H, Chan I, Paller AS, Surucu S, McGrath JA Ectodermal dysplasia-skin fragility syndrome resulti

  2. Kidney Disease

    Science.gov (United States)

    ... Breath? Talking to Your Parents - or Other Adults Kidney Disease KidsHealth > For Teens > Kidney Disease Print A A A What's in this article? ... uh-jist), a doctor who specializes in treating kidney diseases. The doctor will ask you about any concerns ...

  3. Legionnaires' Disease

    Science.gov (United States)

    ... get sick if you Are older than 50 Smoke Have a chronic lung disease Have a weak immune system Legionnaires' disease is serious and can be life-threatening. However, most people recover with antibiotic treatment. Centers for Disease Control and Prevention

  4. Lyme Disease

    Science.gov (United States)

    ... common are at higher risk of getting tick-borne diseases. How is Lyme disease diagnosed? It can be ... If clothes are dirty, wash them in hot water and dry on high heat for 60 minutes. Lyme disease treatment What do I do if I find ...

  5. Infectious Diseases

    International Development Research Centre (IDRC) Digital Library (Canada)

    GeneratinG knowledGe. IDRC-supported researchers have successfully applied ecohealth approaches to produce knowledge on the root causes of infectious diseases worldwide. Fighting chagas disease in guatemala. Chagas disease is a serious infection transmitted from animals to humans by a reduvidae bug. In.

  6. Lyme Disease.

    Science.gov (United States)

    Taylor, George C.

    1991-01-01

    This overview of the public health significance of Lyme disease includes the microbiological specifics of the infectious spirochete, the entomology and ecology of the ticks which are the primary disease carrier, the clinical aspects and treatment stages, the known epidemiological patterns, and strategies for disease control and for expanded public…

  7. The Speed of Invasion: Rates of Spread for Thirteen Exotic Forest Insects and Diseases

    Directory of Open Access Journals (Sweden)

    Alexander M. Evans

    2016-05-01

    Full Text Available Invasive, exotic insects and diseases have a devastating effect on North American forests. The rate of spread, or range expansion, is one of the main determinants of an invasive organism’s impact, and can play a major role in structuring management response options. To better understand how exotic organisms have spread through our forests, this study employs a consistent, rigorous analytical framework to analyze a comprehensive geospatial database for the spread of seven exotic insects and six diseases. This study includes new data for six insects and two diseases in combination with five invasive species previously analyzed using the same technique. The quantile regression analysis of over 3000 records of infestation over the preceding century show that the rate of spread of invasive forest insects and diseases ranges from 4.2 km·year−1 to 57.0 km·year−1. The slowest disease spread was white pine blister rust (Cronartium ribicola at 7.4 km·year−1 while the most rapid disease spread was chestnut blight (Cryphonectria parasitica at 31.3 km·year−1. The slowest insect spread was balsam woolly adelgid (Adelges piceae (4.2 km·year−1 while the fastest was emerald ash borer (Agrilus planipennis at 57.0 km·year−1. Species that can fly long distances or are vectored by flying insects have spread faster than those that are passively dispersed. This analysis highlights the difficulty of estimating spread rates from studies of individual dispersal or flight distances, but the estimated spread rates in this study are generally in line with previous estimates.

  8. Lyme Disease

    OpenAIRE

    Ozdemir, Davut; İnce, Nevin

    2015-01-01

    Lyme disease (LD) is caused by the spirochete, Borrelia burgdorferi sensu lato complex. Humans are infected by a tick bite to the skin. This disease is a non-contagious infectious disease. It has been known since the 19th century. LD has a worldwide distribution. It is endemic in Europe, North and South America. There are case reports since 1990 in Turkey. The clinical presentation varies depending on the stage of the disease. Lyme disease is classified into three stages: early localized dise...

  9. Kinetics of the conversion of copper sulfide to blister copper

    Directory of Open Access Journals (Sweden)

    Carrillo, F.

    2002-10-01

    Full Text Available The desulfurization of copper sulfide by air and oxygen has been studied in two laboratory reactors where the gas is blown onto the melt surface. Rates of oxidation in a vertical resistance furnace may be explained by the mass transfer control in the gas phase. However, results for a horizontal tube suggest that the chemical resistance is controlling.

    La desulfuración del sulfuro cuproso con aire y oxígeno se ha estudiado en dos reactores de laboratorio, en los cuales el gas se sopla sobre la superficie del fundido. La velocidad de reacción en un horno de resistencias verticales se puede explicar considerando como controlante la resistencia a la transferencia de materia de la fase gas. Sin embargo, los resultados del horno horizontal indican que la resistencia química es la controlante.

  10. Microbiological Quality of Blister Pack Tablets in Community ...

    African Journals Online (AJOL)

    materials, packaging materials, personnel that come in contact with the product during the manufacturing process ... Twenty two different brands of tablet dosage forms that contained 8 pharmaceutical active ingredients were ... and their drugs approved by the Jordan Food and Drug Administration (JFDA). For each brand,.

  11. "Blister beetles" and cantharidin | Tagwireyi | Zimbabwe Science News

    African Journals Online (AJOL)

    Beetles or, scientifically, Coleoptera are a common sight in the outdoor life of an average Zimbabwean. These insects are generally viewed as friendly, docile, non-biting or non-stinging insects, which can be handled without any untoward effects on the handler. Unfortunately not all beetles fall within this generalisation.

  12. Blistering rash in a young male child | Sanders | South African ...

    African Journals Online (AJOL)

    ... lower limbs. He had visited three different general practitioners prior to presentation and had been given several drugs and ointments, including three different classes of antibiotics. During his admission, the diagnosis of linear IgA bullous dermatitis was made. Dapsone and prednisone were given and he recovered well.

  13. OBTENCION DIRECTA DE COBRE BLISTER EN REACTOR DE CAPA FUNDIDA

    OpenAIRE

    WILKOMIRSKY F., IGOR

    2009-01-01

    El proyecto logró diseñar, construir y operar un reactor que puede ser utilizado tanto en las etapas de fusión como de conversión en el proceso pirometalúrgico de producción primaria de cobre a partir de concentrados sulfurados. El concepto de reactor desarrollado combina dos características innovadoras respecto de la tecnología estándar. El primero se refiere a una operación a temperaturas sensiblemente más altas que los procesos convencionales y el segundo a la zona donde se producen las...

  14. Combined therapeutic use of oral alitretinoin and narrowband ultraviolet-B therapy in the treatment of Hailey-Hailey disease

    Directory of Open Access Journals (Sweden)

    Kaitlin A. Vanderbeck

    2014-11-01

    Full Text Available Hailey-Hailey disease (HHD is a chronic familial bullous disease characterized by recurrent blisters and erosions typically at friction-prone areas of the body accompanied by acantholysis upon histologic examination. There are a number of therapies used in the management of HHD. Its symptoms have been effectively treated with antimicrobial therapies, corticosteroids and other agents such as cyclosporine and prednisone. However, such treatments are not always effective. Therefore, there is a need for new treatments for the management of HHD. In this report, a patient with long-standing HHD responsive only to high levels of prednisone is described. After the successful tapering and cessation of oral prednisone the patient began a new combination therapy of complementary doses of oral alitretinoin, and narrowband UVB therapy, which yielded a favorable response within 2-3 weeks. After 6 weeks, a mono-therapy of daily (30 mg oral alitretinoin was sufficient to maintain successful near-complete remission of the disease.

  15. Sensitive and specific assays for routine serological diagnosis of epidermolysis bullosa acquisita.

    Science.gov (United States)

    Komorowski, Lars; Müller, Ralf; Vorobyev, Artem; Probst, Christian; Recke, Andreas; Jonkman, Marcel F; Hashimoto, Takashi; Kim, Soo-Chan; Groves, Richard; Ludwig, Ralf J; Zillikens, Detlef; Stöcker, Winfried; Schmidt, Enno

    2013-03-01

    Epidermolysis bullosa acquisita (EBA) is a severe autoimmune subepidermal blistering disease characterized by autoantibodies against the N-terminal collagenous domain (NC1) of type VII collagen (Col VII). Development of reliable assays for the detection of anti-Col VII-NC1 antibodies. NC1 was expressed in human HEK293 cells and used as target antigen in an enzyme-linked immunosorbent assay (ELISA) and in an immunofluorescence assay (IFA). These two assays were probed in a large cohort of patients with EBA (n = 73), bullous pemphigoid (BP, n = 72), anti-p200 pemphigoid (n = 24), anti-laminin 332 mucous membrane pemphigoid (MMP, n = 15), pemphigus vulgaris (PV, n = 24), and healthy control subjects (n = 254). The cut-off for the ELISA was optimized for accuracy by receiver-operating characteristics (area under the curve [AUC] = 0.9952). IgG reactivity against NC1 was detected in 69 of 73 EBA (94.5%) and 5 control sera (2 healthy controls and 3 BP patients), resulting in a specificity of 98.7%. The IFA showed a sensitivity of 91.8% and specificity of 99.8%. Reproducibility of the ELISA was demonstrated by an intra-class correlation coefficient of 0.97. IgG subclass analyses by ELISA revealed IgG1, IgG2, IgG3, and IgG4 anti-NC1 reactivity in 83.6%, 85.3%, 37.7%, and 83.6% of EBA sera, respectively. The novel assays were not evaluated prospectively and their use in monitoring serum levels during the disease course was not tested. The two assays are highly specific and sensitive to diagnose EBA. Their diagnostic competence was demonstrated in a large cohort of well-characterized EBA sera. Copyright © 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  16. Serum autoantibodies against the dermal-epidermal junction in patients with chronic pruritic disorders, elderly individuals and blood donors prospectively recruited.

    Science.gov (United States)

    van Beek, N; Dohse, A; Riechert, F; Krull, V; Recke, A; Zillikens, D; Schmidt, E

    2014-04-01

    Bullous pemphigoid (BP) is a subepidermal blistering autoimmune disease characterized by autoantibodies against two structural proteins of the epidermal basal membrane zone (BMZ), BP180 (type XVII collagen) and BP230. Patients are usually old and suffer from severe pruritus. Advanced age and severe pruritus have been hypothesized as potential risk factors for the development of autoantibodies in BP. To prospectively determine anti-BMZ antibodies in sera from patients with advanced age and/or pruritus compared with regular blood donors. Sera from (i) patients with chronic pruritic skin disorders (PSD, n = 78; mean age 62 years), (ii) patients with noninflammatory skin disease aged ≥ 70 years (n = 93; mean age 78 years), and (iii) blood donors (n = 50; mean age 41 years) were included. A large panel of validated test systems used for routine diagnosis were employed comprising indirect immunofluorescence (IF) microscopy on monkey oesophagus and human salt-split skin, BP180 NC16A- and BP230-specific enzyme-linked immunosorbent assay (ELISA) systems, and immunoblotting with various substrates, including LAD-1 (the soluble ectodomain of BP180), BP180, BP230, laminin 332, p200 antigen, laminin γ1 and type VII collagen. No statistically significant difference was seen between the three study groups. The same result was obtained when data for IF microscopy, ELISA and immunoblotting were analysed separately. Neither advanced age nor chronic pruritus have been verified as risk factors for autoantibodies against the epidermal BMZ. © 2014 British Association of Dermatologists.

  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. [Tangier disease].

    Science.gov (United States)

    Saku, K; Jimi, S; Ohta, T; Arakawa, K

    1994-12-01

    Tangier disease, a familial HDL-deficiency syndrome, was first reported by Fredrickson et al. in 1961. Since then, a great deal research on the mechanism of HDL-deficiency in this disease has been done from the prospective of lipoprotein compositions, in vivo HDL kinetics, cell and receptor interactions and genomic DNA analysis. These studies have found a relatively increased fractional catabolic rate of apo HDL with essentially no change in the synthesis of apo HDL. As yet, no structural defect has been found in the apo A-I gene. However, there have been reports of irregular lipid metabolism and of disorders in the Golgi apparatus and lysosome processing. Interestingly, the frequency of coronary heart disease is low in patients with Tangier disease. In this report, we describe the clinical and biochemical characteristics of Tangier disease and suggest some possible mechanisms for preventing coronary heart disease.

  19. Morgellons Disease

    OpenAIRE

    Ohn, Jungyoon; Park, Seon Yong; Moon, Jungyoon; Choe, Yun Seon; Kim, Kyu Han

    2017-01-01

    Morgellons disease is a rare disease with unknown etiology. Herein, we report the first case of Morgellons disease in Korea. A 30-year-old woman presented with a 2-month history of pruritic erythematous patches and erosions on the arms, hands, and chin. She insisted that she had fiber-like materials under her skin, which she had observed through a magnifying device. We performed skin biopsy, and observed a fiber extruding from the dermal side of the specimen. Histopathological examination sho...

  20. Infectious disease

    Science.gov (United States)

    Pierson, Duane L.

    1990-01-01

    This is a collection of viewgraphs on the Johnson Space Center's work on infectious disease. It addresses their major concern over outbreaks of infectious disease that could jeopardize the health, safety and/or performance of crew members engaged in long duration space missions. The Antarctic environment is seen as an analogous location on Earth and a good place to carry out such infectious disease studies and methods for proposed studies as suggested.

  1. Celiac disease

    Directory of Open Access Journals (Sweden)

    Radlović Nedeljko

    2013-01-01

    Full Text Available Celiac disease is a multysystemic autoimmune disease induced by gluten in wheat, barley and rye. It is characterized by polygenic predisposition, high prevalence (1%, widely heterogeneous expression and frequent association with other autoimmune diseases, selective deficit of IgA and Down, Turner and Williams syndrome. The basis of the disease and the key finding in its diagnostics is symptomatic or asymptomatic inflammation of the small intestinal mucosa which resolves by gluten-free diet. Therefore, the basis of the treatment involves elimination diet, so that the disorder, if timely recognized and adequately treated, also characterizes excellent prognosis.

  2. Celiac disease

    Directory of Open Access Journals (Sweden)

    Holtmeier Wolfgang

    2006-03-01

    Full Text Available Abstract Celiac disease is a chronic intestinal disease caused by intolerance to gluten. It is characterized by immune-mediated enteropathy, associated with maldigestion and malabsorption of most nutrients and vitamins. In predisposed individuals, the ingestion of gluten-containing food such as wheat and rye induces a flat jejunal mucosa with infiltration of lymphocytes. The main symptoms are: stomach pain, gas, and bloating, diarrhea, weight loss, anemia, edema, bone or joint pain. Prevalence for clinically overt celiac disease varies from 1:270 in Finland to 1:5000 in North America. Since celiac disease can be asymptomatic, most subjects are not diagnosed or they can present with atypical symptoms. Furthermore, severe inflammation of the small bowel can be present without any gastrointestinal symptoms. The diagnosis should be made early since celiac disease causes growth retardation in untreated children and atypical symptoms like infertility or neurological symptoms. Diagnosis requires endoscopy with jejunal biopsy. In addition, tissue-transglutaminase antibodies are important to confirm the diagnosis since there are other diseases which can mimic celiac disease. The exact cause of celiac disease is unknown but is thought to be primarily immune mediated (tissue-transglutaminase autoantigen; often the disease is inherited. Management consists in life long withdrawal of dietary gluten, which leads to significant clinical and histological improvement. However, complete normalization of histology can take years.

  3. Wilson disease

    Science.gov (United States)

    ... nervous system and peripheral nervous system Copper urine test References Cox DW, Roberts EA. Wilson disease. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal ...

  4. Dent's disease

    National Research Council Canada - National Science Library

    Devuyst, Olivier; Thakker, Rajesh V

    2010-01-01

    Dent's disease is a renal tubular disorder characterized by manifestations of proximal tubule dysfunction, including low-molecular-weight proteinuria, hypercalciuria, nephrolithiasis, nephrocalcinosis...

  5. [Periodontal disease].

    Science.gov (United States)

    Michikawa, Makoto

    2014-04-01

    It has been shown that the inflammatory pathways are activated in the brains of patients with Alzheimer disease (AD), and the use of anti-inflammatory drugs reduces risk to develop AD. It is understood that molecules involved in this inflammation promote pathological processes leading to AD, whereas other molecules work to protect neuron/brain function from toxicity found in AD pathogenesis. Periodontal disease is one of the diseases causing inflammation and recent lines of evidence show the link between these two diseases. In this paper, relationship between periodontitis and AD will be reviewed and the possible mechanisms, by which periodontitis may affect the onset and progression of AD, will be discussed.

  6. Pompe Disease

    Science.gov (United States)

    ... reliably identified via genetic mutation analysis. View Full Definition Treatment Individuals with Pompe disease are best treated by a team of specialists (such as cardiologist, neurologist, and respiratory ...

  7. Sandhoff Disease

    Science.gov (United States)

    ... SEARCH Definition Treatment Prognosis Clinical Trials Organizations Publications Definition Sandhoff disease is a rare, inherited lipid storage disorder that progressively destroys nerve cells in ...

  8. Behcet's Disease

    Science.gov (United States)

    ... Seizures Information Page Fibromuscular Dysplasia Information Page Foot Drop Information Page Friedreich's Ataxia Information Page Gaucher Disease Information Page Generalized Gangliosidoses Information Page Gerstmann's Syndrome ...

  9. Binswanger's Disease

    Science.gov (United States)

    ... Seizures Information Page Fibromuscular Dysplasia Information Page Foot Drop Information Page Friedreich's Ataxia Information Page Gaucher Disease Information Page Generalized Gangliosidoses Information Page Gerstmann's Syndrome ...

  10. Leigh's Disease

    Science.gov (United States)

    ... Seizures Information Page Fibromuscular Dysplasia Information Page Foot Drop Information Page Friedreich's Ataxia Information Page Gaucher Disease Information Page Generalized Gangliosidoses Information Page Gerstmann's Syndrome ...

  11. Is "Parkinson's disease" one disease?

    OpenAIRE

    Calne, D B

    1989-01-01

    Consideration is given to how and why categories of ill health are divided into diseases. Aetiology is a fundamental criterion for the delineation of individual diseases. The same clinical and pathological picture may have many different causes; for example meningococcal meningitis and pneumococcal meningitis are distinct diseases that may display the same symptoms and signs. On the other hand, a single aetiology may lead to quite separate clinical and pathological phenomena; for example, neu...

  12. Liver Disease

    Science.gov (United States)

    ... A through E and beyond. National Institute of Diabetes and Digestive and Kidney Diseases. http://digestive.niddk.nih.gov/ddiseases/pubs/viralhepatitis/index.aspx. Accessed March 25, 2014. July 15, 2014 Original article: http://www.mayoclinic.org/diseases-conditions/liver- ...

  13. Menetrier's Disease

    Science.gov (United States)

    ... with Ménétrier’s disease who also had Helicobacter pylori (H. pylori) infection. H. pylori is a bacterium that is a cause of ... the small intestine. In these cases, treatment for H. pylori reversed and improved the symptoms of Ménétrier’s disease. ...

  14. Pick disease

    Science.gov (United States)

    ... rare form of dementia that is similar to Alzheimer disease , except that it tends to affect only certain areas of the brain. Causes People ... Volunteer services People with Pick disease and their family may need to seek legal advice early in the course of the disorder. ... (Prognosis) The disorder ...

  15. Sycamore diseases

    Science.gov (United States)

    F. I. McCracken

    1989-01-01

    The canker stain disease, one of several fungi that cause cankers of sycamore, can cause serious loss of sycamores in natural stands, plantations, and urban areas. As many as 35 percent of the trees in some stands may be diseased. Affected trees develop thin crowns, twig dieback, small leaves and epicormic branches. The narrow, elongate, bark covered, flat, spiraling...

  16. Angara disease

    African Journals Online (AJOL)

    Jane

    2011-10-12

    Oct 12, 2011 ... The farmers were advised to use quality vaccines against Angara disease as the vaccines can provide. 100% protection against the disease if they are given at the proper age. REFERENCES. Afzal M, Ahmed I (1990). Efficacy of an inactivated vaccine against. Hydropericardium syndrome in broilers, Vet.

  17. Crohn's Disease

    Science.gov (United States)

    ... Syndrome (IBS) in Children Lactose Intolerance Ménétrier’s Disease Microscopic Colitis Ostomy Surgery of the Bowel Pancreatitis Definition & Facts ... Upper GI Endoscopy Related Conditions & Diseases Ulcerative Colitis Microscopic Colitis Your Digestive System & How it Works The digestive ...

  18. Infectious Diseases

    Science.gov (United States)

    ... such as undercooked hamburger or unpasteurized fruit juice. Risk factors While anyone can catch infectious diseases, you may be more likely to get ... have been linked to a long-term increased risk of cancer: Human ... In addition, some infectious diseases may become silent, only to appear again ...

  19. Coeliac disease

    DEFF Research Database (Denmark)

    Reilly, Norelle R; Husby, Steffen; Sanders, David S

    2018-01-01

    , these guidelines are not universally accepted. In this Perspective, we discuss the pros and cons of a biopsy-avoiding pathway for the diagnosis of coeliac disease, especially in this current era of the call for more biopsies, even from the duodenal bulb, in the diagnosis of coeliac disease. In addition, a contrast...

  20. [Caroli's disease].

    Science.gov (United States)

    Duvnjak, Marko; Supanc, Vladimir; Virović, Lucija; Tomasić, Vedran; Dojcinović, Bojan

    2003-01-01

    Caroli's disease is a rare condition characterized by congenital polycystic dilatation of the intrahepatic bile ducts. The most frequent clinical presentation of a simple type (Caroli's disease) is recurrent cholangitis, gallstone with pain, obstructive jaundice and episodes of pancreatitis in childhood and early adulthood. A more frequent type combined with congenital hepatic fibrosis is usually manifested with bleeding from esophageal varices consequential to portal hypertension. Treatment options, both conservative and surgical, are relatively limited and depend on the clinical presentation, localization of cysts in the liver, and stage of the disease. A 20-year-old man with Caroli's disease manifested with cholelithiasis and choledocholithiasis with recurrent pancreatitis at the age of 16 is presented. The diagnosis was confirmed by endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography. Treatment with ursodeoxycholic acid was initiated and after two years of follow-up the disease is stable.

  1. Alzheimer's disease.

    Science.gov (United States)

    Scheltens, Philip; Blennow, Kaj; Breteler, Monique M B; de Strooper, Bart; Frisoni, Giovanni B; Salloway, Stephen; Van der Flier, Wiesje Maria

    2016-07-30

    Although the prevalence of dementia continues to increase worldwide, incidence in the western world might have decreased as a result of better vascular care and improved brain health. Alzheimer's disease, the most prevalent cause of dementia, is still defined by the combined presence of amyloid and tau, but researchers are gradually moving away from the simple assumption of linear causality as proposed in the original amyloid hypothesis. Age-related, protective, and disease-promoting factors probably interact with the core mechanisms of the disease. Amyloid β42, and tau proteins are established core cerebrospinal biomarkers; novel candidate biomarkers include amyloid β oligomers and synaptic markers. MRI and fluorodeoxyglucose PET are established imaging techniques for diagnosis of Alzheimer's disease. Amyloid PET is gaining traction in the clinical arena, but validity and cost-effectiveness remain to be established. Tau PET might offer new insights and be of great help in differential diagnosis and selection of patients for trials. In the search for understanding the disease mechanism and keys to treatment, research is moving increasingly into the earliest phase of disease. Preclinical Alzheimer's disease is defined as biomarker evidence of Alzheimer's pathological changes in cognitively healthy individuals. Patients with subjective cognitive decline have been identified as a useful population in whom to look for preclinical Alzheimer's disease. Moderately positive results for interventions targeting several lifestyle factors in non-demented elderly patients and moderately positive interim results for lowering amyloid in pre-dementia Alzheimer's disease suggest that, ultimately, there will be a future in which specific anti-Alzheimer's therapy will be combined with lifestyle interventions targeting general brain health to jointly combat the disease. In this Seminar, we discuss the main developments in Alzheimer's research. Copyright © 2016 Elsevier Ltd. All

  2. Coeliac disease.

    Science.gov (United States)

    Leeds, John S; Hopper, Andrew D; Sanders, David S

    2008-01-01

    Coeliac disease is a common but often under diagnosed condition with important complications. It is due to immune-mediated gluten intolerance and may present in a number of ways. It has become more frequently diagnosed due to the recognition of the atypical presentations. In recent years, more sensitive and specific serological markers have been developed but the gold standard of diagnosis remains duodenal biopsy. Compliance with a strict, lifelong gluten-free diet is the cornerstone of management, improving symptoms and reducing complications of the disease. For this review, we focused on papers published on coeliac disease in recent years. Particular emphasis was given to clinical papers examining new methods for the diagnosis of coeliac disease or newer therapies for managing complications. The main source was PubMed and the major gastroenterology journals. Coeliac disease is more common than once thought with a prevalence of around 1%. Diagnosis should always be confirmed with a duodenal biopsy. Management of coeliac disease with a gluten-free diet remains the cornerstone of treatment. Some complications of coeliac disease, especially neurological, are not widely accepted despite growing support from the literature. Management of enteropathy-associated lymphoma has been difficult, and the optimal therapy is not known. Current understanding is such that coeliac disease is the most widely understood autoimmune condition. 'Atypical' presentations are becoming the most common presenting features of coeliac disease. Alternatives to the gluten-free diet are about to go into clinical studies. Similarly, better serological screening tests may obviate the need for duodenal biopsy. This review will try to summarize the current understanding of coeliac disease with regard to diagnosis, management, complications and future perspectives.

  3. Celiac Disease

    Directory of Open Access Journals (Sweden)

    Manoochehr Karjoo

    2014-08-01

    Full Text Available Celiac disease also known as gluten-sensitive enteropathy is characterized by intestinal mucosal damage and malabsorption from dietary intake of wheat, rye or barley. Symptoms may appear with introduction of cereal in the first 3 years of life. A second peak in symptoms occurs in adults during the third or forth decade and even as late as eight decade of life. The prevalence of this disease is approximately 1 in 250 adults. The disease is more prevalent in Ireland as high as 1 in 120 adults. The disorder occurs in Arab, Hispanics, Israeli Jews, Iranian and European but is rare in Chinese and African American. To have celiac disease the patient should have the celiac disease genetic markers as HLA DQ 2 and HLA DQ 8. Patient with celiac disease may have 95 per cent for DQ 2 and the rest is by DQ 8. Someone may have the genetic marker and never develops the disease. In general 50 percent with markers may develop celiac disease. To develop the disease the gene needs to become activated. This may happen with a viral or bacterial infection, a surgery, delivery, accident, or psychological stress. After activation of gene cause the tight junction to opens with the release of Zonulin This results in passage of gluten through the tight junction and formation of multiple antibodies and autoimmune disease. This also allows entrance of other proteins and development of multiple food allergies. As a result is shortening, flattening of intestinal villi resulting in food, vitamins and minerals malabsorption.

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

    Directory of Open Access Journals (Sweden)

    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.

  5. Thyroid diseases and cerebrovascular disease

    NARCIS (Netherlands)

    Squizzato, A.; Gerdes, V. E. A.; Brandjes, D. P. M.; Büller, H. R.; Stam, J.

    2005-01-01

    Background and Purpose-Acute cerebral ischemia has been described in different diseases of the thyroid gland, and not only as a result of thyrotoxic atrial fibrillation and cardioembolic stroke. The purpose of this review is to summarize the studies on the relationship between thyroid diseases and

  6. Morgellons Disease.

    Science.gov (United States)

    Ohn, Jungyoon; Park, Seon Yong; Moon, Jungyoon; Choe, Yun Seon; Kim, Kyu Han

    2017-04-01

    Morgellons disease is a rare disease with unknown etiology. Herein, we report the first case of Morgellons disease in Korea. A 30-year-old woman presented with a 2-month history of pruritic erythematous patches and erosions on the arms, hands, and chin. She insisted that she had fiber-like materials under her skin, which she had observed through a magnifying device. We performed skin biopsy, and observed a fiber extruding from the dermal side of the specimen. Histopathological examination showed only mild lymphocytic infiltration, and failed to reveal evidence of any microorganism. The polymerase chain reaction for Borrelia burgdorferi was negative in her serum.

  7. Crohn's disease.

    LENUS (Irish Health Repository)

    Shanahan, Fergus

    2012-02-03

    Crohn\\'s disease is a disorder mediated by T lymphocytes which arises in genetically susceptible individuals as a result of a breakdown in the regulatory constraints on mucosal immune responses to enteric bacteria. Regulation of immune reactivity to enteric antigens has improved understanding of the pathophysiological mechanisms of Crohn\\'s disease, and has expanded therapeutic options for patients with this disorder. Disease heterogeneity is probable, with various underlying defects associated with a similar pathophysiological outcome. Although most conventional drug treatments are directed at modification of host response, therapeutic manipulation of the enteric flora is becoming a realistic option.

  8. Bullous Pemphigoid Induced by Vildagliptin

    Directory of Open Access Journals (Sweden)

    Bengür Taşkıran Bahattin

    2016-12-01

    Full Text Available Bullous pemphigoid (BP is an uncommon chronic, autoimmune, and subepidermal disease. Tense blisters occur on normal or erythematous skin. It can be induced by medications. There is a number of reports on BP induced by dipeptidyl peptidase 4 (DPP-4 inhibitors (vildagliptin, sitagliptin, saxagliptin. DPP-4 (CD26, present as a cell surface molecule on immune cells, also plays an important costimulatory role in immune activation. BP more commonly affects elderly men. We present a case of BP induced by vildagliptin. A 59-year-old male patient who was diagnosed with type 2 diabetes mellitus had initial hemoglobin A1c level of 12.90%. Initial therapy with premix biphasic aspart insulin bid was switched to metformin and vildagliptin 50/1000 mg combo pill bid after A1c level dropped to 5.7% at 9 months of insulin therapy, Five months after vildagliptin was started, tense vesicles 8-10 in number with an erythematous base developed on the forearms and cruris. Histological examination of the lesions confirmed the diagnosis of BP. Oral antidiabetics were discontinued. He was followed up with diet alone. The lesions regressed spontaneously after cessation of antidiabetics and clobetasol propionate cream bid treatment. A1c was 5.7% 5 months after discontinuation of vildagliptin and metformin. In the literature, it has been reported that onset of BP lesions took 10 days to 2 years. Mostly the patients were on combo therapy with metformin. The lesions improved dramatically after cessation of DPP-4 inhibitors avoiding necessity for systemic treatment for BP. This is the first case of BP induced by DPP-4 inhibitors in Turkey.

  9. Immunofluorescence serration pattern analysis as a diagnostic criterion in antilaminin-332 mucous membrane pemphigoid: immunopathological findings and clinical experience in 10 Dutch patients.

    Science.gov (United States)

    Terra, J B; Pas, H H; Hertl, M; Dikkers, F G; Kamminga, N; Jonkman, M F

    2011-10-01

    Antilaminin-332 mucous membrane pemphigoid (anti-LN-332 MMP) is a chronic subepidermal blistering disease characterized by IgG anti-epidermal basement membrane zone (BMZ) autoantibodies against laminin-332 (LN-332). with anti-LN-332 MMP have an increased relative risk of malignancy. Laboratory techniques that are difficult to obtain are needed for diagnosis of anti-LN-332 MMP. Objectives  To incorporate direct immunofluorescence (DIF) serration pattern analysis of IgG depositions in the diagnostic criteria of anti-LN-332 MMP. Patients who met our revised inclusion criteria for anti-LN-332 MMP were selected from our biobank over the period 1997-2009. Inclusion criteria were clinical symptoms, DIF serration pattern analysis, indirect immunofluorescence (IIF) on salt-split skin, and antigen-specificity analysis of the serum including immunoblotting and/or immunoprecipitation and/or enzyme-linked immunosorbent assay (ELISA) against native LN-332. Ten patients met the inclusion criteria. A malignancy was found in two patients (20%). In all patients in whom it was performed (n = 9), DIF showed linear IgG deposition along the BMZ in an n-serrated pattern. Nine sera reacted by salt-split skin analysis and bound to the dermal side of the split skin. ELISA against native LN-332 was positive in 78% of the tested sera. Anti-LN-332 MMP can clinically resemble other forms of pemphigoid. Although state-of-the-art laboratory diagnostics are necessary for definite diagnosis, the combination of simple DIF serration pattern and IIF salt-split skin analysis will exclude other forms of MMP and epidermolysis bullosa acquisita from the differential diagnosis. Because of the increased risk for malignancy patients should be thoroughly oncologically screened. © 2011 The Authors. BJD © 2011 British Association of Dermatologists 2011.

  10. Macropinocytosis of type XVII collagen induced by bullous pemphigoid IgG is regulated via protein kinase C.

    Science.gov (United States)

    Iwata, Hiroaki; Kamaguchi, Mayumi; Ujiie, Hideyuki; Nishimura, Machiko; Izumi, Kentaro; Natsuga, Ken; Shinkuma, Satoru; Nishie, Wataru; Shimizu, Hiroshi

    2016-12-01

    Macropinocytosis is an endocytic pathway that is involved in the nonselective fluid uptake of extracellular fluid. Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease associated with autoantibodies to type XVII collagen (COL17), which is a component of hemidesmosome. When keratinocytes are treated with BP-IgG, COL17 internalizes into cells by way of the macropinocytosis. We investigated the mechanism of COL17 macropinocytosis using DJM-1 cells, a cutaneous squamous cell carcinoma cell line. First, non-hemidesmosomal COL17 was preferentially depleted by stimulation with the BP-IgG in the DJM-1 cells. To investigate the signaling involved in COL17-macropinocytosis, the inhibition of small GTPase family members Rac1 and Cdc42 was found to strongly repress COL17 internalization; in addition, the Rho inhibitor also partially blocked that internalization, suggesting these small GTPases are involved in signaling to mediate COL17-macropinocytosis. Western blotting using Phostag-SDS-PAGE demonstrated high levels of COL17 phosphorylation in DJM-1 cells under steady-state condition. Treatment with BP-IgG increased the intracellular calcium level within a minute, and induced the overabundant phosphorylation of COL17. The overabundant phosphorylation of COL17 was suppressed by a protein kinase C (PKC) inhibitor. In addition, PKC inhibitor repressed COL17 endocytosis using cell culture and organ culture systems. Finally, the depletion of COL17 was not observed in the HEK293 cells transfected COL17 without intracellular domain. These results suggest that COL17 internalization induced by BP-IgG may be mediated by a PKC pathway. In summary, BP-IgG initially binds to COL17 distributed on the plasma membrane, and COL17 may be internalized by means of a macropinocytic pathway related to the phosphorylation of the intracellular domain by PKC.

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

    Directory of Open Access Journals (Sweden)

    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.

  12. Celiac Disease

    Science.gov (United States)

    ... Intestinal Pseudo-obstruction Irritable Bowel Syndrome (IBS) Definition & Facts Symptoms & Causes Diagnosis Treatment Eating, Diet, & Nutrition Clinical Trials Irritable Bowel Syndrome (IBS) in Children Lactose Intolerance Ménétrier’s Disease Microscopic Colitis Ostomy Surgery of the ...

  13. Infectious Diseases

    Science.gov (United States)

    ... yeasts. Athlete's foot is a common fungal infection. Parasites - animals or plants that survive by living on or in other living things. Malaria is an infection caused by a parasite. Infectious diseases can cause many different symptoms. Some ...

  14. Raynaud's Disease

    Science.gov (United States)

    ... who have a rare disease that leads to hardening and scarring of the skin (scleroderma) have Raynaud's. ... conditioner to a warmer temperature. Use insulated drinking glasses. By Mayo Clinic Staff . Mayo Clinic Footer Legal ...

  15. Leishmaniasis Disease

    Science.gov (United States)

    ... Leishmaniasis General Information Leishmaniasis FAQs Epidemiology & Risk Factors Biology Disease Diagnosis Treatment Prevention & Control Resources for Health Professionals Publications Additional Resources Get Email Updates To receive email updates about this page, enter ...

  16. Diverticular Disease

    Science.gov (United States)

    ... The most common conditions of diverticular disease are:Diverticulosis. This is the base condition where the pouches ... more common as you age. Most people with diverticulosis don’t know they have it.Diverticulitis. This ...

  17. Endocrine Diseases

    Science.gov (United States)

    ... hormone disorder. Hormone diseases also occur if your body does not respond to hormones the way it is supposed to. Stress, infection and changes in your blood's fluid and electrolyte balance can also influence hormone levels. In the United ...

  18. Addison's Disease

    Science.gov (United States)

    ... who have Addison’s disease find that taking this medicine improves their mood and sex drive.If you are experiencing an Addisonian crisis, you need immediate medical care. The treatment typically ...

  19. Autoinflammatory Diseases

    Science.gov (United States)

    ... Arthritis Lupus Polymyalgia Rheumatica Reactive Arthritis Psoriatic Arthritis Rheumatoid Arthritis Scleroderma Sjögren’s ... diseases refer to problems with the immune system, which usually fights off viruses, bacteria, and infection. It results when your immune cells ...

  20. Glomerular Diseases

    Science.gov (United States)

    ... Transplant Financial Help for Treatment of Kidney Failure Lupus Nephritis Nephrotic Syndrome in Adults Pain Medicine & Kidney Damage ... has also been implicated as a triggering factor. Lupus nephritis is the name given to the kidney disease ...

  1. Stargardt Disease

    Science.gov (United States)

    ... sharp central vision — for tasks like reading, watching television, and looking at faces. Decreased central vision is ... and ways to prevent it. A decrease in color perception also occurs in Stargardt disease. This is ...

  2. Celiac Disease

    Science.gov (United States)

    ... wheat. However, wheat-free doesn't mean gluten-free . Lawmakers are working to make labels easier for people with celiac disease by requiring companies to identify other components, such as hidden ingredients ...

  3. Huntington's Disease

    Science.gov (United States)

    ... Division of Neuroscience Director, NIH BRAIN Initiative® Health Scientist Administrator Channels Synapses Circuits Cluster Scientific Director, Division of Intramural Research Featured Director's Message menu search Enter Search Term Submit Search Huntington's Disease Information ...

  4. Krabbe Disease

    Science.gov (United States)

    ... Division of Neuroscience Director, NIH BRAIN Initiative® Health Scientist Administrator Channels Synapses Circuits Cluster Scientific Director, Division of Intramural Research Featured Director's Message menu search Enter Search Term Submit Search Krabbe Disease Information ...

  5. Canavan Disease

    Science.gov (United States)

    ... Division of Neuroscience Director, NIH BRAIN Initiative® Health Scientist Administrator Channels Synapses Circuits Cluster Scientific Director, Division of Intramural Research Featured Director's Message menu search Enter Search Term Submit Search Canavan Disease Information ...

  6. Gaucher Disease

    Science.gov (United States)

    ... Division of Neuroscience Director, NIH BRAIN Initiative® Health Scientist Administrator Channels Synapses Circuits Cluster Scientific Director, Division of Intramural Research Featured Director's Message menu search Enter Search Term Submit Search Gaucher Disease Information ...

  7. Thyroid Diseases

    Science.gov (United States)

    ... gland does not make enough thyroid hormones Thyroid cancer Thyroid nodules - lumps in the thyroid gland Thyroiditis - swelling of the thyroid To diagnose thyroid diseases, doctors use a medical history, physical exam, and thyroid tests. They sometimes also ...

  8. Retinal Diseases

    Science.gov (United States)

    ... Linked Retinoschisis (XLRS) X-Linked Retinitis Pigmentosa (XLRP) Usher Syndrome Other Retinal Diseases Glossary News & Research News & Research ... central portion of the retina called the macula. Usher Syndrome Usher syndrome is an inherited condition characterized by ...

  9. Fungal Diseases

    Science.gov (United States)

    ... also can infect the brain, bones, and heart (endocarditis). Superficial infections can occur in children with healthy ... B. Last Updated 11/21/2015 Source Immunizations & Infectious Diseases: An Informed Parent's Guide (Copyright © 2006 American ...

  10. Crohn's disease.

    Science.gov (United States)

    Ballester Ferré, María Pilar; Boscá-Watts, Marta Maia; Mínguez Pérez, Miguel

    2017-12-12

    Crohn's disease is a chronic inflammatory bowel disease of unknown etiology associated with an impaired immune response, with periods of activity and remission. It is characterised by patchy and transmural lesions which can affect the entire gastrointestinal tract, from the mouth to the anus. The most frequent symptoms are abdominal pain and diarrhoea, which can seriously affect patients' quality of life. The increasing incidence and prevalence of the disease in our area has had a large impact on clinical practice, with the rapid development of diagnostic and therapeutic techniques. To reduce the risk of complications, primary care physicians and gastroenterologists should be familiar with the management of the disease. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

  11. Hashimoto's Disease

    Science.gov (United States)

    ... can lead to problems during pregnancy, such as: Preeclampsia Anemia Miscarriage Placental abruption Postpartum bleeding It also ... AIDS Awareness Day National Women's Health Week Supporting Nursing Moms at Work Popular Topics Autoimmune diseases Breastfeeding ...

  12. Hirschsprung Disease

    Science.gov (United States)

    ... but they do know it can run in families and affects boys more often than girls. In fact, Hirschsprung disease is about five times more common in males than females. Children with Down syndrome and genetic heart conditions also ...

  13. Chagas disease

    Science.gov (United States)

    ... major health problems in South America. Due to immigration, the disease also affects people in the United ... nodes Irregular heartbeat Rapid heartbeat Tests include: Blood culture to look for signs of infection Chest x- ...

  14. Krabbe Disease

    Science.gov (United States)

    ... A gene provides a kind of blueprint for producing proteins. If there is an error in this ... Make a donation. Patient Care & Health Info Healthy Lifestyle Symptoms A-Z Diseases & Conditions A-Z Tests & ...

  15. Fabry Disease

    Science.gov (United States)

    ... SEARCH Definition Treatment Prognosis Clinical Trials Organizations Publications Definition Fabry disease is caused by the lack of or faulty enzyme needed to metabolize lipids, fat-like substances that include oils, waxes, and ...

  16. Batten Disease

    Science.gov (United States)

    ... SEARCH Definition Treatment ... In some cases, the early signs are subtle, taking the form of personality and behavior changes, slow learning, clumsiness, or stumbling. Symptoms of Batten disease are ...

  17. Parkinson's Disease

    Science.gov (United States)

    ... a loss of neurons that produce a chemical messenger in your brain called dopamine. When dopamine levels ... Diagnosis & treatment July 07, 2015 Print Share on: Facebook Twitter References Longo DL, et al. Parkinson's disease ...

  18. Huntington disease

    Science.gov (United States)

    ... that may be associated with this disease: Anxiety, stress, and tension Difficulty swallowing Speech impairment Symptoms in children: Rigidity Slow movements Tremor Exams and Tests The health care provider will perform ...

  19. Sever's Disease

    Science.gov (United States)

    ... place on hard surfaces, such as track, basketball, soccer, and gymnastics. Sever's disease also can result from ... plate flat or high arch , which affects the angle of the heel within the foot, causing tightness ...

  20. Crohn disease

    Science.gov (United States)

    Stappenbeck, Thaddeus S.; Rioux, John D.; Mizoguchi, Atsushi; Saitoh, Tatsuya; Huett, Alan; Darfeuille-Michaud, Arlette; Wileman, Tom; Mizushima, Noboru; Carding, Simon; Akira, Shizuo; Parkes, Miles; Xavier, Ramnik J.

    2011-01-01

    Crohn disease (CD) is a chronic and debilitating inflammatory condition of the gastrointestinal tract.1 Prevalence in western populations is 100–150/100,000 and somewhat higher in Ashkenazi Jews. Peak incidence is in early adult life, although any age can be affected and a majority of affected individuals progress to relapsing and chronic disease. Medical treatments rely significantly on empirical corticosteroid therapy and immunosuppression, and intestinal resectional surgery is frequently required. Thus, 80% of patients with CD come to surgery for refractory disease or complications. It is hoped that an improved understanding of pathogenic mechanisms, for example by studying the genetic basis of CD and other forms of inflammatory bowel diseases (IBD), will lead to improved therapies and possibly preventative strategies in individuals identified as being at risk. PMID:20729636

  1. [Wilson's disease

    OpenAIRE

    Duclos-Vallée, Jean-Charles; Ichaï, Philippe; Chapuis, Philippe; Misrahi, Micheline; Woimant, France

    2006-01-01

    http://www.huveaux.fr/fr_santesite.asp; Wilson's disease is an autosomal recessive disorder of copper excess. This illness results from mutations of the ATP7B gene chromosome 13. The discovery of the gene allowed a better understanding of cytosolic copper trafficking its relationship with ceruloplasmin synthesis. Symptomatic patients may present with hepatic, neurologic or psychiatric forms. Clinical and phenotypic evidences provide only presumptive arguments for this disease which can be rou...

  2. Menkes disease

    DEFF Research Database (Denmark)

    Tümer, Zeynep; Møller, Lisbeth B

    2010-01-01

    Menkes disease (MD) is a lethal multisystemic disorder of copper metabolism. Progressive neurodegeneration and connective tissue disturbances, together with the peculiar 'kinky' hair are the main manifestations. MD is inherited as an X-linked recessive trait, and as expected the vast majority...... of surplus copper from cells. Severely affected MD patients die usually before the third year of life. A cure for the disease does not exist, but very early copper-histidine treatment may correct some of the neurological symptoms....

  3. Borna disease.

    OpenAIRE

    Hatalski, C G; Lewis, A J; Lipkin, W. I.

    1997-01-01

    Borna disease virus, a newly classified nonsegmented negative-strand RNA virus with international distribution, infects a broad range of warm-blooded animals from birds to primates. Infection causes movement and behavioral disturbances reminiscent of some neuropsychiatric syndromes. The virus has not been clearly linked to any human disease; however, an association between infection with the virus and selected neuropsychiatric disorders has been suggested. We reviewed recent advances in Borna...

  4. Pilonidal Disease

    OpenAIRE

    Khanna, Amit; Rombeau, John L.

    2011-01-01

    Pilonidal disease presents many therapeutic challenges to surgeons throughout the world. Its varied clinical presentations necessitate a wide range of treatments, thus underscoring the need to tailor the treatment to the patient and the severity of disease. Recent studies confirm the efficacy of smaller, more conservative operations for appropriate indications. When flap closures are performed, every attempt should be directed to placing sutures off (lateral) to the midline gluteal cleft. Met...

  5. Ollier disease

    Directory of Open Access Journals (Sweden)

    Jüppner Harald

    2006-09-01

    Full Text Available Abstract Enchondromas are common intraosseous, usually benign cartilaginous tumors, that develop in close proximity to growth plate cartilage. When multiple enchondromas are present, the condition is called enchondromatosis also known as Ollier disease (WHO terminology. The estimated prevalence of Ollier disease is 1/100,000. Clinical manifestations often appear in the first decade of life. Ollier disease is characterized by an asymmetric distribution of cartilage lesions and these can be extremely variable (in terms of size, number, location, evolution of enchondromas, age of onset and of diagnosis, requirement for surgery. Clinical problems caused by enchondromas include skeletal deformities, limb-length discrepancy, and the potential risk for malignant change to chondrosarcoma. The condition in which multiple enchondromatosis is associated with soft tissue hemangiomas is known as Maffucci syndrome. Until now both Ollier disease and Maffucci syndrome have only occurred in isolated patients and not familial. It remains uncertain whether the disorder is caused by a single gene defect or by combinations of (germ-line and/or somatic mutations. The diagnosis is based on clinical and conventional radiological evaluations. Histological analysis has a limited role and is mainly used if malignancy is suspected. There is no medical treatment for enchondromatosis. Surgery is indicated in case of complications (pathological fractures, growth defect, malignant transformation. The prognosis for Ollier disease is difficult to assess. As is generally the case, forms with an early onset appear more severe. Enchondromas in Ollier disease present a risk of malignant transformation of enchondromas into chondrosarcomas.

  6. [Chagas disease].

    Science.gov (United States)

    Develoux, M; Lescure, F-X; Le Loup, G; Pialoux, G

    2009-08-01

    Chagas disease (human American trypanosomiasis) is a zoonose caused by the protozoan Trypanosoma cruzi. Vectors are Triatoma spp. insects. T. cruzi can also be transmitted by blood transfusion, organ transplantation, and transplacentally. Infection is generally acquired during infancy. The acute infection is rarely symptomatic and is followed by a chronic phase. Chronic infected people are asymptomatic (indeterminate stage) and may remain at this stage for the rest of their lives. About a third of infected people will develop a chronic Chagas disease which affects the heart and the digestive tract. Morbidity and mortality of chronic Chagas cardiomyopathy (CCC) are high. Specific treatment of asymptomatic infected individual could reduce the risk of progression to CCC. With control initiatives case incidence declined in most endemic countries. American trypanosomiasis has become an emerging imported disease in North America and Europe because of the migration of population originating from endemic zones. They are only two available drugs for specific treatment of Chagas disease: benznidazole and nifurtimox. Both have frequent side effects and variable efficacy according the phase of the disease. There is an urgent need for new treatments and better serological tests. Policies must be developed to avoid the risk of transmission trough blood transfusion and transplantation in developed countries.

  7. HIV and Rheumatic Disease

    Science.gov (United States)

    ... A Patient / Caregiver Diseases & Conditions HIV & Rheumatic Diseases HIV and Rheumatic Disease Fast Facts Rheumatic diseases related ... knows he or she has HIV. What are HIV-associated rheumatic diseases? Some diseases of the joints ...

  8. Disease Outbreak News

    Science.gov (United States)

    ... Diseases Biorisk reduction Disease outbreak news Disease Outbreak News (DONs) Latest DONs Rift Valley fever – Gambia ... Disease outbreaks by country RSS feeds Disease outbreak news Related links Ebola virus disease - website Avian influenza ...

  9. NMR-based metabolomics in human disease diagnosis: Applications, limitations, and recommendations

    KAUST Repository

    Emwas, Abdul-Hamid M.

    2013-04-03

    Metabolomics is a dynamic and emerging research field, similar to proteomics, transcriptomics and genomics in affording global understanding of biological systems. It is particularly useful in functional genomic studies in which metabolism is thought to be perturbed. Metabolomics provides a snapshot of the metabolic dynamics that reflect the response of living systems to both pathophysiological stimuli and/or genetic modification. Because this approach makes possible the examination of interactions between an organism and its diet or environment, it is particularly useful for identifying biomarkers of disease processes that involve the environment. For example, the interaction of a high fat diet with cardiovascular disease can be studied via such a metabolomics approach by modeling the interaction between genes and diet. The high reproducibility of NMR-based techniques gives this method a number of advantages over other analytical techniques in large-scale and long-term metabolomic studies, such as epidemiological studies. This approach has been used to study a wide range of diseases, through the examination of biofluids, including blood plasma/serum, urine, blister fluid, saliva and semen, as well as tissue extracts and intact tissue biopsies. However, complicating the use of NMR spectroscopy in biomarker discovery is the fact that numerous variables can effect metabolic composition including, fasting, stress, drug administration, diet, gender, age, physical activity, life style and the subject\\'s health condition. To minimize the influence of these variations in the datasets, all experimental conditions including sample collection, storage, preparation as well as NMR spectroscopic parameters and data analysis should be optimized carefully and conducted in an identical manner as described by the local standard operating protocol. This review highlights the potential applications of NMR-based metabolomics studies and gives some recommendations to improve sample

  10. Celiac disease

    DEFF Research Database (Denmark)

    Hvas, Christian Lodberg; Jensen, Michael Dam; Reimer, Maria Christina

    2015-01-01

    This national clinical guideline approved by the Danish Society for Gastroenterology and Hepatology describes the diagnosis and treatment of celiac disease (CD) in adults. CD is a chronic immunemediated enteropathy of the small intestine triggered by the ingestion of gluten-containing proteins......, which are found in wheat, rye, and barley. The disease prevalence is 0.5-1.0%, but CD remains under-diagnosed. The diagnosis relies on the demonstration of lymphocyte infiltration, crypt hyperplasia, and villous atrophy in duodenal biopsies. Serology, malabsorption, biochemical markers...... the small intestinal mucosa and absorption. Adherence to a GFD usually requires dietary advice from a clinical dietician. The monitoring of antibody levels and malabsorption markers is crucial during follow-up and allows for early treatment of disease complications. Important complications include...

  11. [Dupuytren disease].

    Science.gov (United States)

    Wagner, Pablo; Román, Javier A; Vergara, Jorge

    2012-09-01

    Dupuytren disease (DD) is a connective tissue disorder that consists in fibromatosis of the palmar and digital fascia (in form of nodules or flanges) that leads to the development of flexion contractures of the palm and fingers. The little and ring finger are particularly affected. The disease can limit hand function, reducing the quality of life. The disease can have a traumatic origin and is also associated with conditions such as diabetes mellitus, alcoholism, dyslipidemia, epilepsy and AIDS, among others. However, none of these conditions can fully explain the genesis of DD. A hereditary component is described in 40% of patients and is attributed to an autosomal dominant gene of variable penetrance, probably related to collagen synthesis. However there are also spontaneous and recessive inheritance cases. The diagnosis is clinical and based on physical examination. Treatment ranges from observation or use of injectable collagenase to the surgical option in cases with significant functional limitations.

  12. Alzheimer's disease.

    Science.gov (United States)

    Moody, G H; Drummond, J R; Newton, J P

    1990-07-21

    Alzheimer's disease (AD) is a debilitating and progressive disease that can affect dental practice in a number of ways. Not only will increasing numbers of AD patients be treated by dentists in the future but the dentist and members of his family may also be unfortunate enough to become affected. In the United Kingdom, nearly 10% of the population over 65 years of age, and more than 20% of those over 80 years develop progressive deterioration of memory, resulting in a breakdown of intellectual capacity and personality. More than half of those are suffering from AD. The purpose of this review is to describe the aetiology and behavioural aspects of this distressing disease and to highlight some problems that the dentist may encounter when treating those patients.

  13. Parkinson's disease

    DEFF Research Database (Denmark)

    Astradsson, Arnar; Aziz, Tipu Z

    2015-01-01

    INTRODUCTION: The mean age of onset of Parkinson's disease is about 65 years, with a median time of 9 years between diagnosis and death. METHODS AND OUTCOMES: We conducted a systematic review and aimed to answer the following clinical question: What are the effects of fetal cell or stem cell......-derived therapy in people with Parkinson's disease? We searched: Medline, Embase, The Cochrane Library and other important databases up to September 2014 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from...

  14. Dupuytren's disease.

    Science.gov (United States)

    Worrell, Michael

    2012-01-01

    Dupuytren's disease is a benign contractile disorder of the hand. The condition commonly affects older men of Celtic descent. Although fibroproliferation and collagen alteration play a role in its etiology, defining a cause remains elusive. Nonoperative intervention for advanced disease has shown only short-term benefit. Therefore, open fasciectomy has become the mainstay of treatment. Associated morbidity and recurrence have prompted investigation into less invasive techniques, including needle aponeurotomy and enzymatic fasciotomy. Data from phase III studies using injectable collagenase are changing treatment algorithms. Postoperative rehabilitation includes nighttime splinting and immediate active range of motion exercises to facilitate return to function. Copyright 2012, SLACK Incorporated.

  15. Dent's disease

    Directory of Open Access Journals (Sweden)

    Thakker Rajesh V

    2010-10-01

    Full Text Available Abstract Dent's disease is a renal tubular disorder characterized by manifestations of proximal tubule dysfunction, including low-molecular-weight proteinuria, hypercalciuria, nephrolithiasis, nephrocalcinosis, and progressive renal failure. These features are generally found in males only, and may be present in early childhood, whereas female carriers may show a milder phenotype. Prevalence is unknown; the disorder has been reported in around 250 families to date. Complications such as rickets or osteomalacia may occur. The disease is caused by mutations in either the CLCN5 (Dent disease 1 or OCRL1 (Dent disease 2 genes that are located on chromosome Xp11.22 and Xq25, respectively. CLCN5 encodes the electrogenic Cl-/H+ exchanger ClC-5, which belongs to the CLC family of Cl- channels/transporters. OCRL1 encodes a phosphatidylinositol bisphosphate (PIP2 5-phosphatase and mutations are also associated with Lowe Syndrome. The phenotype of Dent's disease is explained by the predominant expression of ClC-5 in the proximal tubule segments of the kidney. No genotype-phenotype correlation has been described thus far, and there is considerable intra-familial variability in disease severity. A few patients with Dent's disease do not harbour mutations in CLCN5 and OCRL1, pointing to the involvement of other genes. Diagnosis is based on the presence of all three of the following criteria: low-molecular-weight proteinuria, hypercalciuria and at least one of the following: nephrocalcinosis, kidney stones, hematuria, hypophosphatemia or renal insufficiency. Molecular genetic testing confirms the diagnosis. The differential diagnosis includes other causes of generalized dysfunction of the proximal tubules (renal Fanconi syndrome, hereditary, acquired, or caused by exogenous substances. Antenatal diagnosis and pre-implantation genetic testing is not advised. The care of patients with Dent's disease is supportive, focusing on the treatment of hypercalciuria and

  16. Pilonidal disease.

    Science.gov (United States)

    Khanna, Amit; Rombeau, John L

    2011-03-01

    Pilonidal disease presents many therapeutic challenges to surgeons throughout the world. Its varied clinical presentations necessitate a wide range of treatments, thus underscoring the need to tailor the treatment to the patient and the severity of disease. Recent studies confirm the efficacy of smaller, more conservative operations for appropriate indications. When flap closures are performed, every attempt should be directed to placing sutures off (lateral) to the midline gluteal cleft. Meticulous attention to the details of immediate and long-term postoperative care is paramount.

  17. Ostrich diseases.

    Science.gov (United States)

    Verwoerd, D J

    2000-08-01

    Scientific knowledge of ostrich diseases is incomplete and very fragmented, with specific details on technical aspects of diagnostic and/or screening tests completely absent in most cases. Salmonella Typhimurium is common in multispecies collections and causes mortality in chicks younger than three months on commercial farms, but is rarely found in chicks older than six months, or slaughter birds of twelve to fourteen months in southern Africa. Campylobacter jejuni and Chlamydia psittaci are occasionally reported, mainly in young ostriches, but both remain a diagnostic challenge. Crimean-Congo haemorrhagic fever is transmitted to domestic animals including ostriches, principally by ticks of the genus Hyalomma. In the ostrich, the disease causes no clinical symptoms during a viraemia of approximately four days. Spongiform encephalopathy has not been reliably reported in ostriches, while anthrax has occurred rarely in modern times but was reportedly an important cause of death approximately 100 years ago in South Africa. Salmonella Gallinarum and S. Pullorum are unknown in ostriches. Pasteurella multocida occurs but is easily contained with antibiotics. Mycoplasma spp. are regularly found in an upper respiratory disease syndrome complicated by opportunistic bacterial pathogens. Ostriches of all ages are susceptible to challenge by velogenic Newcastle disease virus (NDV), but standard inactivated La Sota poultry vaccines can stimulate protective immunity lasting over six months. The viraemic period in vaccinated slaughter ostriches is between nine and eleven days and there are no indications of a carrier state or presence of the virus in the meat or any other tissues after this period, with peak immunoglobulin G response reached on day fourteen post infection. Haemagglutination inhibition tests are significantly less sensitive and less specific than enzyme-linked immunosorbent assays. Cloacal and choanal swabs used for direct virological screening in clinically

  18. Hansen's disease: a vanishing disease?

    Directory of Open Access Journals (Sweden)

    Sinésio Talhari

    2012-12-01

    Full Text Available The introduction, implementation, successes and failures of multidrug therapy (MDT in all Hansen's disease endemic countries are discussed in this paper. The high efficacy of leprosy treatment with MDT and the global reduction of prevalence led the World Health Organization, in 1991, to establish the goal of elimination of Hansen's disease (less than 1 patient per 10,000 inhabitants to be accomplished by the year 2000. Brazil, Nepal and East Timor are among the few countries that didn't reach the elimination goal by the year 2000 or even 2005. The implications of these aspects are highlighted in this paper. Current data from endemic and previously endemic countries that carry a regular leprosy control programme show that the important fall in prevalence was not followed by the reduction of the incidence. This means that transmission of Mycobacterium leprae is still an issue. It is reasonable to conclude that we are still far from the most important goal of Hansen's disease control: the interruption of transmission and reduction of incidence. It is necessary to emphasize to health managers the need of keeping Hansen's disease control activities to better develop control programmes in the future. The recent international proposal to interrupt the transmission of leprosy by the year 2020 seems to unrealistic and it is discussed in this paper. The possibility of epidemiological impact related to the human immunodeficiency virus/Hansen's disease coinfection is also considered.

  19. Huntington's disease: a perplexing neurological disease ...

    African Journals Online (AJOL)

    Huntington's disease has served as a model for the study of other more common neurodegenerative disorders, such as Alzheimer's disease and Parkinson's disease. Symptomatic treatment of Huntington's disease involves use of Dopamine antagonists, presynaptic dopamine depleters, Antidepressants, Tranquillizers ...

  20. Bone Diseases

    Science.gov (United States)

    ... need to get enough calcium, vitamin D, and exercise. You should also avoid smoking and drinking too much alcohol. Bone diseases can make bones easy to break. Different kinds of bone problems include Low bone density and osteoporosis, which make your bones weak and ...

  1. Wilson Disease

    Science.gov (United States)

    ... people taking zinc usually do not experience side effects, some people may experience stomach upset. A health care provider ... 738–4929 Email: nddic@info.niddk.nih.gov Internet: ... diseases to people with digestive disorders and to their families, health ...

  2. Behcet's Disease

    Science.gov (United States)

    ... síndrome de Behçet? (Esenciales: hojas informativas de fácil lectura) View/Download/Order Publications Behçet’s Disease, Easy-to- ... Media Moderation Policy FOIA Privacy Statement Accessibility Disclaimer Digital Strategy Open Source Data Public Data Listing NIH... ...

  3. Fifth Disease

    Science.gov (United States)

    ... recently infected. The blood test may be particularly helpful for pregnant women who may have been exposed to parvovirus B19 and are suspected to have fifth disease. Top of Page Prevention & Treatment Prevention There is no vaccine or medicine that can prevent parvovirus B19 infection. ...

  4. [Trophoblastic diseases

    NARCIS (Netherlands)

    Kate-Booij, M.J. ten; Lok, C.A.; Verheijen, R.H.; Massuger, L.F.A.G.; Trommel, N.E. van

    2008-01-01

    Hydatidiform mole is the most frequently-occurring gestational trophoblastic disease (GTD). Patients with GTD have elevated human chorionic gonadotrophin (HCG) produced by the trophoblast. After evacuation of the mole, weekly serum HCG determinations can be used to assess whether the trophoblast is

  5. Prionic diseases

    Directory of Open Access Journals (Sweden)

    Abelardo Q-C Araujo

    2013-09-01

    Full Text Available Prion diseases are neurodegenerative illnesses due to the accumulation of small infectious pathogens containing protein but apparently lacking nucleic acid, which have long incubation periods and progress inexorably once clinical symptoms appear. Prions are uniquely resistant to a number of normal decontaminating procedures. The prionopathies [Kuru, Creutzfeldt-Jakob disease (CJD and its variants, Gerstmann-Sträussler-Scheinker (GSS syndrome and fatal familial insomnia (FFI] result from accumulation of abnormal isoforms of the prion protein in the brains of normal animals on both neuronal and non-neuronal cells. The accumulation of this protein or fragments of it in neurons leads to apoptosis and cell death. There is a strong link between mutations in the gene encoding the normal prion protein in humans (PRNP - located on the short arm of chromosome 20 – and forms of prion disease with a familial predisposition (familial CJD, GSS, FFI. Clinically a prionopathy should be suspected in any case of a fast progressing dementia with ataxia, myoclonus, or in individuals with pathological insomnia associated with dysautonomia. Magnetic resonance imaging, identification of the 14-3-3 protein in the cerebrospinal fluid, tonsil biopsy and genetic studies have been used for in vivo diagnosis circumventing the need of brain biopsy. Histopathology, however, remains the only conclusive method to reach a confident diagnosis. Unfortunately, despite numerous treatment efforts, prionopathies remain short-lasting and fatal diseases.

  6. Huntington's disease

    DEFF Research Database (Denmark)

    Hjermind, Lena Elisabeth; Law, Ian; Jønch, Aia

    2011-01-01

    In this open-label pilot study, the authors evaluated the effect of memantine on the distribution of brain glucose metabolism in four Huntington's disease (HD) patients as determined by serial 18-fluoro-deoxyglucose [F(18)]FDG-PET scans over a period of 3-4 months (90-129 days, with one patient...

  7. Parasitic Diseases

    Science.gov (United States)

    ... to live. You can get them from contaminated food or water, a bug bite, or sexual contact. Some parasitic diseases are easily treated and some are not. Parasites range in size from tiny, one-celled organisms called protozoa to worms that can be seen with the naked eye. ...

  8. Blount Disease

    Science.gov (United States)

    ... a while. You'll also probably need physical therapy . The good news is that most teens make a complete recovery. The Outlook Most teens who have surgery to correct Blount disease can return to all their normal activities, even competitive sports. One lesson many people take away from dealing ...

  9. Graves' Disease

    Science.gov (United States)

    ... to pump enough blood to the body (congestive heart failure). Thyroid storm. A rare, but life-threatening complication of Graves' disease is thyroid storm, also known as accelerated hyperthyroidism or thyrotoxic crisis. It's more likely when severe hyperthyroidism is untreated ...

  10. Cushing disease

    Science.gov (United States)

    ... Symptoms Symptoms of Cushing disease include: Upper body obesity (above the waist) and thin arms and legs ... blood glucose and A1C to test for diabetes Lipid and cholesterol ... Medicine, Division of Metabolism, Endocrinology & Nutrition, University of Washington School of Medicine, ...

  11. Crohn's Disease

    Science.gov (United States)

    ... nutrition are sometimes given to Crohn’s disease patients to give the inflamed intestine a respite from solid food. top of page This page was reviewed on May 24, 2016 Send us your feedback Did you find the information you were looking ...

  12. Rh Disease

    Science.gov (United States)

    ... immunoglobulin called Rho(D) immune globulin (brand name RhoGAM®). RhoGAM can prevent your body from producing Rh antibodies ... and future pregnancies won’t get Rh disease. RhoGAM doesn’t work if your body has already ...

  13. Cardiovascular disease

    African Journals Online (AJOL)

    user

    +2348153319600. ABSTRACT: Background: Cardiovascular disease is a global epidemic; the prevalence is currently stable in the developed world but .... that culminate in malnutrition are believed to predominate. However, urbanization of .... risk factors promotes migration of inflammatory cells from the circulation and the ...

  14. Periodontal diseases.

    Science.gov (United States)

    Kinane, Denis F; Stathopoulou, Panagiota G; Papapanou, Panos N

    2017-06-22

    Periodontal diseases comprise a wide range of inflammatory conditions that affect the supporting structures of the teeth (the gingiva, bone and periodontal ligament), which could lead to tooth loss and contribute to systemic inflammation. Chronic periodontitis predominantly affects adults, but aggressive periodontitis may occasionally occur in children. Periodontal disease initiation and propagation is through a dysbiosis of the commensal oral microbiota (dental plaque), which then interacts with the immune defences of the host, leading to inflammation and disease. This pathophysiological situation persists through bouts of activity and quiescence, until the affected tooth is extracted or the microbial biofilm is therapeutically removed and the inflammation subsides. The severity of the periodontal disease depends on environmental and host risk factors, both modifiable (for example, smoking) and non-modifiable (for example, genetic susceptibility). Prevention is achieved with daily self-performed oral hygiene and professional removal of the microbial biofilm on a quarterly or bi-annual basis. New treatment modalities that are actively explored include antimicrobial therapy, host modulation therapy, laser therapy and tissue engineering for tissue repair and regeneration.

  15. Fabry disease

    Directory of Open Access Journals (Sweden)

    Germain Dominique P

    2010-11-01

    Full Text Available Abstract Fabry disease (FD is a progressive, X-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal α-galactosidase A activity. FD is pan-ethnic and the reported annual incidence of 1 in 100,000 may underestimate the true prevalence of the disease. Classically affected hemizygous males, with no residual α-galactosidase A activity may display all the characteristic neurological (pain, cutaneous (angiokeratoma, renal (proteinuria, kidney failure, cardiovascular (cardiomyopathy, arrhythmia, cochleo-vestibular and cerebrovascular (transient ischemic attacks, strokes signs of the disease while heterozygous females have symptoms ranging from very mild to severe. Deficient activity of lysosomal α-galactosidase A results in progressive accumulation of globotriaosylceramide within lysosomes, believed to trigger a cascade of cellular events. Demonstration of marked α-galactosidase A deficiency is the definitive method for the diagnosis of hemizygous males. Enzyme analysis may occasionnally help to detect heterozygotes but is often inconclusive due to random X-chromosomal inactivation so that molecular testing (genotyping of females is mandatory. In childhood, other possible causes of pain such as rheumatoid arthritis and 'growing pains' must be ruled out. In adulthood, multiple sclerosis is sometimes considered. Prenatal diagnosis, available by determination of enzyme activity or DNA testing in chorionic villi or cultured amniotic cells is, for ethical reasons, only considered in male fetuses. Pre-implantation diagnosis is possible. The existence of atypical variants and the availability of a specific therapy singularly complicate genetic counseling. A disease-specific therapeutic option - enzyme replacement therapy using recombinant human α-galactosidase A - has been recently introduced and its long term outcome is currently still being investigated. Conventional management consists of pain relief with

  16. Morgellons disease?

    Science.gov (United States)

    Accordino, Robert E; Engler, Danielle; Ginsburg, Iona H; Koo, John

    2008-01-01

    Morgellons disease, a pattern of dermatologic symptoms very similar, if not identical, to those of delusions of parasitosis, was first described many centuries ago, but has recently been given much attention on the internet and in the mass media. The present authors present a history of Morgellons disease, in addition to which they discuss the potential benefit of using this diagnostic term as a means of building trust and rapport with patients to maximize treatment benefit. The present authors also suggest "meeting the patient halfway" and creating a therapeutic alliance when providing dermatologic treatment by taking their cutaneous symptoms seriously enough to provide both topical ointments as well as antipsychotic medications, which can be therapeutic in these patients.

  17. [Crohn's disease].

    Science.gov (United States)

    Márquez, V R; Sanz, M; Calderaro-di Ruggiero, F; Daza Ramírez, A; Casale Ochoa, E; Márquez Atars, I

    1989-01-01

    A 48 years old male patient was admitted in the Risquez Hospital of Caracas, Venezuela presenting intestinal obstruction. An exploratory laparotomy was performed and two stenotic lesions in the small intestine were found. One of them in the proximal jejunum and the other in distal ileum. Both were resected and terminal jejunum-jejunum anastomosis and terminal jejunum-ileum anastomosis were performed. Pathological diagnosis was Crohn's Disease. Post operative evolution was satisfactory and four years after the patient is well.

  18. Pattern classification of enterovirus 71-associated hand, foot, and mouth disease in chinese medicine: A retrospective study in 433 cases.

    Science.gov (United States)

    Liu, Yan; He, Li-Yun; Wen, Tian-Cai; Yan, Shi-Yan; Bai, Wen-Jing; Liu, Bao-Yan

    2017-10-17

    To determine whether patterns of enterovirus 71 (EV71)-associated hand, foot, and mouth disease (HFMD) were classified based on symptoms and signs, and explore whether individual characteristics were correlated with membership in particular pattern. Symptom-based latent class analysis (LCA) was used to determine whether patterns of EV71-HFMD existed in a sample of 433 cases from a clinical data warehouse system. Logistic regression was then performed to explore whether demographic, and laboratory data were associated with pattern membership. LCA demonstrated a two-subgroup solution with an optimal fifit, deduced according to the Bayesian Information Criterion minima. Hot pattern (59.1% of all patients) was characterized by a very high fever and high endorsement rates for classical HFMD symptoms (i.e., rash on the extremities, blisters, and oral mucosa lesions). Non-hot pattern (40.9% of all patients) was characterized by classical HFMD symptoms. The multiple logistic regression results suggest that white blood cell counts and aspartate transaminase were positively correlated with the hot pattern (adjust odds ratio=1.07, 95% confifidence interval: 1.006-1.115; adjust odds ratio=1.051, 95% confifidence interval: 1.019-1.084; respectively). LCA on reported symptoms and signs in a retrospective study allowed different subgroups with meaningful clinical correlates to be defifined. These fifindings provide evidence for targeted prevention and treatment interventions.

  19. 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

    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

  20. Heavy Chain Diseases

    Science.gov (United States)

    ... heavy chain produced: Alpha Gamma Mu Alpha Heavy Chain Disease Alpha heavy chain disease (IgA heavy chain ... disease or lead to a remission. Gamma Heavy Chain Disease Gamma heavy chain disease (IgG heavy chain ...

  1. Diabetic Eye Disease

    Science.gov (United States)

    ... Disease, & Other Dental Problems Diabetes & Sexual & Urologic Problems Diabetic Eye Disease What is diabetic eye disease? Diabetic eye disease is a group ... eye diseases that can threaten your sight are Diabetic retinopathy The retina is the inner lining at ...

  2. Chronic Beryllium Disease

    Science.gov (United States)

    ... Science Education & Training Home Conditions Chronic Beryllium Disease Chronic Beryllium Disease Make an Appointment Find a Doctor Ask a ... MD, MSPH, FCCP (February 01, 2016) What is chronic beryllium disease (CBD)? Chronic beryllium disease (CBD) is a disease ...

  3. What Is Vascular Disease?

    Science.gov (United States)

    ... Policy What Is Vascular Disease? What Is Vascular Disease? Vascular disease is any abnormal condition of the blood ... Privacy Policy × Your ticket for the: What Is Vascular Disease? Title What Is Vascular Disease? USD Close Print

  4. Vimentin may reflect areas of pathologic involvement in biopsies from patients with autoimmune skin diseases

    Directory of Open Access Journals (Sweden)

    Ana Maria Abreu Velez

    2014-04-01

    Full Text Available Introduction: Autoimmune bullous skin diseases (ABDs represent a group of disorders of the skin and mucosa commonly associated with deposits of immunoglobulins, complement and fibrinogen, and usually directed against distinct adhesion molecules. After studing these diseases for many years, we noted alterations not only between the cells junctions of the epidermis and/or the dermal/epidermal junction, but also in dermal skin appendageal structures and in mesenchymal tissue around the blisters. Based on our findings, we wanted to determine if the observed patterns of autoimmunity correlated with cutaneous vimentin expression. Materials and Methods: Archival biopsies previously diagnosed with ABDs by clinical, hematoxylin and eosin (H&E and direct and/or immunofluorescence data were stained with antibodies directed against vimentin via immunohistochemistry (IHC. 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, 14 with dermatitis herpetiformis (DH and 3 with Senear-Usher syndrome. Results: The H&E, DIF and vimentin patterns of positivity in the different ABDs confirmed that vimentin was compartmentalized around areas of dermal inflammation, around skin appendages and in epidermal, dermal and mesenchymal cell junction areas. Conclusion: Vimentin may be a useful tool for highlighting patterns of microenvironmental tissue alteration in multiple ABDs. The vimentin staining pattern observed was analogous to that we have previously described for proteases and protease inhibitors in patients affected by ABDs, expanding the concept that the autoimmune process extends beyond cell junctions.

  5. Cardiovascular Disease

    Directory of Open Access Journals (Sweden)

    Cheung Angela

    2004-08-01

    Full Text Available Abstract Health Issue Cardiovascular disease (CVD is the leading cause of death in Canadian women and men. In general, women present with a wider range of symptoms, are more likely to delay seeking medial care and are less likely to be investigated and treated with evidence-based medications, angioplasty or coronary artery bypass graft than men. Key Findings In 1998, 78,964 Canadians died from CVD, almost half (39,197 were women. Acute myocardial infarction, which increases significantly after menopause, was the leading cause of death among women. Cardiovascular disease accounted for 21% of all hospital admissions for Canadian women over age 50 in 1999. Admissions to hospital for ischemic heart disease were more frequent for men, but the mean length of hospital stay was longer for women. Mean blood pressure increases with age in both men and women. After age 65, however, high blood pressure is more common among Canadian women. More than one-third of postmenopausal Canadian women have hypertension. Diabetes increases the mortality and morbidity associated with CVD in women more than it does in men. Depression also contributes to the incidence and recovery from CVD, particularly for women who experience twice the rate of depression as men. Data Gaps and Recommendations CVD needs to be recognized as a woman's health issue given Canadian mortality projections (particularly heart failure. Health professionals should be trained to screen, track, and address CVD risk factors among women, including hypertension, elevated lipid levels, smoking, physical inactivity, depression, diabetes and low socio-economic status.

  6. [MENETRIER DISEASE].

    Science.gov (United States)

    Maev, I V; Andreev, D N; Samsonov, A A

    2015-01-01

    Menetrier disease (MD) is a very rare stomach pathology of unknown etiology characterized by manifest hypertrophy of gastric mucosa. The main causes of MD are believed to be Helicobacter pylori and cytomegalovirus infections. The most frequent symptom is epigastric pain. Also common are peripheral oedema due to hypoalbuminemia and increased permeability of gastric mucosa. The main diagnostic signs of MD include diffusive enhancement of mucosal folds, foveolar hyperplasia and glandular atrophy with a decrease in the number of main and parietal cells, hypoalbuminemia and peripheral oedema. MD being a very rare condition, the optimal methodfor its treatment is unknown.

  7. Cardiovascular disease.

    Science.gov (United States)

    Gavagan, Thomas

    2002-06-01

    The primary care physician is in a position to advise patients on the efficacy of alternative and complementary therapies as they relate to cardiovascular diseases. Anti-oxidant vitamin supplementation has not been shown to be efficacious in decreasing cardiovascular events. N-3 fatty acids appear to be beneficial in secondary prevention of cardiovascular events but their use in primary prevention is not clear. Adoption of vegetable-based diets, including whole grains, can be recommended to decrease cardiovascular events, lower cholesterol and help lower blood pressure. For patients with hypercholesterolemia, cholestin, a red-yeast rice supplement, has been shown to be effective. Garlic supplements may have some mild cholesterol-lowering effect, but this effect is not significant enough to recommend clinically. Herbal therapies with hawthorn and ubiquinone (Q10) are of possible benefit in congestive heart failure. An integrated program of rigorous diet, exercise and stress reduction in motivated patients with cardiovascular disease may have value as an alternative to cardiovascular medications and surgical interventions.

  8. Association between periodontal diseases and systemic diseases

    OpenAIRE

    Patrícia Weidlich; Renata Cimões; Claudio Mendes Pannuti; Rui Vicente Oppermann

    2008-01-01

    Current evidence suggests that periodontal disease may be associated with systemic diseases. This paper reviewed the published data about the relationship between periodontal disease and cardiovascular diseases, adverse pregnancy outcomes, diabetes and respiratory diseases, focusing on studies conducted in the Brazilian population. Only a few studies were found in the literature focusing on Brazilians (3 concerning cardiovascular disease, 7 about pregnancy outcomes, 9 about diabetes and one r...

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

    Science.gov (United States)

    Licarete, Emilia; Ganz, Susanne; Recknagel, Martin J; Di Zenzo, Giovanni; Hashimoto, Takashi; Hertl, Michael; Zambruno, Giovanna; Hundorfean, Gheorghe; Mudter, Jonas; Neurath, Markus F; Bruckner-Tuderman, Leena; Sitaru, Cassian

    2012-04-04

    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. 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). 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. 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 prove a useful tool for clinical and translational

  10. 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

  11. Alzheimer Disease

    Science.gov (United States)

    Apostolova, Liana G.

    2016-01-01

    ABSTRACT Purpose of Review: This article discusses the recent advances in the diagnosis and treatment of Alzheimer disease (AD). Recent Findings: In recent years, significant advances have been made in the fields of genetics, neuroimaging, clinical diagnosis, and staging of AD. One of the most important recent advances in AD is our ability to visualize amyloid pathology in the living human brain. The newly revised criteria for diagnosis of AD dementia embrace the use for biomarkers as supportive evidence for the underlying pathology. Guidelines for the responsible use of amyloid positron emission tomography (PET) have been developed, and the clinical and economic implications of amyloid PET imaging are actively being explored. Summary: Our improved understanding of the clinical onset, progression, neuroimaging, pathologic features, genetics, and other risk factors for AD impacts the approaches to clinical diagnosis and future therapeutic interventions. PMID:27042902

  12. Hirayama disease

    Directory of Open Access Journals (Sweden)

    Atul T Tayade

    2010-01-01

    Full Text Available A 17-year-old male, who gave up his favorite sport cricket and started playing football, presented with one-year history of slowly progressive atrophic weakness of forearms and hands. Neurological examination showed weak and wasted arms, forearms and hand but no evidence of pyramidal tract, spinothalmic tract and posterior column lesions. Plain cervical spine radiographs showed no abnormal findings. Cervical magnetic resonance imaging (MRI showed asymmetric cord atrophy; images obtained with neck flexed showed the anterior shifting of the posterior wall of the lower cervical dural sac resulting in cord compression. These findings suggest Hirayama disease, a kind of cervical myelopathy related to the flexion movements of the neck.

  13. Vibroacoustic disease.

    Science.gov (United States)

    Branco, N A A Castelo; Alves-Pereira, M

    2004-01-01

    Vibroacoustic disease (VAD) is a whole-body, systemic pathology, characterized by the abnormal proliferation of extra-cellular matrices, and caused by excessive exposure to low frequency noise (LFN). VAD has been observed in LFN-exposed professionals, such as, aircraft technicians, commercial and military pilots and cabin crewmembers, ship machinists, restaurant workers, and disk-jockeys. VAD has also been observed in several populations exposed to environmental LFN. This report summarizes what is known to date on VAD, LFN-induced pathology, and related issues. In 1987, the first autopsy of a deceased VAD patient was performed. The extent of LFN induced damage was overwhelming, and the information obtained is, still today, guiding many of the associated and ongoing research projects. In 1992, LFN-exposed animal models began to be studied in order to gain a deeper knowledge of how tissues respond to this acoustic stressor. In both human and animal models, LFN exposure causes thickening of cardiovascular structures. Indeed, pericardial thickening with no inflammatory process, and in the absence of diastolic dysfunction, is the hallmark of VAD. Depressions, increased irritability and aggressiveness, a tendency for isolation, and decreased cognitive skills are all part of the clinical picture of VAD. LFN is a demonstrated genotoxic agent, inducing an increased frequency of sister chromatid exchanges in both human and animal models. The occurrence of malignancies among LFN-exposed humans, and of metaplastic and displastic appearances in LFN-exposed animals, clearly corroborates the mutagenic outcome of LFN exposure. The inadequacy of currently established legislation regarding noise assessments is a powerful hindrance to scientific advancement. VAD can never be fully recognized as an occupational and environmental pathology unless the agent of disease--LFN--is acknowledged and properly evaluated. The worldwide suffering of LFN-exposed individuals is staggering and it is

  14. Newcastle Disease Virus (PDQ)

    Science.gov (United States)

    ... to Ask about Your Treatment Research Newcastle Disease Virus (PDQ®)–Patient Version Overview Go to Health Professional ... Question 8 ). Questions and Answers About Newcastle Disease Virus What is Newcastle disease virus? Newcastle disease virus ( ...

  15. Kidney Disease Basics

    Science.gov (United States)

    ... Heart Disease Mineral & Bone Disorder What Is Chronic Kidney Disease? Chronic kidney disease (CKD) means your kidneys ... work, be active, and enjoy life. Will my kidneys get better? Kidney disease is often “progressive”, which ...

  16. Living with Kawasaki Disease

    Science.gov (United States)

    ... Research Home / Kawasaki Disease Kawasaki Disease What Is Kawasaki (KAH-wah-SAH-ke) disease is a rare ... condition involves inflammation of the blood vessels. In Kawasaki disease, the walls of the blood vessels throughout ...

  17. Progression of Liver Disease

    Science.gov (United States)

    ... The Progression of Liver Disease Diagnosing Liver Disease – Liver Biopsy and Liver Function Tests Clinical Trials Liver Transplant ... The Progression of Liver Disease Diagnosing Liver Disease: Liver Biopsy and Liver Function Tests Clinical Trials Liver Transplant ...

  18. Poorly Responsive Celiac Disease

    Science.gov (United States)

    ... Celiac Disease › Poorly Responsive Celiac Disease Poorly Responsive Celiac Disease It is estimated that up to 30% of ... continuing to ingest gluten. Causes of Poorly Responsive Celiac Disease Continuing Gluten Ingestion The most common reason for ...

  19. Testing for Kidney Disease

    Science.gov (United States)

    ... hypertension artérielle Heart Disease Mineral & Bone Disorder Chronic Kidney Disease Tests & Diagnosis How can I tell if I have kidney disease? Early kidney disease usually doesn’t have any ...

  20. Fatty Liver Disease

    Science.gov (United States)

    ... two main types: Nonalcoholic fatty liver disease (NAFLD) Alcoholic fatty liver disease, also called alcoholic steatohepatitis What is nonalcoholic ... lead to cirrhosis or liver cancer. What is alcoholic fatty liver disease? Alcoholic fatty liver disease is due to ...

  1. Parkinson's Disease Dementia

    Science.gov (United States)

    ... Find your local chapter Join our online community Parkinson's Disease Dementia Parkinson's disease dementia is an impairment ... disease. About Symptoms Diagnosis Causes & risks Treatments About Parkinson's disease dementia The brain changes caused by Parkinson's ...

  2. Thyroid Disease and Teens

    Science.gov (United States)

    ... Situations Talking to Your Parents - or Other Adults Thyroid Disease KidsHealth > For Teens > Thyroid Disease Print A ... other parts of your body. continue What Is Thyroid Disease? Thyroid disease occurs when the thyroid gland ...

  3. Thyroid Disease (for Parents)

    Science.gov (United States)

    ... Late for the Flu Vaccine? Eating Disorders Arrhythmias Thyroid Disease KidsHealth > For Parents > Thyroid Disease Print A ... many other parts of the body. What Is Thyroid Disease? Thyroid disease is when the thyroid gland ...

  4. About Chronic Kidney Disease

    Science.gov (United States)

    ... Advocacy Donate A to Z Health Guide About Chronic Kidney Disease Tweet Share Print Email Chronic kidney disease (CKD) ... Learn about Glomerular Filtration Rate (GFR) What is chronic kidney disease (CKD)? Chronic kidney disease includes conditions that damage ...

  5. Men and Heart Disease

    Science.gov (United States)

    ... Pressure Salt Cholesterol Million Hearts® WISEWOMAN Men and Heart Disease Fact Sheet Recommend on Facebook Tweet Share Compartir Source: Interactive Atlas of Heart Disease and Stroke Heart Disease Facts in Men Heart disease is the leading ...

  6. [Castleman disease].

    Science.gov (United States)

    Belletti, Gerardo A; Savio, Verónica; Minoldo, Daniel; Caminos, Susana; Yorio, Marcelo A

    2004-01-01

    A 66 years female, who was since last year under astenia, arthralgias, pimply lesions in spread plates and tests showing eritrosedimentation over 100 mm, anemi, leucocitosis with neutrofilia, policlonal hypergammaglobulinemia, slight proteinuria and IgE on 900. This patient was sporadically treated with corticoids. When made the medical consult had lost 34lb., was under anorexy, as well as dyspepsia. Hemoglobyn 6.9 gr/dl, leucocytes 20000/mm3, neutrofils at 90%, proteinogram the same as former, with hypoalbuminemia. She was taking prednisona, 16 mg/day. When examined showed depress of conscience, astenia, and dermic lesions already quoted. 4 cm nonpainful right axillary adenopaty adhered to deep planes. Medulogram with increased iron, hyperegenerative. Ganglionar biopsia: linfoid hyperplasic process linked to inmune response. Toracoabdominal tomography with adenomegalia in torax and retroperitoneo. Skin biopsia: neutrofilic vasculitis. The patient suspends the 16 mg of prednisona and fever as well as generalized adenopatias come up. After laying aside other ethiologies, and understanding as Castleman Multicentric disease, it is started to supply prednisona 1 mg/kg of weight with a clinical and biochemical fast and outstanding response. After 7 months it was progressively suspended the esteroids and 60 days later, the process fall back; for that, corticoids are restarted, with a good evolution. The illness of Castleman although it is not very frequent, it should be considered as differential diagnosis in those clinical cases that are accompanied with important general commitment, linphadenopaties and respons to steroid therapy.

  7. [Fukuhara disease].

    Science.gov (United States)

    Fukuhara, Nobuyoshi

    2008-01-01

    MERRF is an acronym of myoclonus epilepsy associated with ragged-red fibers and was first reported as a new nosological entity belonging to mitochondrial encephalomyopathies in San Remo symposium on "Mitochondrial Pathology" in 1982. MERRF was named Fukuhara disease by Rowland (1983). The first reported patient had been diagnosed as having Ramsay Hunt syndrome associated with Friedreich's ataxia. However, nowadays, the previously reported cases as having Ramsay Hunt syndrome associated with Friedreich's ataxia are regarded as having been suffered from MERRF. The history in establishing the nosological entity of MERRF was described. Patients with MERRF develop myoclonus, epileptic seizures, cerebellar ataxia, dementia, sensorineural hearing disturbance, optic atrophy, muscular wasting, and foot deformities at the advanced stage. Pathological findings show degeneration of the dentate nuclei, globus pallidus, and red nuclei, substantia nigra, inferior olivary nuclei, cerebellar cortex, and spinal cord. The posterior columns, the spinocerebellar tracts, and Clark's columns are degenerating in the spinal cord. The pyramidal tracts never show a severe degeneration as in Friedreich's ataxia. The skeletal muscles show mitochondrial abnormalities histologically and electron microscopically. Clinical features of MERRF are not necessarily uniform in the early stage and muscle biopsy findings are also very mild in some patients with MERRF, necessitating genetic analysis for diagnosis. Most of patients show a point mutation (A --> G) of nt 8344 in mitochondrial DNA.

  8. Castleman disease and associated autoimmune disease.

    Science.gov (United States)

    Muskardin, Theresa W; Peterson, Bruce A; Molitor, Jerry A

    2012-01-01

    Castleman disease can occur in association with autoimmune connective tissue disease and confound the clinical picture, resulting in delayed diagnosis and suboptimal treatment. This review focuses on the intersection of Castleman disease and autoimmunity with an emphasis on shared pathology and mutually beneficial treatments. Targeting CD-20, interleukin-6, and the nuclear factor-κB pathway has shown promise in achieving long-term remission in patients with Castleman disease and associated autoimmune features. Advances in understanding of pathogenic cell types and cytokines in Castleman disease have allowed the development of targeted therapies successful in the treatment of both Castleman disease and associated autoimmune disease.

  9. Dermatitis herpetiformis – diagnostic difficulties based on the presentation of own cases

    Directory of Open Access Journals (Sweden)

    Katarzyna Łoza

    2014-03-01

    Full Text Available Introduction . Dermatitis herpetiformis (Duhring disease is an autoimmune blistering subepidermal dermatosis characterized by pruritic polymorphic skin eruption accompanied by a clinically asymptomatic gluten-sensitive enteropathy. The etiopathogenesis of the disease is associated with the presence of antibodies against tissue and epidermal transglutaminase. Diagnosis is based on direct immunopathological examination in which granular IgA deposits on the top of dermal papillae are detected. Sulfones are effective in the therapy of skin changes. Objective . The aim of the study is to present difficulties in evaluation of clinical picture, diagnosis and treatment, and monitoring the safety of the therapy in Duhring disease on the basis of three own cases. Case report . Case 1. 37-year-old patient. Itchy skin lesions occurred in mid-2012 and have been treated until now with antihistamines and local corticosteroids without improvement. The diagnosis of dermatitis herpetiformis was established on the basis of direct immunopathology test. Treated with gluten-free diet and dapsone 100 mg/day with improvement. Case 2. 62-year-old patient, in whom the first itchy eruption appeared in October 2012. Treated with antihistamines and topical corticosteroids without improvement. In laboratory examinations elevated level of IgE and triglycerides was found. Histopathological and immunological examinations confirmed the diagnosis of dermatitis herpetiformis. Treated with gluten-free diet and dapsone 150 mg/day with improvement. Case 3. 58-year-old patient. The first changes of typical morphology appeared about 2 years ago. The patient was treated with antihistamines and corticosteroids without improvement. Laboratory tests confirmed the diagnosis of Duhring disease. The introduction of gluten-free diet and dapsone 100 mg/day caused regression of skin changes. Conclusions . Our cases, treated for a long time as eczematous changes, despite the lack of improvement

  10. Prospective studies on the routine use of a novel multivariant enzyme-linked immunosorbent assay for the diagnosis of autoimmune bullous diseases.

    Science.gov (United States)

    van Beek, Nina; Dähnrich, Cornelia; Johannsen, Nora; Lemcke, Susanne; Goletz, Stephanie; Hübner, Franziska; Di Zenzo, Giovanni; Dmochowski, Marian; Drenovska, Kossara; Geller, Shamir; Horn, Michael; Kowalewski, Cezary; Medenica, Ljiljana; Murrell, Dedee F; Patsatsi, Aikaterini; Uzun, Soner; Vassileva, Snejina; Zillikens, Detlef; Schlumberger, Wolfgang; Schmidt, Enno

    2017-05-01

    Serologic diagnosis of autoimmune blistering disease (AIBD) usually follows a sophisticated multistep algorithm. We sought validation of a multivariant enzyme-linked immunosorbent assay (ELISA) in the routine diagnosis of AIBD. The multivariant ELISA comprising 6 recombinant immunodominant forms of major AIBD target antigens, ie, desmoglein 1, desmoglein 3, envoplakin, BP180, BP230, and type VII collagen was applied in: (1) a cohort of well-characterized AIBD (n = 173) and control sera (n = 130), (2) a prospective multicenter study with 204 sera from patients with newly diagnosed AIBD with positive direct immunofluorescence microscopy, and (3) a prospective monocenter study with 292 consecutive sera from patients with clinical suspicion of AIBD in comparison with the conventional multistep diagnostic algorithm. Concordant results in the multivariant ELISA compared with direct immunofluorescence microscopy were seen in 94% of patients with pemphigus and 71% of patients with pemphigoid (Cohen κ value, 0.95 and 0.66) and with the conventional multistep diagnostic approach in 91% of patients with pemphigus and 88% of patients with bullous pemphigoid and 93% of autoantibody-negative sera (Cohen κ, 0.95, 0.84, and 0.78). IgA autoantibodies and less common target antigens were not analyzed. The multivariant ELISA is a practical, highly standardized, and widely available novel diagnostic tool for the routine diagnosis of AIBD. Copyright © 2016 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  11. Associated Autoimmune Diseases

    Science.gov (United States)

    ... commonly associated with celiac disease - Grave’s disease and Hashimoto’s disease. Grave’s Disease: An overactive thyroid. Symptoms may ... feeling too warm, restlessness, insomnia, diarrhea, irritability, palpitations. Hashimoto’s Disease: An underactive thyroid. Symptoms may include weight ...

  12. Alzheimer's Disease: The Death of the Disease.

    Science.gov (United States)

    McBroom, Lynn W.

    1987-01-01

    Alzheimer's disease, a form of dementia in middle-age and older adults is becoming more evident because of growing numbers of older people and better diagnosis and detection methods. Describes the behavioral and physical symptoms of the disease as well as specific suggestions for care of patients with Alzheimer's disease, including dealing with…

  13. Chronic kidney disease and cardiovascular disease

    African Journals Online (AJOL)

    2007-08-16

    Aug 16, 2007 ... disease (CKD). It is unclear how much of the association between kidney and vascular disease results from. • vascular disease causing kidney failure. • kidney failure causing vascular ... patients with CKD, with acute myocardial infarction accounting for 20% of ... failure and death. Valvular calcification may.

  14. Lysosomal storage disease 2 - Pompe's disease

    NARCIS (Netherlands)

    van der Ploeg, Ans T.; Reuser, Arnold J. J.

    2008-01-01

    Pompe's disease, glycogen-storage disease type II, and acid maltase deficiency are alternative names for the same metabolic disorder. It is a pan-ethnic autosomal recessive trait characterised by acid alpha-glucosidase deficiency leading to lysosomal glycogen storage. Pompe's disease is also

  15. Understanding cardiovascular disease

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/patientinstructions/000759.htm Understanding cardiovascular disease To use the sharing features on this ... lead to heart attack or stroke. Types of Cardiovascular Disease Coronary heart disease (CHD) is the most ...

  16. Learn About Neuromuscular Disease

    Science.gov (United States)

    ... for MDA Blog Donate Search MDA.org Close Learn About Neuromuscular Disease Muscular dystrophy, ALS and related ... power of a multiple-disease approach, MDA leverages learnings from one disease to accelerate progress in others ...

  17. Heart Disease and Stroke

    Science.gov (United States)

    ... email updates Enter email Submit Heart Disease and Stroke Heart disease and stroke are important health issues ... Stroke risk factors View more Heart Disease and Stroke resources Related information Heart-healthy eating Stress and ...

  18. Pediatric Celiac Disease

    Science.gov (United States)

    ... of Pediatric Gastroenterology and Nutrition Nurses Print Share Celiac Disease Many kids have sensitivities to certain foods, and ... protein found in wheat, rye, and barley. Pediatric Celiac Disease If your child has celiac disease, consuming gluten ...

  19. Pregnancy and Kidney Disease

    Science.gov (United States)

    ... Donate A to Z Health Guide Pregnancy and Kidney Disease Tweet Share Print Email A new baby is ... disease and pregnancy. Can a woman with "mild" kidney disease have a baby? That depends. There is good ...

  20. Chronic Kidney Diseases

    Science.gov (United States)

    ... Weight for Me? Your Teeth Heart Murmurs Chronic Kidney Diseases KidsHealth > For Kids > Chronic Kidney Diseases Print A ... pressure at a healthy level. continue Kinds of Kidney Diseases Like any complicated machine, not all kidneys work ...

  1. Parkinson disease - discharge

    Science.gov (United States)

    Your doctor has told you that you have Parkinson disease . This disease affects the brain and leads ... have you take different medicines to treat your Parkinson disease and many of the problems that may ...

  2. Chronic Kidney Disease (CKD)

    Science.gov (United States)

    ... store Donate Now Give Monthly Give In Honor Chronic kidney disease (CKD) www.kidneyfund.org > Kidney Disease > Chronic Kidney ... treated? Kidney-friendly diet for CKD What causes chronic kidney disease (CKD)? Anyone can get CKD. Some people are ...

  3. Lyme Disease Data

    Science.gov (United States)

    ... Lyme disease FAQ Health care providers Educational materials Data and Statistics Recommend on Facebook Tweet Share Compartir ... sixth most common Nationally Notifiable disease . Lyme Disease Data File To facilitate the public health and research ...

  4. 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 ...

  5. Liver Disease and IBD

    Science.gov (United States)

    ... Home > Resources > Liver Disease and IBD Go Back Liver Disease and IBD Email Print + Share Several complications ... be necessary to make the definitive diagnosis. FATTY LIVER DISEASE (HEPATCI STEATOSIS) This is the most common ...

  6. Eye Disease Simulations

    Science.gov (United States)

    ... USAJobs Home > Eye Health Information > Eye Disease Simulations Eye Disease Simulations Age-Related Macular Degeneration Cataract Diabetic ... information page Back to top Diabetic Retinopathy Diabetic Eye Disease information page Back to top Glaucoma Glaucoma ...

  7. Quiz: Alzheimer's Disease

    Science.gov (United States)

    ... of this page please turn JavaScript on. Feature: Alzheimer's Disease Quiz: Alzheimer's Disease Past Issues / Winter 2015 Table of Contents ... How many Americans over age 65 may have Alzheimer's disease? as many as 5 million as many ...

  8. Pelvic Inflammatory Disease (PID)

    Science.gov (United States)

    Pelvic Inflammatory Disease (PID) - CDC Fact Sheet Untreated sexually transmitted diseases (STDs) can cause pelvic inflammatory disease (PID), a serious condition, in women. 1 in 8 women with a history of ...

  9. Celiac Disease Tests

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    ... Acidosis and Alkalosis Adrenal Insufficiency and Addison Disease Alcoholism Allergies Alzheimer Disease Anemia Angina Ankylosing Spondylitis Anthrax ... Seems to Be on the Rise, Mainly in Elderly: Study Blood markers for the disease rose from ...

  10. Heart Diseases and Disorders

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    ... Resources Heart Diseases & Disorders Back to Patient Resources Heart Diseases & Disorders Millions of people experience irregular or abnormal ... harmless and happen in healthy people free of heart disease. However, some abnormal heart rhythms can be serious ...

  11. Heart Disease (For Kids)

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    ... System Taking Care of Your Teeth Bad Breath Heart Disease KidsHealth > For Kids > Heart Disease Print A A ... chest pain, heart attacks, and strokes . What Is Heart Disease? The heart is the center of the cardiovascular ...

  12. Lyme disease (image)

    Science.gov (United States)

    Lyme disease is an acute inflammatory disease characterized by skin changes, joint inflammation and symptoms similar to ... that is caused by the bacterium Borrelia burgdorferi . Lyme disease is transmitted by the bite of a ...

  13. Parkinson's Disease Videos

    Medline Plus

    Full Text Available ... Anxiety in Parkinson's Disease Expert Briefings: Cognitive Issues: Advice for Parkinson's Care Partners Expert Briefings: Nutrition and Parkinson's Disease NY Nightly News with Chuck ...

  14. PDE4 Inhibition as Potential Treatment of Epidermolysis Bullosa Acquisita

    NARCIS (Netherlands)

    Koga, Hiroshi; Recke, Andreas; Vidarsson, Gestur; Pas, Hendri H.; Jonkman, Marcel F.; Hashimoto, Takashi; Kasprick, Anika; Ghorbanalipoor, Saeedeh; Tenor, Hermann; Zillikens, Detlef; Ludwig, Ralf J.

    2016-01-01

    Pemphigoid diseases such as epidermolysis bullosa acquisita (EBA) may be difficult to treat. In pemphigoid diseases, mucocutaneous blistering is caused by autoantibodies to hemidesmosomal antigens; in EBA the autoantigen is type VII collagen. Despite growing insights into pemphigoid disease

  15. Childhood epidermolysis bullosa acquisita: Confirmation of diagnosis by skin deficient in Type VII Collagen, enzyme-linked immunosorbent assay, and immunoblotting

    Directory of Open Access Journals (Sweden)

    Nupur Goyal

    2016-01-01

    Full Text Available Epidermolysis bullosa acquisita (EBA is an acquired subepidermal bullous disorder characterized by autoantibodies against Type VII collagen. It usually affects adults; childhood EBA is rare. We describe a 10-year-old girl presenting with recurrent tense blisters predominantly on legs, dorsa of hands and feet accompanied by oral erosions since the age of 5 years. Direct immunofluorescence (IF microscopy showed linear deposition of IgG and C3 along the basement membrane zone (BMZ; indirect IF microscopy on salt-split skin revealed staining of IgG to the dermal side of the split. The patient's serum did not show BMZ staining in recessive dystrophic epidermolysis bullosa skin deficient for Type VII collagen, thus confirming autoantibody reactivity against Type VII collagen. Circulating antibodies against the immunodominant noncollagenous 1 domain of Type VII collagen were detected by ELISA and immunoblotting studies. The patient was treated with oral corticosteroids and dapsone with good improvement.

  16. Factitious Dermatitis Due to Thermal Burn With Histologic Features Simulating Fixed Drug Eruption.

    Science.gov (United States)

    Persad, Leah; Salim, Sadia; Motaparthi, Kiran

    2017-08-01

    Factitious dermatitis (FD) (dermatitis artefacta) is rare and often difficult to diagnose because of conflicting history and nonspecific clinical and histologic findings. It can present with varied clinical features including geometric ulcers, erosions, and less commonly bullae secondary to external trauma from chemicals, electric burns, heat, and suction. Herein, we describe a case of bullous FD due to thermal burn with histologic features demonstrating overlap with fixed drug eruption. Histopathology demonstrated a subepidermal blister with epidermal necrosis along with pigment incontinence and dermal eosinophils and neutrophils. Although these features, and the clinician's impression, were suggestive of fixed drug eruption, several morphologic findings allowed accurate diagnosis of FD: sharp demarcation of necrotic keratinocytes from adjacent uninvolved epidermis, elongated keratinocytes reminiscent of thermal or electrical artifact, and multinucleated keratinocytes. Although FD is often considered a diagnosis of exclusion, these clues may help dermatopathologists distinguish this entity from inflammatory dermatoses.

  17. Pemphigoid Vegetans in Childhood: A Case Report and Short Review of Literature

    Science.gov (United States)

    Khatib, Yasmeen; Makhija, Meena; Patel, Richa D; Karad, Gayatri

    2015-01-01

    Pemphigoid vegetans is a very rare type of bullous pemphigoid which usually affects the elderly and has not been reported in children. It shows a clinical resemblance to pemphigus vegetans but has distinct histological and immunopathological features of bullous pemphigoid. A 9-year-old girl presented with recurrent purulent and verrucous vegetating lesions on her forehead, groin and vulva along with scaling, crusted, bullous and purulent lesions on the eyelids, periorbital, periauricular, perioral region and lips. She had oral lesions and a cerebriform tongue. Though she showed clinical features of pemphigus vegetans, histology revealed a subepidermal blister with the absence of acantholysis. Direct immunofluorescence studies were suggestive of bullous pemphigoid. On clinicopathological correlation, a diagnosis of pemphigoid vegetans was made. She responded well to oral corticosteroids and dapsone therapy with complete resolution of the lesions. PMID:26288447

  18. Pemphigoid vegetans in childhood: A case report and short review of literature

    Directory of Open Access Journals (Sweden)

    Yasmeen Khatib

    2015-01-01

    Full Text Available Pemphigoid vegetans is a very rare type of bullous pemphigoid which usually affects the elderly and has not been reported in children. It shows a clinical resemblance to pemphigus vegetans but has distinct histological and immunopathological features of bullous pemphigoid. A 9-year-old girl presented with recurrent purulent and verrucous vegetating lesions on her forehead, groin and vulva along with scaling, crusted, bullous and purulent lesions on the eyelids, periorbital, periauricular, perioral region and lips. She had oral lesions and a cerebriform tongue. Though she showed clinical features of pemphigus vegetans, histology revealed a subepidermal blister with the absence of acantholysis. Direct immunofluorescence studies were suggestive of bullous pemphigoid. On clinicopathological correlation, a diagnosis of pemphigoid vegetans was made. She responded well to oral corticosteroids and dapsone therapy with complete resolution of the lesions.

  19. Childhood Epidermolysis Bullosa Acquisita: Confirmation of Diagnosis by Skin Deficient in Type VII Collagen, Enzyme-linked Immunosorbent Assay, and Immunoblotting.

    Science.gov (United States)

    Goyal, Nupur; Rao, Raghavendra; Balachandran, C; Pai, Sathish; Bhogal, Balbir S; Schmidt, Enno; Zillikens, Detlef

    2016-01-01

    Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disorder characterized by autoantibodies against Type VII collagen. It usually affects adults; childhood EBA is rare. We describe a 10-year-old girl presenting with recurrent tense blisters predominantly on legs, dorsa of hands and feet accompanied by oral erosions since the age of 5 years. Direct immunofluorescence (IF) microscopy showed linear deposition of IgG and C3 along the basement membrane zone (BMZ); indirect IF microscopy on salt-split skin revealed staining of IgG to the dermal side of the split. The patient's serum did not show BMZ staining in recessive dystrophic epidermolysis bullosa skin deficient for Type VII collagen, thus confirming autoantibody reactivity against Type VII collagen. Circulating antibodies against the immunodominant noncollagenous 1 domain of Type VII collagen were detected by ELISA and immunoblotting studies. The patient was treated with oral corticosteroids and dapsone with good improvement.

  20. Biomarker for Glycogen Storage Diseases

    Science.gov (United States)

    2017-07-03

    Fructose Metabolism, Inborn Errors; Glycogen Storage Disease; Glycogen Storage Disease Type I; Glycogen Storage Disease Type II; Glycogen Storage Disease Type III; Glycogen Storage Disease Type IV; Glycogen Storage Disease Type V; Glycogen Storage Disease Type VI; Glycogen Storage Disease Type VII; Glycogen Storage Disease Type VIII