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

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Joycelin Fernandes

2014-01-01

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

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

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

2014-11-01

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

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

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Daniel Chang

2012-02-01

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

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

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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.

Bullous Wells’ syndrome

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Bengu Cevirgen Cemil

2016-01-01

Full Text Available Wells’ syndrome (WS is an uncommon inflammatory skin disease which typically presents single or multiple erythematous and edematous urticarial plaques similar to cellulitis. The lesions may evolve into blue-grey morphea-like lesions and may persist for weeks or months. They ultimately heal without scar. Other clinical presentations reported in literature include papular and nodular and, rarely, bullous eruptions. Previously, bullous Wells’ syndrome was rarely reported in the literature. Herein, we describe a case of a female patient with bullous Wells’ syndrome localized to the upper limbs without any associated disorders.

Measuring of quality of life in autoimmune blistering disorders in Poland. Validation of disease - specific Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires.

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Kalinska-Bienias, Agnieszka; Jakubowska, Beata; Kowalewski, Cezary; Murrell, Dedee F; Wozniak, Katarzyna

2017-03-01

Autoimmune bullous dermatoses (AIBD) are rare, severe diseases resulting from some antibodies activity against the different adhesion structures within the skin and/or mucosa. Few studies investigated quality of life (QOL) in AIBD by generic and dermatology-specific instruments, all reporting strong impact on QOL. Recently, disease-specific measurement tools have been developed: Autoimmune Bullous Disease Quality of Life (ABQOL) and Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires. The aim of this study was to test the reliability and validity of ABQOL and TABQOL by developing the first foreign language versions and to evaluate ABQOL and TABQOL in Polish patients. The study enrolled 80 patients from the tertiary referral center for AIBD at the outpatient clinic or on admission to the hospital. Sixty six patients completed the 17-item questionnaires of each ABQOL and TABQOL at day 0 and after 5-7 days. Both questionnaires were translated into Polish according to protocol. The internal consistency and test-retest reliability were high (Cronbach α=0.95 for ABQOL, α=0.87 for TABQOL), (R=0.98 for ABQOL, R=0.86 for TABQOL). In convergent validity, the correlation of ABQOL and TABQOL was strong (R=0.81), but low with objective disease activity scales. The strongest impact of AIBD on QOL has been observed in flares and in patients with the onset below 70 years of age. The patients with bullous pemphigoid had the highest QOL compared to other AIBD patients. The ABQOL and TABQOL are reliable and valid instruments for the assessment of QOL in AIBD. Copyright © 2016 Medical University of Bialystok. Published by Elsevier B.V. All rights reserved.

Linear IgA Bullous Dermatosis

DEFF Research Database (Denmark)

Lings, Kristina; Bygum, Anette

2015-01-01

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

IgA LINEAR BULLOUS DERMATOSIS IN CHILDHOOD.

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Ivelina Yordanova; Valentin Valtchev; Dimitar Gospodinov; Snejina Vassileva

2015-01-01

IgA linear bullous dermatosis, also known as chronic bullous dermatosis of childhood, is an autoimmune disease which may be idiopathic or drug-induced. The disease affects children and adults. We present a 4 years old girl with itchy polymorphic eruptions. The skin rash was presented by bullous-erosive rosette-like lesions with reddish-brown crust in the center, distributed on the skin of the face, trunk and extremities. The vesicles were filled with serous and hemorrhagic content. Laboratory...

Autoimmune responses in patients with linear IgA bullous dermatosis: both autoantibodies and T lymphocytes recognize the NC16A domain of the BP180 molecule.

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Lin, Mong-Shang; Fu, Chang-Ling; Olague-Marchan, Monica; Hacker, Mary K; Zillikens, Detlef; Giudice, George J; Fairley, Janet A

2002-03-01

Linear IgA bullous disease (LABD) is an autoimmune skin disease characterized by subepidermal blisters and IgA autoantibodies directed against the epidermal basement membrane zone (BMZ) of the skin. Various antigens have been identified as targets of IgA autoantibodies including BP180, a type II glycoprotein that spans the BMZ and lamina lucida. Previously, we have identified a subset of LABD patients whose sera contained IgA antibodies against the 16th noncollagenous (NC16A) domain of BP180. NC16A was previously shown to harbor epitopes that are recognized by both autoantibodies and T cells from patients with bullous pemphigoid and herpes gestationis and is thought to be associated with the development of these immunobullous diseases. The aim of this study was to determine whether T lymphocytes from LABD patients with anti-NC16A IgA autoantibodies respond to epitopes in the same region of the BP180 protein. Indeed, of the four LABD patients in our study, all had T cells that specifically proliferated in response to NC16A. Moreover, two subfragments of NC16A were identified as the predominant targets of LABD T cells. Further analysis of T cell lines and clones derived from these patients revealed that these cells express a CD4 memory T cell phenotype and secrete a Th1/Th2 mixed-cytokine profile, characteristics similar to those of T cells in bullous pemphigoid patients. Our data suggest that the BP180 protein, typically the NC16A region, is the common target of both cellular and humoral immune responses in some LABD patients. This information helps to further elucidate the autoimmune mechanisms in this disease.

Idiopathic linear IgA bullous dermatosis: prognostic factors based on a case series of 72 adults.

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Gottlieb, J; Ingen-Housz-Oro, S; Alexandre, M; Grootenboer-Mignot, S; Aucouturier, F; Sbidian, E; Tancrede, E; Schneider, P; Regnier, E; Picard-Dahan, C; Begon, E; Pauwels, C; Cury, K; Hüe, S; Bernardeschi, C; Ortonne, N; Caux, F; Wolkenstein, P; Chosidow, O; Prost-Squarcioni, C

2017-07-01

Linear IgA bullous dermatosis (LABD) is a clinically and immunologically heterogeneous, subepidermal, autoimmune bullous disease (AIBD), for which the long-term evolution is poorly described. To investigate the clinical and immunological characteristics, follow-up and prognostic factors of adult idiopathic LABD. This retrospective study, conducted in our AIBD referral centre, included adults, diagnosed between 1995 and 2012, with idiopathic LABD, defined as pure or predominant IgA deposits by direct immunofluorescence. Clinical, histological and immunological findings were collected from charts. Standard histology was systematically reviewed, and indirect immunofluorescence (IIF) on salt-split skin (SSS) and immunoblots (IBs) on amniotic membrane extracts using anti-IgA secondary antibodies were performed, when biopsies and sera obtained at diagnosis were available. Prognostic factors for complete remission (CR) were identified using univariate and multivariate analyses. Of the 72 patients included (median age 54 years), 60% had mucous membrane (MM) involvement. IgA IIF on SSS was positive for 21 of 35 patients tested; 15 had epidermal and dermal labellings. Immunoelectron microscopy performed on the biopsies of 31 patients labelled lamina lucida (LL) (26%), lamina densa (23%), anchoring-fibril zone (AFz) (19%) and LL+AFz (23%). Of the 34 IgA IBs, 22 were positive, mostly for LAD-1/LABD97 (44%) and full-length BP180 (33%). The median follow-up was 39 months. Overall, 24 patients (36%) achieved sustained CR, 19 (29%) relapsed and 35% had chronic disease. CR was significantly associated with age > 70 years or no MM involvement. No prognostic immunological factor was identified. Patients with LABD who are < 70 years old and have MM involvement are at risk for chronic evolution. © 2017 British Association of Dermatologists.

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

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

2017-07-01

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

Remission Time after Rituximab Treatment for Autoimmune Bullous Disease: A Proposed Update Definition.

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Iranzo, Pilar; Pigem, Ramon; Giavedoni, Priscila; Alsina-Gibert, Mercè

2015-01-01

A therapeutic endpoint is a very important tool to evaluate response in clinical trials. In 2005, a consensus statement identified two late endpoints of disease activity in pemphigus: complete remission off therapy and complete remission on therapy, both definitions applying to patients without lesions for at least 2 months. The same period of time was considered for partial remission off/on therapy. These definitions were later applied to bullous pemphigoid and are considered in most studies on autoimmune bullous disease. These endpoints were established for different adjuvant agents, but at that moment, rituximab was not considered. Rituximab is known for the long duration of its effect, and in most studies relapses have been reported later than 6 months after treatment. In our opinion, time to remission after rituximab treatment should be redefined. © 2015 S. Karger AG, Basel.

IgA LINEAR BULLOUS DERMATOSIS IN CHILDHOOD.

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Ivelina Yordanova

2015-12-01

Full Text Available IgA linear bullous dermatosis, also known as chronic bullous dermatosis of childhood, is an autoimmune disease which may be idiopathic or drug-induced. The disease affects children and adults. We present a 4 years old girl with itchy polymorphic eruptions. The skin rash was presented by bullous-erosive rosette-like lesions with reddish-brown crust in the center, distributed on the skin of the face, trunk and extremities. The vesicles were filled with serous and hemorrhagic content. Laboratory examinations were within normal values according the age. Histopathological examination of the lesional skin revealed sub epidermal blister. Direct immunofluorescence of perilesional skin demonstrated linear deposition of IgA in the basement membrane. Systemic treatment with Methylprednisolon and Claritromycin was applied with satisfactory effect. The patient is under observation.

Modes of Action of Intravenous Immunoglobulin in Bullous Pemphigoid.

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Li, Ning; Culton, Donna; Diaz, Luis A; Liu, Zhi

2018-06-01

Bullous pemphigoid is an autoantibody-mediated skin blistering disease. Previous studies revealed that intravenous Ig is therapeutic in animal models of bullous pemphigoid by saturating the IgG-protective receptor FcRn, thereby accelerating degradation of pathogenic IgG. Sasaoka et al. demonstrate that the inhibitory effects of intravenous Ig on bullous pemphigoid are also associated with negative modulation of cytokine production by keratinocytes. Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.

Bleb Point: Mimicker of Pneumothorax in Bullous Lung Disease

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Gelabert, Christopher

2015-05-01

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

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

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Hojat Eftekhari

2016-07-01

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

Coexisting Morphea and Bullous Lichen Sclerosus Et Atrophicus: A Case Report

OpenAIRE

Nurcan Metin; Mahizer Yaldız; Teoman Erdem; Şahin Erdem

2015-01-01

Morphea and lichen skleroatrofikans (LSA)are localized fibrosing diseases characterized by well-defined fibrotic plaques. Blisters rarely occur on morphea plaque, and these blisters are mostly considered as bullous morphea which is a rare type of morphea. The cases of bullous LSA growing on plaque morphea are rarer. In this case report, we presented a patient has bullous lesions compatible with of lichen skleroatrofikans clinically and histopathologically on morphea lesions, to draw attention...

[Humoral immune diseases: Cutaneous vasculitis and auto-immune bullous dermatoses].

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Wechsler, Janine

2018-02-01

Humoral immunity is the cause of multiple diseases related to antibodies (IgA, IgG, IgM) produced by the patient. Two groups of diseases are identified. The first group is related to circulating antigen-antibody complexes. The antigens are various. They are often unknown. These immune complexes cause a vascular inflammation due to the complement fixation. Consequently, this group is dominated by inflammatory vasculitis. In the second group, the pathology is due to the fixation in situ of antibodies to a target antigen of the skin that is no more recognized by the patient. This group is represented by the auto-immune bullous dermatoses. Copyright © 2017. Published by Elsevier Masson SAS.

Subepidermal moisture detection of heel pressure injury: The pressure ulcer detection study outcomes.

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Bates-Jensen, Barbara M; McCreath, Heather E; Nakagami, Gojiro; Patlan, Anabel

2018-04-01

We examined subepidermal moisture (SEM) and visual skin assessment of heel pressure injury (PrI) among 417 nursing home residents in 19 facilities over 16 weeks. Participants were older (mean age 77 years), 58% were female, over half were ethnic minorities (29% African American, 12% Asian American, 21% Hispanic), and at risk for PrI (mean Braden Scale Risk score = 15.6). Blinded concurrent visual assessments and SEM measurements were obtained at heels weekly. Visual skin damage was categorised as normal, erythema, stage 1 PrI, deep tissue injury (DTI) or stage 2 or greater PrI. PrI incidence was 76%. Off-loading occurred with pillows (76% of residents) rather than heel boots (21%) and often for those with DTI (91%). Subepidermal moisture was measured with a device where higher readings indicate greater moisture (range: 0-70 tissue dielectric constant), with normal skin values significantly different from values in the presence of skin damage. Subepidermal moisture was associated with concurrent damage and damage 1 week later in generalised multinomial logistic models adjusting for age, diabetes and function. Subepidermal moisture detected DTI and differentiated those that resolved, remained and deteriorated over 16 weeks. Subepidermal moisture may be an objective method for detecting PrI. © 2017 Medicalhelplines.com Inc and John Wiley & Sons Ltd.

Origin of the subepidermal tissue in Piper L. leaves.

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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.

Bullous pemphigoid induced by vildagliptin: a report of three cases.

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Béné, Johana; Jacobsoone, Aurélie; Coupe, Patrick; Auffret, Marine; Babai, Samy; Hillaire-Buys, Dominique; Jean-Pastor, Marie-Josèphe; Vonarx, Marlène; Vermersch, Annie; Tronquoy, Anne-Fleur; Gautier, Sophie

2015-02-01

To report three cases of bullous pemphigoid in patients treated with vildagliptin. Case 1: An 86-year-old woman presented with bullous pemphigoid after 1 month of treatment with vildagliptin and metformin. After introduction of clobetasol, the symptoms resolved although vildagliptin was continued. However, the skin lesions reappeared 3 months later. Sustained remission was achieved only after definitive withdrawal of vildagliptin. Case 2: A 79-year-old man presented with bullous pemphigoid after 37-month treatment with gliclazide, vildagliptin and metformin. The disease at first responded to clobetasol but 3 months later the lesions reappeared. They finally regressed when the gliptin was discontinued. Case 3: A 77-year-old woman, treated with gliclazide and vildagliptin for 26 months, presented with bullous pemphigoid, which responded well to discontinuation of the gliptin and topical clobetasol. Gliptins are new molecules for treatment of type 2 diabetes mellitus, which have been suspected of implication in bullous pemphigoid. Such cases have been described in the literature (seven with vildagliptin and three with sitagliptin). In nine of these cases, the gliptin was associated with metformin, but the latter had never been considered responsible. The mechanism implicated in the development of bullous pemphigoid has not yet been clearly identified, but may involve a modified immune response or alteration of the antigenic properties of the epidermal basement membrane. These reports support the risk of bullous pemphigoid in patients exposed to gliptins. © 2014 Société Française de Pharmacologie et de Thérapeutique.

Linear IgA dermatosis associated with ulcerative colitis: complete and sustained remission after total colectomy

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Vargas,Thiago Jeunon de Sousa; Fialho,Mônica; Santos,Luiza Tavares dos; Rodrigues,Palmira Assis de Jesus Barreto; Vargas,Ana Luisa Bittencourt Sampaio Jeunon; Sousa,Maria Auxiliadora Jeunon

2013-01-01

Linear IgA dermatosis has been increasingly associated with inflammatory bowel diseases, particularly ulcerative colitis. A 13-year-old male patient with an 11-month history of ulcerative colitis developed vesicles, pustules and erosions on the skin of the face, trunk and buttocks and in the oral mucosa. The work-up revealed a neutrophil-rich sub-epidermal bullous disease and linear deposition of IgA along the dermoepidermal junction, establishing the diagnosis of linear IgA dermatosis. The p...

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.

Linear IgA Bullous Dermatosis:A Retrospective Study of 23 Patients in Denmark

OpenAIRE

Lings, Kristina; Bygum, Anette

2015-01-01

Linear IgA bullous dermatosis (LAD) is an autoimmune, chronic bullous disease affecting primarily young children and adults. Studies on LAD are relatively sparse and from Scandinavia we could only find a few case reports. Therefore we decided to conduct a retrospective investigation of patients seen at our department since 1972. The objective is to give a description of the different subgroups of patients with LAD with regard to precipitating factors, demographics, treatments, course of disea...

Association of Autoantibodies to BP180 with Disease Activity in Greek Patients with Bullous Pemphigoid

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Aikaterini Patsatsi

2012-01-01

Full Text Available 39 bullous pemphigoid (BP patients were studied to assess the clinical significance of anti-BP180 and anti-BP230 circulating autoantibodies of BP and correlate their titers with the clinical scores of the BP Disease Area Index (BPDAI and the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS as well as with the intensity of pruritus measured by the BPDAI pruritus component. All parameters were evaluated by the time of diagnosis (baseline, month 3, and month 6. Titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (, and ABSIS (, values, as well as with BPDAI component for the intensity of pruritus (, at baseline. At month 3, titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (, and ABSIS (, values, as well as with the BPDAI component for the intensity of pruritus (, . At month 6, titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (, and ABSIS (, values, as well as with the BPDAI component for the intensity of pruritus (, . There was no statistically significant correlation between titers of anti-BP230 autoantibodies and the BPDAI, ABSIS, and BPDAI component for the intensity of pruritus at the same time points.

Bullous Henoch–Schonlein purpura with involvement of face

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Devdeep Mukherjee

2017-01-01

Full Text Available Henoch–Schonlein purpura (HSP with facial involvement with bullous rashes are extremely rare. A 12-year-old boy presented with abdominal pain and features of arthritis. He also had multiple purpuric rashes over his lower limbs. Gradually, he developed bullous rashes which were seen on his legs and hands and progressed to involve the face. He was confirmed to be suffering from HSP from clinical presentation and skin biopsy. The child responded well to oral steroids. Bullous lesions may be seen in HSP. However, there is neither prognostic significance of this nor does it alter the management. Other causes of bullous lesions should be ruled out. As facial involvement is associated with renal and gastrointestinal involvement, these children should be monitored for sequelae.

Treatment of Intraepidermal Autoimmune Bullous Diseases Sürekli Eğitim

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Tamer İrfan Kaya

2011-06-01

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

Intertriginous bullous morphea: A clue for the pathogenesis?

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Kavala Mukaddes

2007-01-01

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

Bullous pilomatricoma: A stage in transition to secondary anetoderma?

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Premanshu Bhushan

2012-01-01

Full Text Available Pilomatricoma is an uncommon hamartomatous tumor of the hair matrix. Bullous and anetodermic changes over pilomatricoma are rare. We report an 18-year-old male with an asymptomatic nodule with overlying cystic changes on the left arm of 6-month duration with clinical and histological features of both bullous and anetodermic modifications. We also reviewed the associated literature to conclude that there is sufficient overlap in these two entities. Both variants show a bullous or pseudobullous appearance clinically and loss of elastin, sparse collagen bundles separated by intense edema, and dilated lymphatics/blood vessels in the dermis overlying the tumor mass histologically. We therefore propose that bullous, pseudoampullary, anetodermic, and lymphangiectatic forms should be considered as synonymous or transitional to the ultimate scar-like anetodermic appearance.

B-cell activating factor detected on both naïve and memory B cells in bullous pemphigoid.

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Qian, Hua; Kusuhara, Masahiro; Li, Xiaoguang; Tsuruta, Daisuke; Tsuchisaka, Atsunari; Ishii, Norito; Koga, Hiroshi; Hayakawa, Taihei; Ohara, Koji; Karashima, Tadashi; Ohyama, Bungo; Ohata, Chika; Furumura, Minao; Hashimoto, Takashi

2014-08-01

B-cell activating factor (BAFF), an important immune regulatory cytokine, is involved in development of autoimmune diseases. Although BAFF is expressed in various cells, including dendritic cells (DCs) and monocytes, BAFF expression on B cells has not been well documented. In the present study, BAFF molecules on DCs and naïve and memory B cells in autoimmune bullous diseases, including pemphigus vulgaris, pemphigus foliaceus and bullous pemphigoid (BP), were analysed by flow cytometry. Compared with healthy controls (HC), BAFF expression on naïve and memory B cells increased significantly in BP. No difference in BAFF receptor expression in naïve and memory B cells was shown among all study groups. Furthermore, BAFF expression in both naïve and memory B cells of BP, but not HC, was detected by confocal microscopic analysis. These results implied that BAFF expressed by B cells may play a pathogenic role in autoimmune bullous diseases, particularly BP. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Concurrent pyogenic granuloma and bullous impetigo of a pregnant woman's finger.

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Qin, Rosie; Cohen, Philip R

2017-03-15

Bullous impetigo is a superficial skininfection caused by Staphylococcus aureus (S.aureus). Pyogenic granuloma is a common benigntumor frequently associated with prior trauma.Bullous impetigo and pyogenic granuloma may occurin pregnant women. The features of a pregnant womanwith pyogenic granuloma and bullous impetigoconcurrently present in a lesion on her finger aredescribed. PubMed was used to search the followingterms: bullous impetigo, pregnancy, and pyogenicgranuloma. All papers were reviewed; relevantarticles, along with their references, were evaluatedResults: A red ulcerated nodule with a collaretteof epithelium around the tumor and surroundingbullae appeared on the fifth digit of the left hand of a31-year-old woman who was at 36 weeks gestation. Abacterial culture grew methicillin sensitive S. aureus.An excisional biopsy was performed. Histologicfindings revealed not only a benign vascular tumorwith an infiltrate of mixed inflammatory cells, butalso an intraepidermal blister. She received oralantibiotics and there was complete resolution of thefinger lesion and infection with preservation of digitfunction. Albeit uncommon, pyogenic granulomaand bullous impetigo may concurrently occur in thesame lesion. Therapeutic intervention should focuson treating both the benign skin tumor and theinfection.

Bullous Lichen Planus in an Indian female | Puri | Sudanese Journal ...

African Journals Online (AJOL)

Bullous lichen planus is a rare entity. We describe bullous lichen planus in an. Indian female who had itchy erythematous papular lesions with vesicles and bullae predominantly over the dorsa of both legs. The patient was subjected to histopathological examination after which the diagnosis was confirmed. The patient was ...

Vanishing Lung Syndrome: Compound Effect of Tobacco and Marijuana Use on the Development of Bullous Lung Disease – A Joint Effort

OpenAIRE

Wiesel, Shimshon; Siddiqui, Faraz; Khan, Tahir; Hossri, Sami; El-Sayegh, Dany

2017-01-01

Marijuana use has been increasing across the United States due to its legalization as both a medicinal and recreational product. A small number of case reports have described a pathological entity called vanishing lung syndrome (VLS), which is a rare bullous lung disease usually caused by tobacco smoking. Recent case reports have implicated marijuana in the development of VLS. We present a case of a 47-year-old man, who presented to our hospital with shortness of breath, fevers and a producti...

Association of linear IgA bullous disease with ulcerative colitis: a case of successful treatment with infliximab.

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Yamada, S; Makino, T; Jinnin, M; Sakai, K; Fukushima, S; Inoue, Y; Ihn, H

2013-01-01

Linear IgA bullous disease (LABD) has been reported in association with inflammatory bowel disease, in particular ulcerative colitis (UC). We reporting a 34-year-old female who developed LABD during a flare-up of UC. We administered infliximab, which has been approved for the treatment of UC; infliximab dramatically improved the cutaneous lesions and bowel symptoms. This is the first report showing a marked effect of infliximab on LABD. First, we hypothesize that infliximab works for UC and then calms down excessive production of inflammatory cytokines and autoantibodies, and so stricter control of UC by infliximab is beneficial against the skin condition of LABD. Second, we suggest that TNF-α production in the lesion of LABD is increased, so TNF-α plays an important role in developing cutaneous lesions. This case suggests that infliximab, a monoclonal antibody against TNF-α, is efficacious in the cutaneous symptoms of LABD.

A case of bullous pemphigoid exacerbated by irradiation after breast conservative radiotherapy

International Nuclear Information System (INIS)

Isohashi, Fumiaki; Konishi, Koji; Umegaki, Noriko; Tanei, Tomonori; Koizumi, Masahiko; Yoshioka, Yasuo

2011-01-01

We present a case, considered to be a form of the Koebner phenomenon, of bullous pemphigoid that was exacerbated mainly within the irradiated field after breast conservative radiotherapy. In May 2009, a 60-year-old woman was diagnosed with bullous pemphigoid, which was treated with steroid therapy. The following month, she was diagnosed with breast cancer (invasive ductal carcinoma, pT1cN0M0). After breast conservative surgery in December 2009, conservative radiotherapy to the right breast was performed (50 Gy in 25 fractions). Portal skin showed no serious change (up to grade 1 skin erythema) and no bullous neogenesis during conservative radiotherapy. However, 2 months after conservative radiotherapy, new blisters became exacerbated mainly within the irradiated field but also in the area outside the irradiated field. Increasing the dosage of oral steroid and minocycline resulted in relief of bullous pemphigoid, although patchy skin pigmentation remained especially in the irradiated skin. (author)

Successful treatment of bullous lichen planus with acitretin monotherapy. Review of treatment options for bullous lichen planus and case report.

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Rallis, Efstathios; Liakopoulou, Angeliki; Christodoulopoulos, Constantinos; Katoulis, Alexandros

2016-12-31

Bullous lichen planus (BLP) is a rare variant of lichen planus, characterized by the development of vesicular and bullous lesions, of skin, nails, hair and/or mucosa. We present a case of 63-year-old woman with BLP, unresponsive to previous therapies with topical corticosteroids, topical calcipotriol, antihistamines and oral cyclosporine (4 mg/kg/day for 4 months). She was already receiving treatment for arterial hypertension, hyperlipidemia, atrial fibrillation and uncontrolled diabetes mellitus. Acitretin was administered for 5 months with complete remission of BLP lesions and no major side effects. This is probably the first reported case of BLP treated with acitretin monotherapy. In this case acitretin was an efficacious and well-tolerated therapeutic option for BLP.

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

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Minezaki T

2013-07-01

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

HLA-DPDQDR is expressed in all lesional skin from patients with autoimmune skin diseases

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Ana Maria Abreu Velez

2014-04-01

Full Text Available Introduction: Human genes responsible for human antigen presentation and transplant rejection functions are located on the short arm of Chromosome 6 and are called the Major Histocompatibility Complex (MHC. Moreover, the primary physiologic function of MHC molecules is to present peptides to T lymphocytes. MHC molecules are integral components of the ligands that most T cells recognize, since the T cell receptor (TCR has specificity for complexes of foreign antigenic peptides, as well as self-MHC molecules. Aim: Our investigation attempts to investigate the presence of HLA-DPDQDR within lesional skin biopsies from patients affected by autoimmune skin blistering diseases (ABDs. Materials and Methods: We utilized immunohistochemistry (IHC to evaluate the presence of HLA-DPDQDR in lesional skin biopsies of patients affected by ABDs. We tested 30 patients with endemic pemphigus foliaceus (EPF, 15 controls from the EPF endemic area, and 15 biopsies from healthy controls from the USA. We also tested archival biopsies from patients with selected ABDs, including 30 patients with bullous pemphigoid (BP, 20 with pemphigus vulgaris (PV, 8 with pemphigus foliaceus (PF, 14 with dermatitis herpetiformis (DH and 2 with epidermolysis bullosa acquisita (EBA. Results: Most ABD biopsies stained positive for HLA-DPDQDR in the lesional blisters and/or inflamed neurovascular plexus in the superficial dermis, and also at mesenchymal-endothelial like-cell junctions in the dermis. In BP, EBA and EPF, the HLA-DPDQDR staining was also seen in the dermal eccrine sweat gland coils and and ducts. Conclusion: Here, we document that HLA-DPDQDR is expressed in several anatomic areas of lesional skin in patients with ABDs. Notably, HLA-DPDQDR positivity was also consistently present in areas of the classic immune response in pemphigus epidermal keratinocytic intercellular junctions, and at basement membrane sites in bullous pemphigoid and other subepidermal blistering diseases.

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

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Chanprapaph, K; Sawatwarakul, S; Vachiramon, V

2017-10-01

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

The value of Tzanck smear test in diagnosis of erosive, vesicular, bullous, and pustular skin lesions.

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Durdu, Murat; Baba, Mete; Seçkin, Deniz

2008-12-01

Tzanck smear is generally used for the diagnosis of the pemphigus group of autoimmune bullous diseases and mucocutaneous herpesvirus infections. There are only a few studies in the literature investigating its diagnostic value. We aimed to investigate Tzanck smear findings and to determine the diagnostic value of this test in moist (erosive, vesicular, bullous, and pustular) skin lesions. We also aimed to develop an algorithmic approach for the diagnosis of these types of skin lesions according to the Tzanck smear findings. Samples were stained with May-Grünwald-Giemsa and evaluated by the same dermatologist. In some patients, methylene blue and Gram staining or direct immunofluorescence examinations were additionally performed. In all of the study cases, after the evaluation of clinical and laboratory findings (including, when appropriate, potassium hydroxide examination; viral serology; bacterial and fungal cultures; histopathology; direct and indirect immunofluorescence; patch testing), the definite diagnosis was established. We also determined the sensitivity and the specificity of certain Tzanck smear findings. Tzanck smear was performed in a total of 400 patients with moist skin lesions. The sensitivities of multinucleated giant cells and acantholytic cells in herpetic infections, dyskeratotic acantholytic cells and cocci in bullous impetigo, pseudohyphae in candidiasis, acantholytic cells in pemphigus and more than 10 tadpole cells (magnification x100) in spongiotic dermatitis were 84.7%, 92%, 100%, 100%, and 81.5%, respectively. Because Tzanck smears were evaluated by the same dermatologist, no comment could be made regarding the interobserver reliability of this test and how the level of experience with this technique might affect the results. Also, the sensitivity and the specificity of Tzanck smear test findings for certain diseases could not be calculated because of an insufficient number of patients. The Tzanck smear test is an inexpensive, useful

Compositional analysis of Chinese water chestnut (Eleocharis dulcis) cell-wall material from parenchyma, epidermis, and subepidermal tissues.

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Grassby, Terri; Jay, Andrew J; Merali, Zara; Parker, Mary L; Parr, Adrian J; Faulds, Craig B; Waldron, Keith W

2013-10-09

Chinese water chestnut (Eleocharis dulcis (Burman f.) Trin ex Henschel) is a corm consumed globally in Oriental-style cuisine. The corm consists of three main tissues, the epidermis, subepidermis, and parenchyma; the cell walls of which were analyzed for sugar, phenolic, and lignin content. Sugar content, measured by gas chromatography, was higher in the parenchyma cell walls (931 μg/mg) than in the subepidermis (775 μg/mg) or epidermis (685 μg/mg). The alkali-extractable phenolic content, measured by high-performance liquid chromatography, was greater in the epidermal (32.4 μg/mg) and subepidermal cell walls (21.7 μg/mg) than in the cell walls of the parenchyma (12.3 μg/mg). The proportion of diferulic acids was higher in the parenchyma. The Klason lignin content of epidermal and subepidermal cell walls was ~15%. Methylation analysis of Chinese water chestnut cell-wall polysaccharides identified xyloglucan as the predominant hemicellulose in the parenchyma for the first time, and also a significant pectin component, similar to other nongraminaceous monocots.

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

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Isadora Gracia

2006-04-01

Full Text Available A case of chronic bullous type mucocutaneous disease involving oral mucosa was reported from a 56 years old man with never healing oral ulcers and wound on the perianal skin for three years. There were also red and black spots on the limb and back skin and a lesion on nail. Painful oral lesion consisted of mucous erosion, desquamative gingivitis, and sloughing area on palate and tongue. The patient is diabetic. The first perianal skin diagnosis was granulomatous candidasis with differential diagnosis pemphigus vegetates and acuminarum condiloma. However the histopathologic examination did not support these diagnosis. After several histopathologic examinations, the latest perianal skin diagnosis was lichen planus with differential diagnosis granulomatous vasculitis, bowenoid papulosis and pyodema gangrenosum. Other skin diagnosis was erythema multiforme. Oral diagnosis was mucous membrane pemphigoid with differential diagnosis lichen planus, Behçet's syndrome and erythema multiforme. Oral histopathologic examinations showed a sub-epithelial blister, which supported mucous membrane pemphigoid. A lip balm, prednisone 5 mg oral rinse and multivitamins were given but oral improvement started after blood sugar level was controlled. Conclusion: It is not yet known whether skin and oral mucous lesions are from the same disease or not.

Ceratopatia bolhosa: etiopatogênese e tratamento Bullous keratopathy: etiopathogenesis and treatment

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Eliana Domingues Gonçalves

2008-12-01

Full Text Available A ceratopatia bolhosa caracteriza-se pelo edema corneano estromal acompanhado de bolhas epiteliais e subepiteliais devido à perda de células e/ou alterações da junção endotelial. Nos casos mais avançados, ocorre espessamento do estroma e presença de fibrose subepitelial e vascularização corneana. Apresenta baixa de acuidade visual devido à diminuição da transparência da córnea e pode estar acompanhada de sensação de corpo estranho, lacrimejamento e dor devido as alterações epiteliais como a presença de bolhas íntegras ou rotas. Ceratite bolhosa é uma das principais causas de transplante de córnea em diferentes regiões e países. A principal etiologia é a perda de células endoteliais, principalmente após cirurgia de catarata e na distrofia endotelial de Fuchs. Sabe-se que atualmente há cerca de 20 milhões de pessoas com catarata no mundo, e esta complicação pode afetar 1 a 2% das cirurgias de catarata. Este texto faz uma revisão sobre a etiopatogênese da ceratopatia bolhosa e sobre os tratamentos clínicos e cirúrgicos disponíveis para a doença.Bullous keratopathy is characterized by corneal stromal edema with epithelial or subepithelial bullae due to cell loss and endothelial decompensation. In more advanced cases, subepithelial fibrosis, formation of a posterior collagenous layer or retrocorneal fibrous membrane, and corneal vascularization can occur. Decreased vision is present because the loss of corneal transparency with symptoms like tearing and pain caused by epithelial bullae that can rupture. Currently, bullous keratopathy is the most common indication for penetrating keratoplasty and regraft. The main etiology is the endothelial cell loss after ophthalmic surgeries as cataract surgery. Cataract affects approximately 20 million people worldwide, and this complication can occur in 1 to 2% of cataract surgeries. This study revised bullous keratopathy etiopathogenesis, clinical and surgical treatment

Short-term evaluation of non-absorbable microgranular hydroxyapatite infiltration in the guinea pig subepidermal abdominal region

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A.P. Xavier

2002-01-01

Full Text Available Non-absorbable microgranular hydroxyapatite was infiltrated into the subepidermal abdominal region of guinea pigs in order to assess the possibility of using this material to correct deficiencies in orbital volume. Microgranular hydroxyapatite (2.0 ml was subepidermally infiltrated into the abdominal region of 20 guinea pigs. The animals were divided into four experimental groups of 5 animals each, which were killed 7 (G1, 15 (G2, 30 (G3 and 60 (G4 days after infiltration. The area and the largest and smallest diameters of the nodules formed by infiltration were evaluated at the site of infiltration and histological examination was performed. The mean granuloma area was similar in all groups. Histopathological examination showed that the material remained isolated from surrounding tissues by a pseudocapsule that became denser throughout the experiment. A host reaction started with young fibroblastic tissue that evolved to dense tissue until cartilaginous tissue was formed in G4, progressively advancing towards the center of the granuloma from G1 to G4. Non-absorbable microgranular hydroxyapatite is an inert material that was well tolerated by the animals studied, with maintenance of the infiltrated volume, and may perhaps be useful to fill anophthalmic cavities.

Identification of the target areas to be resected in patients with non-bullous emphysema by using gas and perfusion SPECT images

International Nuclear Information System (INIS)

Sugi, Kazuro; Ueda, Kazuhiro; Fujita, Nobuhiro; Nawata, Kouichi; Kaneda, Yoshikazu; Nawata, Sumihiko; Esato, Kensuke

1997-01-01

Significant improvement of pulmonary function after the volume reduction surgery has been reported in patients with bullous emphysema. However, there has been no successful report in patients with non-bullous emphysema. The reason of failure in patients with non-bullous emphysema should lie mainly on the difficulty to identify the target areas to be resected. We describe how to identify the target areas in the patients with non-bullous emphysema by using gas and perfusion single-photon emission computed tomography (SPECT). Twelve patients with non-bullous emphysema were studied by 133 Xe gas and 99m Tc MAA SPECT, which revealed that abnormal retention and low perfusion areas were located in the apex of the upper lobe and the basal segment of the lower lobe. The resections were performed thoracoscopically focused on the target areas by hockey-stick shape resection in the upper lobe and spiral shape resection in the lower lobe without deformity formation of the residual lung. Slight but significant improvement in the pulmonary functions was observed after the surgery in patients with non-bullous emphysema. The Xe gas and Tc MAA SPECT were useful procedures to identify the target areas to be resected in patients with non-bullous emphysema. (author)

Herpes Simplex Virus Infection Mimicking Bullous Disease in an Immunocompromised Patient

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Anne L.Y. Lecluse

2010-06-01

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

Squamous cell carcinoma of penis with bullous pemphigoid masquerading as lymphogranuloma venereum

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Venugopal, V.; Gnanaraj, Pushpa; Fernandes, S. D.; Narasimhan, Murali; Karthikeyan, Subashini; Rahul, Shwetha

2013-01-01

We report a case of a 60-year old unmarried male who presented with multiple ulcers and foul smelling discharge from the groin since 4 months and multiple tense bullae over the trunk of 1 month duration. Groove sign was present. Investigations for lymphogranuloma venereum (LGV) and other sexually transmitted diseases were negative. Histopathology from the ulcer in the groin and growth in the penis revealed squamous cell carcinoma (SCC). Skin biopsy of bulla was diagnostic of bullous pemphigoid (BP). We report a rare case of SCC masquerading as LGV with BP occurring as a paraneoplastic phenomenon. PMID:23919055

Failure of initial disease control in bullous pemphigoid: a retrospective study of hospitalized patients in a single tertiary center.

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Kremer, Noa; Zeeli, Tal; Sprecher, Eli; Geller, Shamir

2017-10-01

Bullous pemphigoid (BP) is the most prevalent autoimmune blistering skin disease in Western countries and in Israel. Initial disease control is achieved in 60-90% of BP patients within 1-4 weeks of corticosteroid therapy. In the remainder of patients, recalcitrant disease is controlled with additional immunosuppressive treatment. We aimed to evaluate the rate of BP patients who needed adjuvant therapy to achieve initial disease control and to identify potential predictors for recalcitrant disease. We conducted a retrospective study of newly diagnosed BP patients who were hospitalized at the Tel Aviv Sourasky Medical Center between the years 2008-2014. We performed statistical analyses to assess the association between clinical factors and failure of initial disease control. Among 114 hospitalized patients with newly diagnosed BP, 1.8% presented with oral mucosa involvement. Seven patients (6.1%) required systemic agents in addition to corticosteroids to achieve disease control. Hypertension (P = 0.048), involvement of the head region (P = 0.042), and metformin treatment (P = 0.02) were significantly more prevalent among patients with recalcitrant disease. The low frequency of recalcitrant BP (6.1%) and the rarity of involvement of the oral mucosa (1.8%) in this study suggest that Israeli BP patients present a milder phenotype compared to similar patients from other geographic areas. Hypertension, head involvement, and metformin therapy were found to be significantly associated with the need for adjuvant therapy to achieve initial control, suggesting that these parameters may serve as predictors of treatment response in BP. © 2017 The International Society of Dermatology.

Bullous reactions to bed bug bites reflect cutaneous vasculitis

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

AUTOIMMUNE EPIDERMAL BLISTERING DISEASES

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Ana Maria Abreu Velez

2013-11-01

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

Successful Treatment of a Bullous Pemphigoid Patient with Rituximab Who Was Refractory to Corticosteroid and Omalizumab Treatments

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Aslı Bilgiç Temel

2017-02-01

Full Text Available Omalizumab is a humanized monoclonal antibody which is an FDA-approved treatment of severe allergic asthma and inhibits IgE binding to FcεRI. According to increasing evidence of IgE inhibition, omalizumab was suggested as a therapeutic approach for bullous pemphigoid (BP. Rituximab has been reported to be effective in various autoimmune diseases, including autoimmune bullous dermatoses. A specific protocol for the use of rituximab to treat BP patients is not yet available. There are only small case series and case reports about the efficacy and safety of rituximab in BP. Here we present a young BP patient who responded well to rituximab therapy and was refractory to conventional and omalizumab therapies although he had elevated IgE levels and eosinophilia. Our case supports the knowledge about the effectiveness and safety of rituximab not only in pemphigus but also in BP. On the other hand, although it did not work in our case, omalizumab may be a potentially effective agent in some carefully selected patients with certain subtypes of BP.

Staphylococcal bullous impetigo in a neonate.

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

2016-07-16

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

Case Report of Bullous Pemphigoid following Fundus Fluorescein Angiography

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Goktug Demirci

2010-05-01

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

Linear IgA dermatosis associated with ulcerative colitis: complete and sustained remission after total colectomy.

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Vargas, Thiago Jeunon de Sousa; Fialho, Mônica; Santos, Luiza Tavares dos; Rodrigues, Palmira Assis de Jesus Barreto; Vargas, Ana Luisa Bittencourt Sampaio Jeunon; Sousa, Maria Auxiliadora Jeunon

2013-01-01

Linear IgA dermatosis has been increasingly associated with inflammatory bowel diseases, particularly ulcerative colitis. A 13-year-old male patient with an 11-month history of ulcerative colitis developed vesicles, pustules and erosions on the skin of the face, trunk and buttocks and in the oral mucosa. The work-up revealed a neutrophil-rich sub-epidermal bullous disease and linear deposition of IgA along the dermoepidermal junction, establishing the diagnosis of linear IgA dermatosis. The patient experienced unsatisfactory partial control of skin and intestinal symptoms despite the use of adalimumab, mesalazine, prednisone and dapsone for some months. After total colectomy, he presented complete remission of skin lesions, with no need of medications during two years of follow-up. A review of previously reported cases of the association is provided here and the role of ulcerative colitis in triggering linear IgA dermatosis is discussed.

Nursing diagnoses in patients with immune-bullous dermatosis

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Euzeli da Silva Brandão

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

Whole body application of a potent topical corticosteroid for bullous pemphigoid

NARCIS (Netherlands)

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

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

Bullous impetigo associated with Abiotrophia defectiva in an immunocompetent adult.

Science.gov (United States)

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

2012-07-01

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

Mediators of Mast Cells in Bullous Pemphigoid and Dermatitis Herpetiformis

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Agnieszka Zebrowska

2014-01-01

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

A case of bullous pemphigoid ınduced by vildagliptin.

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Keseroglu, Havva Ozge; Taş-Aygar, Gamze; Gönül, Müzeyyen; Gököz, Ozay; Ersoy-Evans, Sibel

2017-06-01

Bullous pemfigoid (BP), an autoimmune disorder, can also be induced by some medications. Vildagliptin is a new drug used to treat diabetes mellitus (DM). Recently, a few cases of vildagliptin-induced BP have been described in the literature. We report a patient with BP in which vildagliptin was thought to be as a possible causative agent. The awareness of BP development risk during gliptin therapy can prevent unnecessary usage of systemic drugs with serious side effects.

Subepidermal moisture (SEM) and bioimpedance: a literature review of a novel method for early detection of pressure-induced tissue damage (pressure ulcers).

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Moore, Zena; Patton, Declan; Rhodes, Shannon L; O'Connor, Tom

2017-04-01

Current detection of pressure ulcers relies on visual and tactile changes at the skin surface, but physiological changes below the skin precede surface changes and have a significant impact on tissue health. Inflammatory and apoptotic/necrotic changes in the epidermal and dermal layers of the skin, such as changes in interstitial fluid (also known as subepidermal moisture (SEM)), may precede surface changes by 3-10 days. Those same epidermal and subepidermal changes result in changes in the electrical properties (bioimpedance) of the tissue, thereby presenting an objective, non-invasive method for assessing tissue damage. Clinical studies of bioimpedance for the detection of pressure ulcers have demonstrated that changes in bioimpedance correlate with increasing severity of pressure ulcer stages. Studies have also demonstrated that at anatomical locations with pressure ulcers, bioimpedance varies with distance from the centre of the pressure ulcers. The SEM Scanner, a handheld medical device, offers an objective and reliable method for the assessment of local bioimpedance, and therefore, assessment of tissue damage before signs become visible to the unaided eye. This literature review summarises pressure ulcer pathophysiology, principles of bioimpedance and clinical research using bioimpedance technology to assess pressure ulcers. © 2016 The Authors. International Wound Journal published by Medicalhelplines.com Inc and John Wiley & Sons Ltd.

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

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Kasetsuwan N

2015-09-01

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

Airway management in a patient with bullous pemphigoid

International Nuclear Information System (INIS)

Yasir, M.; Khan, F.A.

2013-01-01

Airway management in patients with pemphigoid lesions has anaesthetic implications. We report a case of a 23 years old female with bullous pemphigoid who presented with laryngeal stenosis and critical airway narrowing. The airway was initially managed with jet ventilation. Anaesthesia was maintained with propofol infusion and ventilation was performed by introducing a size 10 French gauge suction catheter through the stenotic laryngeal orifice. Thirty minutes into anaesthesia, she developed subcutaneous emphysema and decreased air entry on right side of the chest but remained hemodynamically stable. The airway was further managed by tracheostomy. This case report highlights complications that can occur during the anaesthetic management of such cases. (author)

Cutaneous Mucormycosis Following a Bullous Pemphigoid Flare in a Chronic Lymphocytic Leukemia Patient on Ibrutinib.

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Stein, Matthew K; Karri, Saradasri; Reynolds, Jackson; Owsley, Jeff; Wise, Austin; Martin, Mike G; Zare, Fereshteh

2018-04-01

While the recent development of novel therapeutics in oncology, such as small molecule kinase inhibitors (SMKIs), has enabled our ability to target disease-specific molecular pathways, the prolonged impact of these agents on the immune system and infectious risk remains to be seen. We present a 68-year-old male with refractory chronic lymphocytic leukemia (CLL) on ibrutinib monotherapy for 3 years who developed extensive cutaneous mucormycosis following a severe bullous pemphigoid (BP) flare. He received amphotericin B for 4 weeks and was continued on posaconazole with resolution of his mucormycosis infection. Consistent with a growing evidence of literature identifying opportunistic fungal infections in patients on ibrutinib therapy, providers should be cognizant of medical comorbidities that may predispose to such infections and explore methods of prevention before starting ibrutinib and other SMKIs.

Tissue Destruction in Bullous Pemphigoid Can Be Complement Independent and May Be Mitigated by C5aR2

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Christian M. Karsten

2018-03-01

Full Text Available Bullous pemphigoid (BP, the most frequent autoimmune bullous disorder, is a paradigmatic autoantibody-mediated disease associated with autoantibodies against BP180 (type XVII collagen, Col17. Several animal models have been developed that reflect important clinical and immunological features of human BP. Complement activation has been described as a prerequisite for blister formation, however, the recent finding that skin lesions can be induced by anti-Col17 F(ab′2 fragments indicates complement-independent mechanisms to contribute to blister formation in BP. Here, C5−/− mice injected with anti-Col17 IgG showed a reduction of skin lesions by about 50% associated with significantly less skin-infiltrating neutrophils compared to wild-type mice. Reduction of skin lesions and neutrophil infiltration was seen independently of the employed anti-Col17 IgG dose. Further, C5ar1−/− mice were protected from disease development, whereas the extent of skin lesions was increased in C5ar2−/− animals. Pharmacological inhibition of C5a receptor 1 (C5aR1 by PMX53 led to reduced disease activity when applied in a prophylactic setting. In contrast, PMX-53 treatment had no effect when first skin lesions had already developed. While C5aR1 was critically involved in neutrophil migration in vitro, its role for Col17-anti-Col17 IgG immune complex-mediated release of reactive oxygen species from neutrophils was less pronounced. Our data demonstrate that complement-dependent and -independent mechanisms coexist in anti-Col17-autoantibody-mediated tissue destruction. C5aR1 and C5aR2 seem to play opposing roles in this process with C5aR1 exerting its primary effect in recruiting inflammatory cells to the skin during the early phase of the disease. Further studies are required to fully understand the role of C5aR2 in autoantibody-mediated skin inflammation.

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

OpenAIRE

Yuichiro Endo; Miki Tanioka; Hideaki Tanizaki; Minako Mori; Hiroshi Kawabata; Yoshiki Miyachi

2011-01-01

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

Bullous lesions, sweat gland necrosis and rhabdomyolysis in alcoholic coma

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Neelakandhan Asokan

2014-01-01

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

Linear IgA Diseases

NARCIS (Netherlands)

Horvath, Barbara; Jonkman, Marcel F.; Jonkman, Marcel F

2016-01-01

​This book provides obligatory study material on courses on autoimmune bullous diseases. Residents and researchers in dermatology and adjacent disciplines can use the book as a quick reference guide when dealing with these patients. The book contains protocols and follows international guidelines.​

BP180 dysfunction triggers spontaneous skin inflammation in mice.

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Zhang, Yang; Hwang, Bin-Jin; Liu, Zhen; Li, Ning; Lough, Kendall; Williams, Scott E; Chen, Jinbo; Burette, Susan W; Diaz, Luis A; Su, Maureen A; Xiao, Shengxiang; Liu, Zhi

2018-06-04

BP180, also known as collagen XVII, is a hemidesmosomal component and plays a key role in maintaining skin dermal/epidermal adhesion. Dysfunction of BP180, either through genetic mutations in junctional epidermolysis bullosa (JEB) or autoantibody insult in bullous pemphigoid (BP), leads to subepidermal blistering accompanied by skin inflammation. However, whether BP180 is involved in skin inflammation remains unknown. To address this question, we generated a BP180-dysfunctional mouse strain and found that mice lacking functional BP180 (termed Δ NC16A ) developed spontaneous skin inflammatory disease, characterized by severe itch, defective skin barrier, infiltrating immune cells, elevated serum IgE levels, and increased expression of thymic stromal lymphopoietin (TSLP). Severe itch is independent of adaptive immunity and histamine, but dependent on increased expression of TSLP by keratinocytes. In addition, a high TSLP expression is detected in BP patients. Our data provide direct evidence showing that BP180 regulates skin inflammation independently of adaptive immunity, and BP180 dysfunction leads to a TSLP-mediated itch. The newly developed mouse strain could be a model for elucidation of disease mechanisms and development of novel therapeutic strategies for skin inflammation and BP180-related skin conditions.

A new diagnosis of Williams-Beuren syndrome in a 49-year-old man with severe bullous emphysema.

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Wojcik, Monica H; Carmichael, Nikkola; Bieber, Frederick R; Wiener, Daniel C; Madan, Rachna; Pober, Barbara R; Raby, Benjamin A

2017-08-01

Williams-Beuren syndrome (WBS) is a chromosomal microdeletion syndrome typically presenting with intellectual disability, a unique personality, a characteristic facial appearance, and cardiovascular disease. Several clinical features of WBS are thought to be due to haploinsufficiency of elastin (ELN), as the ELN locus is included within the WBS critical region at 7q11.23. Emphysema, a disease attributed to destruction of pulmonary elastic fibers, has been reported in patients without WBS who have pathogenic variants in ELN but only once (in one patient) in WBS. Here we report a second adult WBS patient with emphysema where the diagnosis of WBS was established subsequent to the discovery of severe bullous emphysema. Haploinsufficiency of ELN likely contributed to this pulmonary manifestation of WBS. This case emphasizes the contribution of rare genetic variation in cases of severe emphysema and provides further evidence that emphysema should be considered in patients with WBS who have respiratory symptoms, as it may be under-recognized in this patient population. © 2017 Wiley Periodicals, Inc.

Extracellular matrix alterations in human corneas with bullous keratopathy

DEFF Research Database (Denmark)

Ljubimov, A V; Burgeson, R E; Butkowski, R J

1996-01-01

PURPOSE. To uncover abnormalities of extracellular matrix (ECM) distribution in human corneas with pseudophakic and aphakic bullous keratopathy (PBK/ABK). METHODS. Indirect immunofluorescence with antibodies to 27 ECM components was used on frozen sections of 14 normal and 20 PBK/ABK corneas...... in some cases, correlated with decreased visual acuity. In normal central corneas, tenascin was never found. Other major ECM abnormalities in PBK/ABK corneas compared to normals included: discontinuous epithelial BM straining for laminin-1 (alpha 1 beta 1 gamma 1), entactin/nidogen and fibronectin......; accumulation of fibronectin and alpha 1-alpha 2 type IV collagen on the endothelial face of the Descemet's membrane; and abnormal deposition of stromal ECM (tenascin, fibronectin, decorin, types I, III, V, VI, VIII, XII, XIV collagen) and BM components (type IV, collagen, perlecan, bamacan, laminin-1, entactin...

Pathophysiology of Autoimmune Bullous Diseases: Nature Versus Nurture.

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Patel, Forum; Wilken, Reason; Patel, Falin B; Sultani, Hawa; Bustos, Itzel; Duong, Christopher; Zone, John J; Raychaudhuri, Siba P; Maverakis, Emanual

2017-01-01

Pemphigus and pemphigoid are the prototypical immunobullous diseases. Although it has been well established that they are caused by deposition of autoreactive antibodies directed against adherence proteins within the skin, the specific genetic and environmental factors leading to development of these diseases continue to be an area of investigation. Herein, we discuss several of the potential environmental triggers that may induce patients to develop immunobullous diseases including medications, viral infections, UV exposure or other radiation injury and dietary factors. In addition, the potential genetic and immunologic mechanisms contributing to the pathogenesis of pemphigus and pemphigoid will be reviewed. The multifactorial nature of these diseases contributes to their complexity and highlights the importance of a detailed personal and family history when caring for these patients.

Skin ulcers related to chronic graft-versus-host disease: clinical findings and associated morbidity.

Science.gov (United States)

Jachiet, M; de Masson, A; Peffault de Latour, R; Rybojad, M; Robin, M; Bourhis, J-H; Xhaard, A; Dhedin, N; Sicre de Fontbrune, F; Suarez, F; Barete, S; Parquet, N; Nguyen, S; Ades, L; Rubio, M-T; Wittnebel, S; Bagot, M; Socié, G; Bouaziz, J-D

2014-07-01

According to the National Institutes of Health classification of chronic graft-versus-host disease (cGVHD), skin ulcers after allogeneic haematopoietic stem-cell transplantation (HSCT) are recorded as having the maximal severity score but published data are scarce. To describe skin ulcers related to cGVHD with an emphasis on clinical findings, associated morbidity, management and evolution. A multicentre retrospective analysis was carried out of patients with a diagnosis of cGVHD skin ulcers. All 25 patients included in the study had sclerotic skin cGVHD and 21 had lichenoid skin lesions associated with the sclerotic skin lesions. Thirteen patients had severe cGVHD without considering the skin, because of the involvement of an extracutaneous organ by cGVHD. The median time from HSCT to the onset of ulcers was 44 months. In addition to scleroderma, initial skin lesions at the site of ulcers were bullous erosive lichen in 21 patients and bullous erosive morphoea in four patients. Fifteen patients had an inaugural oedema. Ulcers were mostly bilateral with a predilection for the lower limbs. They were frequently colonized but few infections occurred. Four patients died during a median follow-up period of 55 months. Chronic graft-versus-host disease skin ulcers occur in patients with sclerodermatous skin cGVHD, are associated with severe cGVHD, often start with bullous lichenoid lesions or bullous morphoea and seem to cause more morbidity than mortality, given the low rate of mortality observed in our series of patients. © 2014 British Association of Dermatologists.

Dry eye and meibomian gland dysfunction in pseudophakic bullous keratopathy.

Science.gov (United States)

Palamar, Melis; Kiyat, Pelin; Yagci, Ayse

2018-02-01

To evaluate dry eye tests and meibography of patients with pseudophakic bullous keratopathy (PBK). Thirty-seven patients with PBK were included. The eyes with PBK were compared with the normal pseudophakic fellow eyes. All patients had undergone a detailed ophthalmic examination including corneal and conjunctival fluorescein staining and Oxford scoring, tear film breakup time, Schirmer 1 test, Ocular Surface Disease Index (OSDI) score assessment, lid margin abnormalities, upper and lower eyelid Meibomian gland evaluation using infrared captures of a biomicroscope. Partial or complete loss of the Meibomian glands (Meibomian dropout) was scored for each eyelid from grade 0 (no loss) through grade 3 (lost area was > 2/3 of the total meibomian gland area). The mean age of the patients was 73.2 ± 8.9 (range, 50-93). Mean tear film breakup time value was statistically lower in PBK eyes (P ≤ 0.001). OSDI, Oxford, lid margin abnormalities, inferior meibography, total meibography score were significantly higher in PBK eyes (P ≤ 0.001). The comparison of Schirmer 1 and superior meibography scores of the groups was insignificant (P = 0.143, P = 0.793, respectively). The Meibomian gland morphology of the PBK eyes demonstrates significant differences when compared with normal fellow eyes and might be related to evaporative dry eye. For this reason, patients with PBK should be monitored for Meibomian gland dysfunction and when needed start prompt treatment in order to prevent further disturbance of the ocular surface.

Perfil do paciente com ceratopatia bolhosa pós-facectomia atendidos em hospital público Profile of patient with aphakic/pseudopfakic bullous keratopaty attended at public hospital

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Marcony Rodrigues de Santhiago

2009-08-01

Full Text Available Objetivo: Identificar e analisar criticamente o perfil dos pacientes com ceratopatia bolhosa atendidos em um hospital municipal. Métodos: Foi realizada análise retrospectiva de 35 olhos de 35 pacientes em acompanhamento no setor de córnea e doenças externas do Hospital da Piedade, que apresentavam ceratopatia bolhosa pós-facectomia. Foram excluídos aqueles onde não se conseguiu determinar características pré e per-operatórias fundamentais em nossa pesquisa. Esta seleção de pacientes foi realizada durante o período de janeiro de 2007 a junho de 2007. Resultados: Como principais resultadosobservou-se que a ceratopatia bolhosa ocorreu em 22.86 % (8 olhos dos casos pós-facectomia extracapsular (FEC e 74.28 % (26 olhos pós- facoemulsificação. Em 25.71 % (9 olhos foi documentado rotura de cápsula posterior (RCP com perda vítrea. Em 94.29 % (33 olhos a lente intraocular se localizava na câmara posterior, e dois pacientes estavam afácicos. A acuidade visual de todos os pacientes era pior que 20/200. O tempo médio de evolução entre a facectomia e o diagnostico de ceratopatia bolhosa foi 11,93 meses (1 - 44 meses. Conclusão: A análise adequada do endotélio corneano é condição essencial para o sucesso da cirurgia de catarata com transparência corneal, e que a ceratopatia bolhosa parece estar mais associada à facoemulsificação. Esta informação deve ser interpretada com cautela, já que esta relação pode vir do maior numero de facoemulsificações realizadas no Hospital onde foi realizado o estudo e muitas destas por cirurgiões em treinamento.Objective: Analyze and identify critically the profile of patients with aphakic/pseudophakic bullous keratopathy attended at a public hospital. Methods: Retrospective analysis of 35 eyes of 35 patients with diagnosis of bullous keratopathy, which has been followed in the department of cornea and external diseases of Hospital da Piedade. The one whose pre and per operative data we

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

Science.gov (United States)

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

2017-04-06

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

Penfigoide de membranas mucosas com estenose esofágica grave Mucous membrane pemphigoid with severe esophageal stricture

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Lívia do Nascimento Barbosa

2011-06-01

Full Text Available O penfigoide de membranas mucosas é entidade nosológica encarada como um fenótipo, que engloba várias dermatoses autoimunes com lesões bolhosas subepidérmicas, ocorrendo predominantemente nas membranas mucosas, com êxito cicatricial. O acometimento esofágico no penfigoide de membranas mucosas é raro e observado em pacientes com lesão disseminada. As alterações mais comuns são múltiplas membranas ou constrições esofagianas. No presente relato, os autores apresentam paciente com PMM sem lesões cutâneas e estenose esofágica grave, que entrou em remissão após uso de imunoglobulina venosaMucous membrane pemphigoid (MMP is a rare nosological entity. MMP consists of a clinical phenotype in which several autoimmune subepidermal bullous diseases are classified. It occurs predominantly in the mucous membranes and usually results in scarring. Esophageal involvement in MMP is rare and is generally seen in patients in whom lesions are widespread. The most common alterations are multiple esophageal membranes or strictures. In the present case, the authors report on a patient with MMP without any skin lesions and with severe esophageal strictures who went into remission following use of intravenous immunoglobulin

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.

Our clinical experience with the use of omalizumab in the treatment of bullous pemphigoid

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Pınar İncel Uysal

2017-12-01

Full Text Available Background and Design: In the era of biological therapies, omalizumab (OMZ, a monoclonal antibody which inhibits IgE, has been postulated to be effective in the treatment of bullous pemphigoid (BP. We report our clinical experience with the use of OMZ in the treatment of BP. Materials and Methods: Retrospective data analyses of eleven patients were performed. Results: Seven patients receiving OMZ treatment demonstrated clinical improvements. Three patients terminated treatment because of intermittent co-morbidities. None of the patients had any significant adverse events. Conclusion: OMZ may be a promising corticosteroid-sparing treatment option for moderate to severe BP patients. Future randomized controlled trials are indicated to evaluate the efficacy of OMZ in the treatment of BP.

The effect of autoimmune blistering diseases on work productivity.

Science.gov (United States)

Wang, E Q; Radjenovic, M; Castrillón, M A; Feng, G H Y; Murrell, D F

2018-05-06

Autoimmune blistering diseases (AIBD) are known to negatively impact upon quality of life (QoL); however, there is a paucity of research on the effect of AIBD on work productivity. AIBD can be quite disfiguring in terms of a patient's appearance due to their blistering nature. To determine the impact of AIBD on work productivity and to determine whether patients are stigmatized at work due to their appearance. Sixty-one patients with AIBD completed the Work Productivity and Activity Impairment Questionnaire-Specific Health Problem (WPAIQ-SHP), the Dermatology Life Quality Index (DLQI), the Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life questionnaires (TABQOL). Non-responders to treatment had more work and activity impairment compared to responders. Worse WPAIQ-SHP scores were correlated with higher ABQOL, TABQOL and DLQI scores. Approximately 14.8% of subjects experienced stigmatization at work due to their appearance. The most common body areas stigmatized were easily visible sites, particularly the hands, arms and feet, with the majority of occurrences related to co-workers; for some patients, this stigmatization occurred on a daily basis. Loss of productivity at work was statistically much higher in those with higher disease severity, ABQOL & TABQOL scores and in non-responders to treatment. Autoimmune blistering diseases negatively impacts upon work productivity and activity. Stigmatization was common in the workplace which leads to increased stress, itself a stimulator of pemphigus. © 2018 European Academy of Dermatology and Venereology.

Epidermolysis bullosa acquisita with moderately severe dysphagia due to esophageal strictures

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Jenny Tu

2011-01-01

Full Text Available Epidermolysis bullosa acquisita (EBA is a chronic, autoimmune condition involving the skin and mucous membranes. Symptomatic mucosal involvement is rare, but can impact on quality of life, due to esophageal strictures and dysphagia. We report a case involving a 60-year-old male presenting with bullous skin lesions on areas of friction on his hands, feet and mouth. Milia were visible on some healed areas. Biopsy showed a subepidermal vesicle. Direct immunofluorescence showed intense linear junctional IgG and C3 at the dermo-epidermal junction. Serological tests also supported the diagnosis of EBA. Screening tests for underlying malignancies were negative. Despite treatment with systemic steroids, the patient developed increasing dysphagia, requiring further investigation with esophagoscopy and a barium swallow. Confirmation of extensive esophageal stricturing prompted adjustment of medications including an increase in systemic steroids and addition of azathioprine. Currently, the patient′s disease remains under control, with improvement in all his symptoms and return of anti-basement membrane antibody levels to normal, whilst he remains on azathioprine 150 mg daily and prednisolone 5 mg daily. This case highlights the fact that the treatment of a given patient with EBA depends on severity of disease and co-morbid symptoms. Newer immunoglobulin and biological therapies have shown promise in treatment resistant disease. Considering that long-term immunosuppressants or biologicals will be required, potential side effects of the drugs should be considered. If further deterioration occurs in this patient, cyclosporin A or intravenous immunoglobulin (IV Ig will be considered. Vigilance for associated co-morbidities, especially malignancies, should always be maintained.

Unusual presentation of childhood Systemic Lupus Erythematosus

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Kumar, Sathish; Agarwal, Indira

2007-01-01

Bullous systemic lupus erythematosus is a rare blistering condition with a distinctive combination of clinical, histological and immunopathologic features that together constitute a unique bullous disease phenotype. It is often associated with autoimmunity to type VII collagen. Here we report a child who presented with bullous systemic lupus erythematosus. Rapid resolution of the blisters occurred following treatment with dapsone. PMID:18028550

Sensitivity of direct immunofluorescence in oral diseases. Study of 125 cases.

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Sano, Susana Mariela; Quarracino, María Cecilia; Aguas, Silvia Cristina; González, Ernestina Jesús; Harada, Laura; Krupitzki, Hugo; Mordoh, Ana

2008-05-01

Direct immunofluorescence (DIF) is widely used for the diagnosis of bullous diseases and other autoimmune pathologies such as oral lichen planus. There is no evidence in the literature on how the following variants influence the detection rate of DIF: intraoral site chosen for the biopsy, perilesional locus or distant site from the clinical lesion, number of biopsies and instrument used. to determine if the following variants influenced the sensitivity (detection rate): intraoral site chosen for the biopsy, perilesional or distant site from the clinical lesion, number of biopsies and instrument used (punch or scalpel). A retrospective study was done at the Cátedra de Patología y Clínica Bucodental II at the Facultad de Odontología, Universidad de Buenos Aires; 136 clinical medical histories were revised for the period March 2000 - March 2005 corresponding to patients with clinical diagnosis of OLP and bullous diseases (vulgar pemphigus, bullous pemphigoid and cicatricial pemphigoid). DIF detection rate was 65.8% in patients with OLP, 66.7% in cicatricial pemphigoid patients, in bullous pemphigoid 55.6%, in pemphigus vulgaris 100%, and in those cases in which certain diagnosis could not be obtained, the DIF positivity rate was 45.5% (Pearson chi(2) (4)= 21.5398 Pr= 0.000). There was no statistically significant difference between the different sites of biopsy (Fisher exact test: 0.825). DIF detection rate in perilesional biopsies was 66.1% and in those distant from the site of clinical lesion was 64.7% (Pearson chi(2) v1)= 0.0073 Pr= 0.932. When the number of biopsies were incremented, DIF detection rate also incremented (Pearson chi(2) = 8.7247 Pr= 0.003). The biopsies taken with punch had a higher detection rate than those taken with scalpel (39.1% versus 71.7%) (Pearson chi(2) = 49.0522 Pr= 0.000). While not statistically significant, the tendency outlined in this study indicates there are intraoral regions in which the detection rate of the DIF technique is

Eosinophils in Autoimmune Diseases

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Daniela Čiháková

2017-04-01

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

Eosinophils in Autoimmune Diseases

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Diny, Nicola L.; Rose, Noel R.; Čiháková, Daniela

2017-01-01

Eosinophils are multifunctional granulocytes that contribute to initiation and modulation of inflammation. Their role in asthma and parasitic infections has long been recognized. Growing evidence now reveals a role for eosinophils in autoimmune diseases. In this review, we summarize the function of eosinophils in inflammatory bowel diseases, neuromyelitis optica, bullous pemphigoid, autoimmune myocarditis, primary biliary cirrhosis, eosinophilic granulomatosis with polyangiitis, and other autoimmune diseases. Clinical studies, eosinophil-targeted therapies, and experimental models have contributed to our understanding of the regulation and function of eosinophils in these diseases. By examining the role of eosinophils in autoimmune diseases of different organs, we can identify common pathogenic mechanisms. These include degranulation of cytotoxic granule proteins, induction of antibody-dependent cell-mediated cytotoxicity, release of proteases degrading extracellular matrix, immune modulation through cytokines, antigen presentation, and prothrombotic functions. The association of eosinophilic diseases with autoimmune diseases is also examined, showing a possible increase in autoimmune diseases in patients with eosinophilic esophagitis, hypereosinophilic syndrome, and non-allergic asthma. Finally, we summarize key future research needs. PMID:28496445

Elevated serum BAFF levels in patients with localized scleroderma in contrast to other organ-specific autoimmune diseases.

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Matsushita, Takashi; Hasegawa, Minoru; Matsushita, Yukiyo; Echigo, Takeshi; Wayaku, Takamasa; Horikawa, Mayuka; Ogawa, Fumihide; Takehara, Kazuhiko; Sato, Shinichi

2007-02-01

Serum levels of B-cell activating factor belonging to the tumor necrosis factor family (BAFF), a potent B-cell survival factor, are elevated in patients with systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis and systemic sclerosis (SSc). The objective of this study was to determine serum BAFF levels and relate the results to the clinical features in patients with organ-specific autoimmune diseases of the skin, such as localized scleroderma and autoimmune bullous diseases. Serum BAFF levels were examined by enzyme-linked immunosorbent assay in 44 patients with localized scleroderma, 20 with pemphigus vulgaris/pemphigus foliaceus, 20 with bullous pemphigoid and 30 healthy controls. Twenty patients with SSc and 20 with SLE were also examined as disease controls. Serum BAFF levels were elevated in localized scleroderma patients compared with healthy controls. Concerning localized scleroderma subgroups, patients with generalized morphea, the severest form of localized scleroderma, had higher serum BAFF levels than linear scleroderma or morphea patients. The BAFF levels of generalized morphea were comparable with those of SSc or SLE. Furthermore, serum BAFF levels correlated positively with antihistone antibody levels and the severity of skin lesion as well as the number of skin lesions. By contrast, serum BAFF levels were not significantly elevated in patients with pemphigus or pemphigoid. These results suggest that BAFF may be contributing to autoimmunity and disease development in localized scleroderma.

Nontargeted Effect after Radiotherapy in a Patient with Non-Small Cell Lung Cancer and Bullous Pemphigoid

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Carsten Nieder

2015-01-01

Full Text Available Purpose. To describe tumor shrinkage of nonirradiated lung metastases in a patient with non-small cell lung cancer. Case Report. The patient had a concurrent autoimmune condition, bullous pemphigoid, which was clinically exacerbated during radiotherapy of mediastinal and axillary lymph node metastases. He also developed a series of infections during and after irradiation, and we hypothesize that the immunological events during this phase might have influenced the size of the nonirradiated metastases. Conclusion. Ionizing radiation generates inflammatory signals and, in principle, could provide both tumor-specific antigens from dying cells and maturation stimuli that are necessary for dendritic cells’ activation of tumor-specific T cells. Even if the detailed mechanisms causing nontargeted immune modulatory effects in individual patients are poorly understood, clinical development of radioimmunotherapy is underway.

Intensity-Modulated Radiotherapy Triggers Onset of Bullous Pemphigoid in a Patient with Advanced Melanoma Treated with Nivolumab

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Kayo Tanita

2018-02-01

Full Text Available Since the efficacy of ipilimumab on nivolumab-resistant advanced melanoma is extremely low, additional supportive therapy for anti-PD-1 antibody therapy-resistant advanced melanoma is needed. Although several supportive therapies that enhance the antitumor immune response of anti-PD-1 antibodies have already been reported, unexpected immune-related adverse events were detected at the same time. In this report, we describe a patient with advanced melanoma treated with nivolumab followed by intensity-modulated radiotherapy, which might have triggered bullous pemphigoid (BP. Although several cases of BP developing in anti-PD-1 antibody-treated patients have already been reported, in this report, we shed light on the possible pathogenesis of BP developing in a patient treated with nivolumab through M2 macrophages.

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

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

2015-01-01

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

Electrocauterización de la membrana de Bowman para el tratamiento de la queratopatía bullosa dolorosa Electrocauterization of Bowman's membrane for the treatment of painful bullous keratopathy

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Elizabeth Escalona Leyva

2002-12-01

Full Text Available El propósito en este trabajo es evaluar la electrocauterización de la membrana de Bowman como alternativa quirúrgica en el tratamiento de la queratopatía bullosa dolorosa. Se estudiaron 30 ojos de 30 pacientes con diagnóstico de queratopatía bullosa dolorosa que no habían logrado mejoría con los tratamientos habituales. Se tomaron como variables fundamentales la edad, el sexo, la etiología, el tiempo de evolución, la epitelización, las complicaciones y el alivio sintomático, los datos se tabularon de forma manual teniendo en cuenta el universo de pacientes. Predominó la queratopatía bullosa posquirúrgica (afáquicas y pseudofáquicas en el 83,3 % de los pacientes. Las edades más afectadas fueron los mayores de 60 años (63,3 %; no se encontraron diferencias significativas en cuanto al sexo. El tiempo de epitelización fue de 7 a 14 días en el 93,3 % de los pacientes y solo se encontraron complicaciones en el 14 % que no fueron de gravedad. El alivio de los síntomas se produjo en el 66,6 % en la primera semana del posoperatorio. La electrocauterización de la membrana de Bowman constituye un método eficaz, sencillo y con resultados satisfactorios en el tratamiento de la queratopatía bullosa dolorosaThe purpose of this paper is to evaluate the electrocauterization of Bowman's membrane as a surgical alternative in the treatment of painful bullous keratopathy. 30 eyes from 30 patients who were diagnosed painful bullous keratopathy and had not improved with the habitual treatments were studied. Age, sex, ethiology, time of evolution, epithelialization, complications and the symptomatic relief were taken as fundamental variables. Data were manually tabulated, taking into account the number of patients. The psotsurgical bullous keratopathy (aphakic y pseudophakic prevailed in 83.3 % of the patients. The most affected were those over 60 (63.3 %. No significant differences were found in relation to sex. The time of epithelialization

Calculation of cut-off values based on the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Pemphigus Disease Area Index (PDAI) pemphigus scoring systems for defining moderate, significant and extensive types of pemphigus.

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Boulard, C; Duvert Lehembre, S; Picard-Dahan, C; Kern, J S; Zambruno, G; Feliciani, C; Marinovic, B; Vabres, P; Borradori, L; Prost-Squarcioni, C; Labeille, B; Richard, M A; Ingen-Housz-Oro, S; Houivet, E; Werth, V P; Murrell, D F; Hertl, M; Benichou, J; Joly, P

2016-07-01

Two pemphigus severity scores, Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Pemphigus Disease Area Index (PDAI), have been proposed to provide an objective measure of disease activity. However, the use of these scores in clinical practice is limited by the absence of cut-off values that allow differentiation between moderate, significant and extensive types of pemphigus. To calculate cut-off values defining moderate, significant and extensive pemphigus based on the ABSIS and PDAI scores. In 31 dermatology departments in six countries, consecutive patients with newly diagnosed pemphigus were assessed for pemphigus severity, using ABSIS, PDAI, Physician's Global Assessment (PGA) and Dermatology Life Quality Index (DLQI) scores. Cut-off values defining moderate, significant and extensive subgroups were calculated based on the 25th and 75th percentiles of the ABSIS and PDAI scores. The median ABSIS, PDAI, PGA and DLQI scores of the three severity subgroups were compared in order to validate these subgroups. Ninety-six patients with pemphigus vulgaris (n = 77) or pemphigus foliaceus (n = 19) were included. The median PDAI activity and ABSIS total scores were 27·5 (range 3-84) and 34·8 points (range 0·5-90·5), respectively. The respective cut-off values corresponding to the first and third quartiles of the scores were 15 and 45 for the PDAI, and 17 and 53 for ABSIS. The moderate, significant and extensive subgroups were thus defined, and had distinguishing median ABSIS (P cut-off values of 15 and 45 for PDAI and 17 and 53 for ABSIS, to distinguish moderate, significant and extensive pemphigus forms. Identifying these pemphigus activity subgroups should help physicians to classify and manage patients with pemphigus. © 2016 British Association of Dermatologists.

Homozygous ALOXE3 Nonsense Variant Identified in a Patient with Non-Bullous Congenital Ichthyosiform Erythroderma Complicated by Superimposed Bullous Majocchi’s Granuloma: The Consequences of Skin Barrier Dysfunction

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Tao Wang

2015-09-01

Full Text Available Non-bullous congenital ichthyosiform erythroderma (NBCIE is a hereditary disorder of keratinization caused by pathogenic variants in genes encoding enzymes important to lipid processing and terminal keratinocyte differentiation. Impaired function of these enzymes can cause pathologic epidermal scaling, significantly reduced skin barrier function. In this study, we have performed a focused, genetic analysis of a probrand affected by NBCIE and extended this to his consanguineous parents. Targeted capture and next-generation sequencing was performed on NBCIE associated genes in the proband and his unaffected consanguineous parents. We identified a homozygous nonsense variant c.814C>T (p.Arg272* in ALOXE3 (NM_001165960.1 in the proband and discovered that his parents are both heterozygous carriers of the variant. The clinical manifestations of the proband’s skin were consistent with NBCIE, and detailed histopathological assessment revealed epidermal bulla formation and Majocchi’s granuloma. Infection with Trichophyton rubrum was confirmed by culture. The patient responded to oral terbinafine antifungal treatment. Decreased skin barrier function, such as that caused by hereditary disorders of keratinization, can increase the risk of severe cutaneous fungal infections and the formation of Majocchi’s granuloma and associated alopecia. Patients with NBCIE should be alerted to the possible predisposition for developing dermatophytoses and warrant close clinical follow-up.

Chronic obstructive pulmonary disease

International Nuclear Information System (INIS)

Karabulut, N.

2012-01-01

Full text: Chronic obstructive pulmonary diseases (COPD) denote progressive lung diseases characterized by airway obstruction. COPD exhibits specific morphologic changes in the lung parenchyma, central and peripheral airways and pulmonary vasculature. A person with COPD may have either emphysema or chronic bronchitis, but most have both. Some people with COPD may also have an asthma-like or reactive component. Imaging modalities play important role in the detection or exclusion of COPD, distribution and extent of disease processes. Combined inspiratory and expiratory high resolution CT allows phenotyping of COPD (emphysema predominant, airway predominant, or mixed) and quantification of severity. Magnetic resonance imaging enables functional evaluation and demonstrates ventilation defects correlating closely with pulmonary function tests. Imaging techniques are also helpful in guiding the treatment, such as bullectomy in patients with bullous emphysema, lung volume reduction surgery or endoscopic interventions in those with severe emphysema, and smoking cessation and medical treatment designed to stop lung destruction in patients with mild or moderate emphysema or bronchiectasis.

Churg-Strauss syndrome with coexistence of eosinophilic vasculitis, granulomatous phlebitis and granulomatous dermatitis in bullous pemphigoid-like blisters.

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Ishibashi, Masafumi; Kudo, Saori; Yamamoto, Kyoko; Shimai, Nobuko; Chen, Ko-Ron

2011-03-01

The main histopathological features in the cutaneous lesions of Churg-Strauss syndrome (CSS) are dermal leukocytoclastic vasculitis with a variable eosinophilic infiltrate and non-vasculitic tissue eosinophilia with granuloma formation. This wide histopathological spectrum may account for the various skin manifestations of CSS. However, the unique histopathological combination of dermal eosinophilic vasculitis and subcutaneous granulomatous phlebitis accompanied by bulla formation has not been previously described. We report an unusual CSS case showing dermal necrotizing eosinophilic vasculitis and granulomatous phlebitis in purpuric lesions coupled with subepidermal blistering. The blisters showed dermal granulomatous dermatitis and eosinophilia without evidence of vasculitis. Dermal necrotizing eosinophilic vasculitis was characterized by fibrinoid alteration of the vessel wall, a prominent perivascular eosinophilic infiltrate, a few infiltrating histiocytes along the affected vessel wall, and the absence of neutrophilic infiltration. The underlying subcutaneous granulomatous phlebitis was characterized by an angiocentric histiocytic infiltrate surrounded by marked eosinophilic infiltrate. Deposition of cytotoxic proteins and radicals derived from eosinophils in the vessel walls and papillary dermis followed by a secondary granulomatous response may account for the unique clinical and histopathological features in this case. Copyright © 2010 John Wiley & Sons A/S.

Nursing diagnoses in patients with immune-bullous dermatosis.

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

2016-08-15

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

Complement Activation in Inflammatory Skin Diseases

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Jenny Giang

2018-04-01

Full Text Available The complement system is a fundamental part of the innate immune system, playing a crucial role in host defense against various pathogens, such as bacteria, viruses, and fungi. Activation of complement results in production of several molecules mediating chemotaxis, opsonization, and mast cell degranulation, which can contribute to the elimination of pathogenic organisms and inflammation. Furthermore, the complement system also has regulating properties in inflammatory and immune responses. Complement activity in diseases is rather complex and may involve both aberrant expression of complement and genetic deficiencies of complement components or regulators. The skin represents an active immune organ with complex interactions between cellular components and various mediators. Complement involvement has been associated with several skin diseases, such as psoriasis, lupus erythematosus, cutaneous vasculitis, urticaria, and bullous dermatoses. Several triggers including auto-antibodies and micro-organisms can activate complement, while on the other hand complement deficiencies can contribute to impaired immune complex clearance, leading to disease. This review provides an overview of the role of complement in inflammatory skin diseases and discusses complement factors as potential new targets for therapeutic intervention.

A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases.

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Lee, Brendon W H; Tan, Jeremy C K; Radjenovic, Melissa; Coroneo, Minas T; Murrell, Dedee F

2018-05-22

Epidermolysis bullosa (EB) and autoimmune blistering diseases (AIBD) describe a group of rare chronic dermatoses characterized by cutaneous fragility and blistering. Although uncommon, significant ocular surface disease (OSD) may occur in both and require ophthalmological assessment. Disease scoring systems have a critical role in providing objective and accurate assessment of disease severity. The objectives of this report were, firstly, to document the prevalence and severity of ocular involvement in EB/AIBD. Secondly, to review and evaluate existing ocular and systemic scoring systems for EB/AIBD. Finally, to identify areas where further development of ocular specific tools in EB/AIBD could be pursued. A literature search was performed in October 2017 utilising Medline, Embase, and Scopus databases. The results were restricted by date of publication, between 01.01.1950 and 31.10.2017. The reference lists of these articles were then reviewed for additional relevant publications. Articles of all languages were included if an English translation was available. Articles were excluded if they were duplicates, had no reference to ocular involvement in EB/AIBD or described ocular involvement in other diseases. Descriptions of ocular involvement in EB/AIBD were identified in 88 peer-reviewed journal articles. Findings reported include but are not limited to: cicatrising conjunctivitis, meibomian gland dysfunction, dry eye disease, trichiasis, symblepharon, fornix fibrosis, keratopathy, ectropion/entropion, ankyloblepharon, corneal ulceration, visual impairment and blindness. Although scoring systems exist for assessment of OSD in mucous membrane pemphigoid, no such tools exist for the other AIBD subtypes or for EB. Several systemic scoring systems exist in the dermatological literature that are efficacious in grading overall EB/AIBD severity, but have limited inclusion of ocular features. To the best of our knowledge, there is no recognised or validated scoring systems

A case of linear IgA bullous dermatosis with IgA anti-type VII collagen autoantibodies.

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Hashimoto, T; Ishiko, A; Shimizu, H; Tanaka, T; Dodd, H J; Bhogal, B S; Black, M M; Nishikawa, T

1996-02-01

In this study we present a patient with the sublamina densa type of linear IgA bullous dermatosis (LABD), with IgA autoantibodies reactive with the 290-kDa type VII collagen (the epidermolysis bullosa acquisita (EBA) antigen) and with immunoblotting of normal human dermal extracts. The clinical and histological features of the present case were compatible with those of LABD but quite different from those of EBA. Although EBA sera reacted with the bacterial fusion protein of the N-terminal globular (NC1) domain of type VII collagen, this patient's serum did not show reactivity. Furthermore, ultrastructural localization of target epitopes on the anchoring fibrils in this patient was considerably different from EBA. These results indicate that, whereas EBA antibodies react with the NC1 domain of type VII collagen, the epitope in this case is different from that of EBA (and is most likely on the central triple helical domain). This difference may be responsible for the clinical presentation in this patient being distinct from that of EBA.

OCT imaging of skin cancer and other dermatological diseases

DEFF Research Database (Denmark)

Mogensen, Mette; Thrane, Lars; Jørgensen, Thomas Martini

2009-01-01

Optical coherence tomography (OCT) provides clinicians and researchers with micrometer-resolution, in vivo, cross-sectional images of human skin up to several millimeter depth. This review of OCT imaging applied within dermatology covers the application of OCT to normal skin, and reports on a lar...... number of applications in the fields of non-melanoma skin cancer, malignant melanomas, psoriasis and dermatitis, infestations, bullous skin diseases, tattoos, nails, haemangiomas, and other skin diseases. (© 2009 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim)......Optical coherence tomography (OCT) provides clinicians and researchers with micrometer-resolution, in vivo, cross-sectional images of human skin up to several millimeter depth. This review of OCT imaging applied within dermatology covers the application of OCT to normal skin, and reports on a large...

Disease: H00691 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available H00691 Bullous congenital ichthyosiform erythroderma (BCIE); Epidermolytic hyperkeratosis...LE ... Histopathologic characterization of epidermolytic hyperkeratosis: a systematic review of histology fr...AUTHORS ... Lacz NL, Schwartz RA, Kihiczak G ... TITLE ... Epidermolytic hyperkeratosis: a keratin 1 or 10 mutat

Epidermolysis bullosa acquisita: clinical manifestations, microscopic findings, and surgical periodontal therapy. A case report.

Science.gov (United States)

Hakki, S S; Celenligil-Nazliel, H; Karaduman, A; Usubütün, A; Ertoy, D; Ayhan, A; Ruacan, S

2001-04-01

Epidermolysis bullosa acquisita (EBA) is an uncommon, acquired, chronic subepidermal bullous disease. This report describes a case of EBA with gingival involvement. A 43-year-old woman with EBA was referred to our clinic for periodontal therapy because of gingival tenderness and bleeding. She has been on cyclosporin A therapy for the last 2 years. Clinical findings were analyzed. Anterior gingivectomy operations were performed in 2 stages. The samples obtained during the surgery were examined using histopathologic, immunohistologic, and electronmicroscopic methods. Long-term effects of the surgical periodontal treatment on gingiva were evaluated both clinically and microscopically. The dentition displayed minimal enamel hypoplasia. Decayed, missing, and filled surfaces score was found to be elevated. Periodontal examination showed generalized diffuse gingival inflammation and gingival enlargement localized mainly to the anterior region. Nikolsky's sign was positive. However, wound healing was uneventful after the operations. Microscopic findings were similar to those obtained from the skin. Twenty-one months after the operations, Nikolsky's sign was negative and no remarkable gingival inflammation was noted. Microscopic examination revealed that the blisters were fewer in number and smaller in size. These results indicate that gingival tissues may also be involved in EBA. Uneventful wound healing after periodontal surgery in this case suggests that periodontal surgery can be performed in patients with EBA. Moreover, both our clinical and histopathologic findings imply that gingivectomy proves useful in maintaining gingival integrity in these patients. Our data may also suggest that the patients with EBA are highly likely to develop dental caries.

Accuracy of molecular diagnostics in pemphigus and bullous pemphigoid: comparison of commercial and modified mosaic indirect immunofluorescence tests as well as enzyme-linked immunosorbent assays.

Science.gov (United States)

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

2017-02-01

Pemphigus and bullous pemphigoid (BP) are identified by autoantibodies (abs) against desmoglein 1, 3 (DSG1/3) and BP180/BP230, respectively. A novel mosaic to indirect immunofluorescence (IIF) using purified BP180 recombinant proteins spotted on slide and transfected cells expressing BP230, DSG1, DSG3 is available. The commercial (IgG detection) and modified (IgG4 detection) mosaic for indirect immunofluorescence (IIFc - IIF commercial, IIFm - IIF modified) and IgG ELISAs were evaluated in pemphigus and bullous pemphigoid (BP) molecular diagnostics. To compare diagnostic accuracy of commercial (IgG detection) and modified (IgG4 detection) mosaic IIF assay and to examine the diagnostic value of ELISAs in relation to mosaic IIF in routine laboratory diagnostics of pemphigus and BP. Sera from 37 BP and 19 pemphigus patients were studied. Associations between tests were assessed using Fisher's exact test. There are associations between the positive/negative samples detected by IIFc with desmoglein1 (DSG1)/desmoglein3 (DSG3)/BP230 transfected cells and ELISAs and no association between anti-BP180 IgG detection by IIFc and ELISA. IIFm with DSG1 and DSG3 showed both 100% sensitivity and 100% and 78% specificity, respectively, and 100% and 83% positive predictive value in relation to IIFc. IIFm with BP230 had 87% specificity, 55% sensitivity, whereas IIFm with BP180 had a 100% sensitivity and 13% specificity in relation to IIFc. The IIFc with DSG1/DSG3/BP230 transfected cells, excluding BP180 spots, is an alternative method to ELISA in pemphigus/BP diagnostics. IgG4 antibodies, both pathogenically and diagnostically important, are inconsistently detectable with IIFm.

Human corneal endothelial cell transplantation using nanocomposite gel sheet in bullous keratopathy.

Science.gov (United States)

Parikumar, Periasamy; Haraguchi, Kazutoshi; Senthilkumar, Rajappa; Abraham, Samuel Jk

2018-01-01

Transplantation of in vitro expanded human corneal endothelial precursors (HCEP) cells using a nanocomposite (D25-NC) gel sheet as supporting material in bovine's cornea has been earlier reported. Herein we report the transplantation of HCEP cells derived from a cadaver donor cornea to three patients using the NC gel sheet. In three patients with bullous keratopathy, one after cataract surgery, one after trauma and another in the corneal graft, earlier performed for congenital corneal dystrophy, not amenable to medical management HCEP cells isolated from a human cadaver donor cornea in vitro expanded using a thermoreversible gelation polymer (TGP) for 26 days were divided into three equal portions and 1.6 × 10 5 HCEP cells were injected on to the endothelium of the affected eye in each patient using the D25-NC gel sheet as a supporting material. The sheets were removed after three days. The bullae in the cornea disappeared by the 3 rd -11 th post-operative day in all the three patients. Visual acuity improved from Perception of light (PL)+/Projection of rays (PR)+ to Hand movements (HM)+ in one of the patients by post-operative day 3 which was maintained at 18 months follow-up. At 18 months follow-up, in another patient the visual acuity had improved from HM+ to 6/60 while in the third patient, visual acuity remained HM+ as it was prior to HCEP transplantation. There were no adverse effects during the follow-up in any of the patients.

Dermatological disease in the older age group: a cross-sectional study in aged care facilities.

Science.gov (United States)

Deo, Maneka S; Kerse, Ngaire; Vandal, Alain C; Jarrett, Paul

2015-12-23

To estimate the prevalence of dermatological disease in aged care facilities, and the relationship between cognitive or physical disability and significant disease. 2 large aged care facilities in Auckland, New Zealand, each providing low and high level care. All 161 residents of the facilities were invited to participate. The only exclusion criterion was inability to obtain consent from the individual or designated guardian. 88 participants were recruited-66 females (75%), 22 males (25%) with average age 87.1 years (SD 5.5 years). Primary--presence of significant skin disease (defined as that which in the opinion of the investigators needed treatment or was identified as a patient concern) diagnosed clinically on full dermatological examination by a dermatologist or dermatology trainee. Secondary--functional and cognitive status (Rehabilitation Complexity Scale and Abbreviated Mental Test Score). 81.8% were found to have at least one significant condition. The most common disorders were onychomycosis 42 (47.7%), basal cell carcinoma 13 (14.8%), asteototic eczema 11 (12.5%) and squamous cell carcinoma in situ 9 (10.2%). Other findings were invasive squamous cell carcinoma 7 (8%), bullous pemphigoid 2 (2.3%), melanoma 2 (2.3%), lichen sclerosus 2 (2.3%) and carcinoma of the breast 1 (1.1%). Inflammatory disease was more common in those with little physical disability compared with those with serious physical disability (OR 3.69; 95% CI 1.1 to 12.6, p=0.04). No significant association was found between skin disease and cognitive impairment. A high rate of dermatological disease was found. Findings ranged from frequent but not life-threatening conditions (eg, onychomycosis), to those associated with a significant morbidity (eg, eczema, lichen sclerosus and bullous pemphigoid), to potentially life-threatening (eg, squamous cell carcinoma, melanoma and breast cancer). Those with less significant physical impairment were found to be at greater risk of inflammatory

Steroid sparing regimens for management of oral immune-mediated diseases

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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.

Accuracy of molecular diagnostics in pemphigus and bullous pemphigoid: comparison of commercial and modified mosaic indirect immunofluorescence tests as well as enzyme-linked immunosorbent assays

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Justyna Gornowicz-Porowska

2017-02-01

Full Text Available Introduction : Pemphigus and bullous pemphigoid (BP are identified by autoantibodies (abs against desmoglein 1, 3 (DSG1/3 and BP180/BP230, respectively. A novel mosaic to indirect immunofluorescence (IIF using purified BP180 recombinant proteins spotted on slide and transfected cells expressing BP230, DSG1, DSG3 is available. The commercial (IgG detection and modified (IgG4 detection mosaic for indirect immunofluorescence (IIFc – IIF commercial, IIFm – IIF modified and IgG ELISAs were evaluated in pemphigus and bullous pemphigoid (BP molecular diagnostics. Aim : To compare diagnostic accuracy of commercial (IgG detection and modified (IgG4 detection mosaic IIF assay and to examine the diagnostic value of ELISAs in relation to mosaic IIF in routine laboratory diagnostics of pemphigus and BP. Material and methods : Sera from 37 BP and 19 pemphigus patients were studied. Associations between tests were assessed using Fisher’s exact test. Results: There are associations between the positive/negative samples detected by IIFc with desmoglein1 (DSG1/desmoglein3 (DSG3/BP230 transfected cells and ELISAs and no association between anti-BP180 IgG detection by IIFc and ELISA. IIFm with DSG1 and DSG3 showed both 100% sensitivity and 100% and 78% specificity, respectively, and 100% and 83% positive predictive value in relation to IIFc. IIFm with BP230 had 87% specificity, 55% sensitivity, whereas IIFm with BP180 had a 100% sensitivity and 13% specificity in relation to IIFc. Conclusions : The IIFc with DSG1/DSG3/BP230 transfected cells, excluding BP180 spots, is an alternative method to ELISA in pemphigus/BP diagnostics. IgG4 antibodies, both pathogenically and diagnostically important, are inconsistently detectable with IIFm.

Vimentin may reflect areas of pathologic involvement in biopsies from patients with autoimmune skin diseases

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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.

Twardzina ograniczona pęcherzowa – przedstawienie dwóch przypadków, trudności terapeutyczne

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Dorota Krasowska

2011-07-01

Full Text Available tissue disease. The main symptoms are atrophy and fibrosis of the connectivetissue stroma, preceded by changes in the small blood vessels.Pathogenesis of morphea is still unknown. The possible role of geneticfactors, immunological abnormalities, especially in cell-mediatedimmunity, trauma, viruses or infection with Borrelia burgdorferi is suspected.Several types of localized scleroderma can be distinguished: plaque morphea, generalized morphea, bullous morphea, linear morphea(scleroderma and deep morphea.Objective. To present two cases of bullous morphea and therapeuticdifficulties connected with this disease.Case report. Two cases of bullous morphea are presented in 70- and 84-year-old women. Each of them was treated with individually chosentherapy. Initially they were treated with crystalline penicillin, hydrocortisone,vascular drugs; and UVA phototherapy. Afterwards, thepatients were treated with pulses of methylprednisolone andmethotrexate. The treatment led to suppression of the disease – blistershealed and the indurations diminished.Conclusions. The treatment of localized scleroderma is not satisfactory.It causes difficulties due to limited effectiveness of applied drugsand their side effects. It is crucial to start treatment as early as possible,when the pathological changes are in the initial stage.

[Personalized cell therapy for early postoperative bullous keratopathy (experimental proof and clinical results)].

Science.gov (United States)

Kasparov, A A; Kasparova, Evg A; Fadeeva, L L; Subbot, A M; Borodina, N V; Kasparova, E A; Kobzova, M V; Musaeva, G M; Pavliuk, A S

2013-01-01

The article presents the results of a long-term research on development and clinical application of personalized cell therapy (PCT) for treatment of early postoperative (manifesting within the first 3 months after surgery) bullous keratopathy (BK). The method of intracameral PCT implies in vitro incubation of the patient's blood sample with poly(A:U) stimulator, separation of the serum with activated leukocytes, and injection of the final cell preparation into the anterior chamber. The fundamental part of the research was aimed at a detailed description of the cell preparation and investigation of its possible mechanisms of action. Cytokine and growth factor level in the cell preparation suggested that its high clinical efficacy might be due to its ability to improve regeneration of damaged corneal endothelium. The clinical study was conducted on a group of 52 patients with early BK. A significant effect (smoothing of the Descement's membrane folds, complete resorption of corneal edema, improvement of corneal transparency, reduction of corneal thickness and increase of visual acuity by 0.49 +/- 0.27) was achieved in 44.2% of patients, while partial effect was seen in 21.1% of patients. There was no clinical effect in 34.6% of patients. In those patients who developed significant or partial clinical effect after the PCT, many endotheliocytes appeared to have multiple nuclei (2 and more). In some patients polyploid nuclei persisted for 3-5 years after the treatment. Polyploidy results from incomplete mitosis which might be due to regenerative processes in the endothelium stimulated by the PCT. Obviously, high efficacy and relative simplicity of the method should promote its further clinical introduction.

Thoracic imaging in AIDS

Energy Technology Data Exchange (ETDEWEB)

Barneveld Binkhuysen, F H [Eemland Hospital, Dept. of Radiology, Amersfoort (Netherlands)

1996-12-31

The contents are infectious diseases i.e. pneumocystis carinii, mycobacterium tuberculosis, atypical mycobacteria, fungal infections, viral infections ; Malignant diseases : kaposi`s sarcoma (KS), malignant lymphoma and other disorders i.e. lymphocytic interstitial pneumonitis, hypersensitivity reactions, bullous changes, pericardial abnormalities (15 refs.).

Thoracic imaging in AIDS

International Nuclear Information System (INIS)

Barneveld Binkhuysen, F.H.

1995-01-01

The contents are infectious diseases i.e. pneumocystis carinii, mycobacterium tuberculosis, atypical mycobacteria, fungal infections, viral infections ; Malignant diseases : kaposi's sarcoma (KS), malignant lymphoma and other disorders i.e. lymphocytic interstitial pneumonitis, hypersensitivity reactions, bullous changes, pericardial abnormalities (15 refs.)

Radiation-induced pemphigus vulgaris of the breast; Pemphigus vulgaire radio-induit du sein

Energy Technology Data Exchange (ETDEWEB)

Vigna-Taglianti, R.; Russi, E.G. [Department of radiotherapy, Santa Croce e Carle general hospital, Via M. Coppino, 12100 Cuneo (Italy); Denaro, N. [Oncology department, university of Messina, Via consolare Valeria no 1, 98100 Messina (Italy); Numico, G. [Department of medical oncology, U. Parini hospital, 11100 Aosta (Italy); Brizio, R. [Department of histopathology, Santa Croce e Carle general hospital, Via M. Coppino, 12100 Cuneo (Italy)

2011-07-15

Pemphigus vulgaris is a rare autoimmune muco-cutaneous bullous disease. Patients with a history of pemphigus vulgaris - who need radiotherapy - may show a long lasting bullous cutaneous manifestation, typical of pemphigus, within radiation fields. The literature describes fewer than 20 radio-induced cases. While systematic corticosteroid therapy has proven to be useful, topical treatment used in association with corticosteroid therapy is rarely described. To our knowledge the use of modern dressing products has never been described. We report our experience in a case in which modern dressing products were usefully associated to systemic therapy. (authors)

The use of typing methods and infection prevention measures to control a bullous impetigo outbreak on a neonatal ward.

Science.gov (United States)

Koningstein, Maike; Groen, Leon; Geraats-Peters, Kathelijn; Lutgens, Suzanne; Rietveld, Ariene; Jira, Petr; Kluytmans, Jan; de Greeff, Sabine C; Hermans, Mirjam; Schneeberger, Peter M

2012-11-20

We describe an outbreak of Bullous Impetigo (BI), caused by a (methicillin susceptible, fusidic acid resistant) Staphylococcus aureus (SA) strain, spa-type t408, at the neonatal and gynaecology ward of the Jeroen Bosch hospital in the Netherlands, from March-November 2011. We performed an outbreak investigation with revision of the hygienic protocols, MSSA colonization surveillance and environmental sampling for MSSA including detailed typing of SA isolates. Spa typing was performed to discriminate between the SA isolates. In addition, Raman-typing was performed on all t408 isolates. Nineteen cases of BI were confirmed by SA positive cultures. A cluster of nine neonates and three health care workers (HCW) with SA t408 was detected. These strains were MecA-, PVL-, Exfoliative Toxin (ET)A-, ETB+, ETAD-, fusidic acid-resistant and methicillin susceptible. Eight out of nine neonates and two out of three HCW t408 strains yielded a similar Raman type. Positive t408 HCW were treated and infection control procedures were reinforced. These measures stopped the outbreak. We conclude that treatment of patients and HCW carrying a predominant SA t408, and re-implementing and emphasising hygienic measures were effective to control the outbreak of SA t408 among neonates.

The use of typing methods and infection prevention measures to control a bullous impetigo outbreak on a neonatal ward

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Koningstein Maike

2012-11-01

Full Text Available Abstract Background We describe an outbreak of Bullous Impetigo (BI, caused by a (methicillin susceptible, fusidic acid resistant Staphylococcus aureus (SA strain, spa-type t408, at the neonatal and gynaecology ward of the Jeroen Bosch hospital in the Netherlands, from March-November 2011. Methods We performed an outbreak investigation with revision of the hygienic protocols, MSSA colonization surveillance and environmental sampling for MSSA including detailed typing of SA isolates. Spa typing was performed to discriminate between the SA isolates. In addition, Raman-typing was performed on all t408 isolates. Results Nineteen cases of BI were confirmed by SA positive cultures. A cluster of nine neonates and three health care workers (HCW with SA t408 was detected. These strains were MecA-, PVL-, Exfoliative Toxin (ETA-, ETB+, ETAD-, fusidic acid-resistant and methicillin susceptible. Eight out of nine neonates and two out of three HCW t408 strains yielded a similar Raman type. Positive t408 HCW were treated and infection control procedures were reinforced. These measures stopped the outbreak. Conclusions We conclude that treatment of patients and HCW carrying a predominant SA t408, and re-implementing and emphasising hygienic measures were effective to control the outbreak of SA t408 among neonates.

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

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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.

Pseudoporfyri er et symptomkompleks

DEFF Research Database (Denmark)

Velander, Marie Juul; Thorsteinsdóttir, Sunna; Bygum, Anette

2015-01-01

Pseudoporphyria is a photosensitive bullous disease, which resembles porphyria cutanea tarda. Normal porphyrin levels in urine, stool and blood define pseudoporphyria. Pseudoporphyria is associated with chronic renal failure, haemodialysis, a variety of drugs (e.g. naproxen, nabumetone, furosemide...

Bullous Impetigo Rapid Diagnostic and Therapeutic Quiz: A Model for Assessing Basic Dermatology Knowledge of Primary Care Providers.

Science.gov (United States)

Simkin, Daren J; Grossberg, Anna L; Cohen, Bernard A

2016-11-01

Bullous impetigo (BI) is a common dermatologic condition, particularly in children, yet confusion regarding its diagnosis and treatment persists. This study measured pediatricians' ability to diagnose and appropriately treat BI and explored factors that might influence pediatricians' accuracy in managing BI. We administered an expert-validated survey to 64 pediatrics house staff and faculty at three Johns Hopkins Medicine facilities. The survey requested demographic information, diagnoses for five "unknown" cases, and preferred treatments for localized and widespread BI. Overall, BI was diagnosed correctly 31.9% of the time. There was little difference between house staff and faculty performance, although faculty 50 years of age and older demonstrated better diagnostic acumen. Regarding treatment of localized BI, 92% of faculty members and 84.6% of house staff listed mupirocin as first- or second-line treatment. The second most common medication listed for localized BI was bacitracin. Regarding treatment of widespread BI, faculty listed cephalexin or clindamycin as first- or second-line treatment 56.0% of the time and house staff listed one of these two medications 51.3% of the time. Results for faculty 50 years of age and older were comparable. Improved pediatrician proficiency in the diagnosis and treatment of BI is needed for safe, cost-effective management. Physician age and experience appear to have a limited effect on the accuracy of BI diagnosis and management. Future educational efforts must be directed at trainees and their instructors. © 2016 Wiley Periodicals, Inc.

Impetigo asacause of sepsis ininfants

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Alicja Moes-Wójtowicz

2015-06-01

Full Text Available Impetigo is a fairly common disease of the childhood. This infection exists in two distinct forms: bullous and non-bullous impetigo. In non-bullous impetigo, a tiny vesicle or pustule shows initially and after 4–6 days develops into a honey-coloured crusted plaque. Bullous impetigo is always caused by Staphylococcus aureus, a bacterium that produces exfoliative toxins (ETA and ETB. Infection is limited to the superficial layers of the skin, and skin changes typically occur on the face and limbs. The disease is most prevalent in children aged 2 to 5, but it can occur at any age. The treatment involves topical or systemic antibiotics depending on the severity of symptoms and the patient’s condition. The differential diagnosis of impetigo includes: staphylococcal scalded-skin syndrome, Herpes simplex infection, eosinophilic pustular folliculitis, epidermolysis bullosa, pemphigus and pemphigoid. The differentiation between impetigo and staphylococcal scalded-skin syndrome can be challenging. Both diseases are caused by the same strain of Staphylococcus aureus that produces exfoliative toxins. However, in impetigo toxins are produced only within skin lesions whereas in staphylococcal scalded-skin syndrome, toxins are produced in organs colonised by Staphylococcus aureus and then released systemically. In the course of staphylococcal scalded-skin syndrome severe tenderness of the skin and a positive Nikolsky’s sign are observed. The paper presents two cases of impetigo in infants. In both cases, the correct diagnosis was not established immediately, which delayed appropriate treatment. In one of the cases, due to the imprecise initial diagnosis and the lack of the holistic approach to the patient, a correct diagnosis was established so late, that a systemic infection developed.

Research

African Journals Online (AJOL)

ebutamanya

2016-04-22

Apr 22, 2016 ... Papulosquamous disorders, Infectious disorders, Connective tissue diseases, Bullous disorders, ... surgical pathology specimen received within the study period. The modal age ... enable appropriate management. ... basic clinical data including age, gender were noted. ... Confidentiality of the identity of the.

HLA class II alleles of susceptibility and protection in Brazilian and Dutch pemphigus foliaceus

NARCIS (Netherlands)

de Sena Nogueira Maehara, L; De-Souza-Santana, F C; Porro, A M; Marcos, E V C; Ura, S; Nolte, I M; Pas, H H; Jonkman, M F; Tomimori, J

Pemphigus foliaceus (PF) is a worldwide chronic autoimmune bullous disease targeting desmoglein 1 (Dsg 1) on epithelial cell surface of skin and mucous membranes, causing lesions in skin but sparing mucosa, since the latter contain compensating desmoglein 3(1) . Several environmental triggers for

Pemphigus. S2 Guideline for diagnosis and treatment - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV)

NARCIS (Netherlands)

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

BACKGROUND: Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, the prognosis of pemphigus was almost fatal. Due to its rarity, only few prospective

Young donor-graft assisted endothelial keratoplasty (PDEK/DMEK) with epithelial debridement for chronic pseudophakic bullous keratopathy.

Science.gov (United States)

Agarwal, Amar; Narang, Priya; Kumar, Dhivya A; Agarwal, Ashvin

2017-10-01

The aim of this study was to describe the applicability and report visual outcomes for the treatment of subepithelial fibrosis and anterior stromal scarring in cases of chronic pseudophakic bullous keratopathy (PBK) with epithelial debridement and endothelial keratoplasty (EK) (pre-Descemet endothelial keratoplasty [PDEK]; Descemet membrane endothelial keratoplasty [(DMEK]) using young donor tissue. Prospective, single-centre, interventional study. 6 cases with chronic PBK (> 1 year duration). Case 1 underwent PDEK with glued intraocular lens (IOL) as a single-stage procedure, whereas cases 2 and 3 underwent glued IOL followed by DMEK and PDEK, respectively, as a second-stage procedure. Cases 4 and 6 underwent PDEK, whereas case 5 underwent DMEK. Epithelial debridement was performed in all cases at the time of EK, and young donor grafts were used. The main outcome measures were best spectacle-corrected visual acuity, clearance of corneal scar and haze, central corneal thickness (CCT), specular microscopy, and endothelial cell count (ECC). Postoperatively, all cases demonstrated significant improvement in visual acuity. The mean value of depth of subepithelial haze was 121±71.7 µm and 25.3 ± 19.8 µm in the preoperative and postoperative periods, respectively (p = 0.028). At the 1-month follow-up, the mean preoperative CCT of 676 ± 92.7 µm was reduced to 534.6 ± 21.1µm. At the 6-month follow-up, the mean ECC loss resulting from the procedure was 36.5 ± 10.4%. EK with epithelial debridement performed for the treatment of chronic PBK resulted in significantly improved visual acuity to a functional level, with the clearance of subepithelial fibrosis and anterior stromal scar, in most patients. Copyright © 2017 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.

Enfisema bolhoso associado a consumo de drogas

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Sandra Figueiredo

2010-01-01

Full Text Available Resumo: O enfisema bolhoso é caracterizado pelo aumento dos espaços aéreos distais ao bronquíolo terminal com perda das paredes alveolares. Várias etiologias têm sido descritas, nomeadamente a deficiência de alfa1-antitripsina e o consumo de tabaco. À excepção da deficiência de alfa1-antitripsina, alguns casos de enfisema bolhoso no jovem continuam sem etiologia conhecida. Têm sido descritos alguns casos associados a consumo de drogas. O tabagismo associado ao consumo de drogas possivelmente criará as condições para o desenvolvimento de bolhas, com particular exuberância pelos lobos superiores. Os autores descrevem dois casos clínicos de doentes jovens, com história de consumo de drogas inaladas e enfisema bolhoso.Rev Port Pneumol 2010; XVI (1: 157-161 Abstract: Bullous emphysema is characterized by permanent enlargement of airspaces distal to the terminal bronchiole accompanied by destruction of alveolar walls. Several aetiologies have been described, namely deficiency of α1-antitrypsin and the consumption of tobacco. With the exception of the deficiency of α1-antitrypsin, some cases of bullous emphysema in the young adult continue without known aetiology. Some cases of bullous emphysema have been described, emphasising the relationship between the disease and drug consumption. The association possibly creates the conditions for the development of large lung bullae, with particular exuberance for the superior lobes. The authors describe two clinical cases of young patients, with history of consumption of smoked substance abuse and bullous emphysema.Rev Port Pneumol 2010; XVI (1: 157-161 Palavras-chave: Enfisema bolhoso, drogas inaladas, Key-words: Bullous emphysema, smoked substance abuse

Enfisema bolhoso associado a consumo de drogas

Directory of Open Access Journals (Sweden)

Sandra Figueiredo

2010-01-01

Full Text Available Resumo: O enfisema bolhoso é caracterizado pelo aumento dos espaços aéreos distais ao bronquíolo terminal com perda das paredes alveolares. Várias etiologias têm sido descritas, nomeadamente a deficiência de alfa1-antitripsina e o consumo de tabaco. À excepção da deficiência de alfa1-antitripsina, alguns casos de enfisema bolhoso no jovem continuam sem etiologia conhecida. Têm sido descritos alguns casos associados a consumo de drogas. O tabagismo associado ao consumo de drogas possivelmente criará as condições para o desenvolvimento de bolhas, com particular exuberância pelos lobos superiores. Os autores descrevem dois casos clínicos de doentes jovens, com história de consumo de drogas inaladas e enfisema bolhoso. Abstract: Bullous emphysema is characterized by permanent enlargement of airspaces distal to the terminal bronchiole accompanied by destruction of alveolar walls. Several aetiologies have been described, namely deficiency of α1-antitrypsin and the consumption of tobacco. With the exception of the deficiency of α1-antitrypsin, some cases of bullous emphysema in the young adult continue without known aetiology. Some cases of bullous emphysema have been described, emphasising the relationship between the disease and drug consumption. The association possibly creates the conditions for the development of large lung bullae, with particular exuberance for the superior lobes. The authors describe two clinical cases of young patients, with history of consumption of smoked subs tance abuse and bullous emphysema. Palavras-chave: Enfisema bolhoso, drogas inaladas, Key-words: Bullous emphysema, smoked substance abuse

Relationship between theoretical molecular weight and blister fluid/serum ratio of cytokines and five other molecules evaluated in patients with bullous pemphigoid.

Science.gov (United States)

D'Auria, L; Pimpinelli, F; Ferraro, C; D'Ambrogio, G; Giacalone, B; Bellocci, M; Ameglio, F

1998-01-01

Bullous pemphigoid (BP) blisters contain several molecules, some of which spread into the blisters from the interstitial fluid, while others are produced locally and migrate into the circulation. The calculation of the ratios between blister/serum concentrations may help to distinguish between these two types of molecules. The rules regulating the diffusion of the molecules have been described only in suction blisters, where the theoretical molecular weight (MW) represents one of the principal influencing factors. The aim of the present study was to analyse the relationship between theoretical MWs and the ratios of concentrations of several molecules evaluated both in sera and in blister fluids. Eight cytokines (interleukin-2, interleukin-3, interleukin-4, interleukin-5, interleukin-10, tumor necrosis factor-alpha, oncostatin-M and vascular endothelial growth factor), two acute phase reactants (alpha-1 acid glycoprotein, haptoglobin), albumin, one soluble membrane molecule with adhesion functions (sICAM-1) and the eosinophil cathionic protein (ECP) were measured in samples from 15 patients affected with BP by means of commercially available tests. The data suggest that the MW may influence the rate of diffusion throughout the blister, both in input and output directions, despite the discontinuity observed at the basement membrane level on the BP blister floor.

Eosinophils in lichen sclerosus et atrophicus.

Science.gov (United States)

Keith, Phillip J; Wolz, Michael M; Peters, Margot S

2015-10-01

The classic histopathologic features of lichen sclerosus et atrophicus (LS) include lymphoplasmacytic inflammation below a zone of dermal edema and sclerosis. The presence of eosinophils in LS has received little attention, but the finding of tissue eosinophils, particularly eosinophilic spongiosis in LS, has been suggested as a marker for the coexistence of autoimmune bullous disease or allergic contact dermatitis (or both). We sought to determine whether the histopathologic presence of dermal eosinophils or eosinophilic spongiosis (or both) in biopsies from patients with LS is associated with autoimmune bullous disease, autoimmune connective tissue disease or allergic contact dermatitis. A retrospective review of the histopathology and medical records of 235 patients with LS who were evaluated from June 1992 to June 2012 was performed. Sixty-nine patients (29%) had eosinophils on histopathology. Among patients with associated diseases, a statistically significant association between the eosinophil cohort and the cohort without eosinophils was not detected. The importance of eosinophils is uncertain, but our data suggest that the finding of tissue eosinophils alone is not sufficient to prompt an extensive workup for additional diagnoses. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Broncho-pleural fistula with hydropneumothorax at CT: Diagnostic implications in mycobacterium avium complex lung disease with pleural involvement

International Nuclear Information System (INIS)

Yoon, Hyun Jung; Chung, Myung Jin; Lee, Kyung Soo; Park, Hye Yun; Koh, Won Jung; Kim, Jung Soo

2016-01-01

To determine the patho-mechanism of pleural effusion or hydropneumothorax in Mycobacterium avium complex (MAC) lung disease through the computed tomographic (CT) findings. We retrospectively collected data from 5 patients who had pleural fluid samples that were culture-positive for MAC between January 2001 and December 2013. The clinical findings were investigated and the radiological findings on chest CT were reviewed by 2 radiologists. The 5 patients were all male with a median age of 77 and all had underlying comorbid conditions. Pleural fluid analysis revealed a wide range of white blood cell counts (410-100690/µL). The causative microorganisms were determined as Mycobacterium avium and Mycobacterium intracellulare in 1 and 4 patients, respectively. Radiologically, the peripheral portion of the involved lung demonstrated fibro-bullous changes or cavitary lesions causing lung destruction, reflecting the chronic, insidious nature of MAC lung disease. All patients had broncho-pleural fistulas (BPFs) and pneumothorax was accompanied with pleural effusion. In patients with underlying MAC lung disease who present with pleural effusion, the presence of BPFs and pleural air on CT imaging are indicative that spread of MAC infection is the cause of the effusion

Broncho-pleural fistula with hydropneumothorax at CT: Diagnostic implications in mycobacterium avium complex lung disease with pleural involvement

Energy Technology Data Exchange (ETDEWEB)

Yoon, Hyun Jung; Chung, Myung Jin; Lee, Kyung Soo; Park, Hye Yun; Koh, Won Jung [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of); Kim, Jung Soo [Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Inha University Hospital, Inha University School of Medicine, Incheon (Korea, Republic of)

2016-04-15

To determine the patho-mechanism of pleural effusion or hydropneumothorax in Mycobacterium avium complex (MAC) lung disease through the computed tomographic (CT) findings. We retrospectively collected data from 5 patients who had pleural fluid samples that were culture-positive for MAC between January 2001 and December 2013. The clinical findings were investigated and the radiological findings on chest CT were reviewed by 2 radiologists. The 5 patients were all male with a median age of 77 and all had underlying comorbid conditions. Pleural fluid analysis revealed a wide range of white blood cell counts (410-100690/µL). The causative microorganisms were determined as Mycobacterium avium and Mycobacterium intracellulare in 1 and 4 patients, respectively. Radiologically, the peripheral portion of the involved lung demonstrated fibro-bullous changes or cavitary lesions causing lung destruction, reflecting the chronic, insidious nature of MAC lung disease. All patients had broncho-pleural fistulas (BPFs) and pneumothorax was accompanied with pleural effusion. In patients with underlying MAC lung disease who present with pleural effusion, the presence of BPFs and pleural air on CT imaging are indicative that spread of MAC infection is the cause of the effusion.

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

Directory of Open Access Journals (Sweden)

Melissa Ann Brown

2012-06-01

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

The “Pearls” of Multidisciplinary Team: Conquering the Uncommon Rosette Rash

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Nitin Verma

2016-01-01

Full Text Available Linear IgA disease of childhood (LAD also known as chronic bullous disease of childhood is an autoimmune disease with IgA deposition at the basement membrane zone leading to a vesiculobullous rash. It has a clinical appearance which frequently is described as resembling “strings of pearls” or rosette-like. Diagnosis is usually clinical but sometimes biopsy is required. Dapsone is widely considered to be the first line therapy in the treatment of LAD. A 5-year-old girl presented with 4-day history of a widespread painful rash and pyrexia. The rash transformed into painful blisters. A recent contact with chickenpox was present. She remained apyrexial but hemodynamically stable and was treated as chickenpox patient with secondary infection. Due to persistent symptoms after repeated attendance she was reviewed by Dermatology team and diagnosed with linear IgA disease also known as chronic bullous disease of childhood. This was based on the presence of blistering rash with rosette appearance and string of pearl lesions. The clinical features of LAD can be difficult to distinguish from more common skin infections. Benefiting from the experience of other multidisciplinary teams can sometimes be a game changer and can lead to the correct diagnosis and treatment.

Delayed diagnosis of bullous pyoderma gangrenosum with acute ...

African Journals Online (AJOL)

2016-05-10

May 10, 2016 ... Infectious Disease, School of Medicine, Inönü University, Malatya, Turkey. Access this article online .... because prompt administration of glucocorticoids or other immunosuppressive ... corneal keratolysis. Am J Clin Pathol ...

'An eye, for eyes - mission' - From dream to reality

OpenAIRE

Parikumar P; Haraguchi K; Senthilkumar R; Abraham S

2013-01-01

Corneal transplantation has been in routine practice to treat corneal endothelial diseases like Bullous Keratopathy, in which either the whole cornea or the partial cornea (the endothelium alone) is transplanted from the cadaver donor to the recipient with the endothelial disease [1]. In whole corneal or partial corneal transplant one cadaver donor's cornea can be used to treat one recipient cornea only, which leads to a huge global shortage of donor corneas [2]. At this juncture Yokoo et al ...

[Management of corneal endothelial decompensation with Descemet's membrane endothelial keratoplasty in a patient with Ahmed glaucoma valve implant].

Science.gov (United States)

Röck, T; Bartz-Schmidt, K-U; Röck, D; Yoeruek, E

2014-05-01

Currently, the main causes for developing bullous keratopathy are from problems related to intraocular surgery, trauma, infection, Fuchs' endothelial dystrophy and chronically elevated intraocular pressure. In the 1990s penetrating keratoplasty was once considered the therapy of choice for treatment of bullous keratopathy but in recent years it has been replaced by posterior lamellar keratoplasty. The Descemet membrane endothelial keratoplasty (DMEK) procedure represents the final development of posterior lamellar keratoplasty. The question now arises whether DMEK can be used in patients with bullous keratopathy and Ahmed glaucoma valve implant. A 72-year-old man was referred to our hospital for further evaluation with the diagnosis of bullous keratopathy and pseudoexfoliative glaucoma. The bullous keratopathy was caused by a variety of previous operations as well as decompensation of intraocular pressure. This article describes the therapy of bullous keratopathy by DMEK with existing Ahmed glaucoma valve implant. After surgery the cornea became clear and the best-corrected visual acuity improved from hand movement to 0.2. The intraocular pressure remained normal (10-14 mmHg) without antiglaucoma medication and the endothelial cell count decreased only slightly over a follow-up of 13 months. No complications were encountered. The DMEK surgical procedure seems to be possible in patients with Ahmed glaucoma valve implant and endothelial decompensation. However, further studies with a larger number of patients should follow to validate the replacement of penetrating keratoplasty and other posterior lamellar procedures by DMEK.

Outbreak of bullous impetigo caused by Staphylococcus aureus strains of phage type 3C/71 in a maternity ward linked to nasal carriage of a healthcare worker.

Science.gov (United States)

Piechowicz, Lidia; Garbacz, Katarzyna; Budzyńska, Anna; Dąbrowska-Szponar, Maria

2012-01-01

We describe an outbreak of bullous impetigo (BI) that occurred in a maternity unit and show phenotypic and genotypic properties and relatedness of isolated Staphylococcus aureus strains. Clinical material was obtained from 11 affected neonates. Additionally, nasal swabs from 67 healthy care workers (HCWs) as well as 107 environmental swabs were investigated. All isolates were screened for exfoliative toxin genes (eta, etb), antibiotic susceptibility and phage typed. Chromosomal DNA was genotyped by MLVF method and PCR/RFLP of coagulase gene were tested. Affected neonates were infected by two clusters of eta-positive S. aureus of phage type 3C/71: (1) MLVF type A isolates resistant only to penicillin, and (2) MLVF type B isolates resistant to penicillin and erythromycin/clindamycin. All isolates were susceptible to methicillin. We found 19 of 67 HCWs to be S. aureus nasal carriers. Two nasal isolates from HCWs were related to the outbreak on the basis of phage typing, PCR detection of eta/etb genes, antibiotyping and genotyping. Additionally, environmental swabs from the maternity unit revealed a 3C/71 S. aureus in the mattress of a baby bed. This is the first documented case of an outbreak of BI caused by phage type 3C/71 eta-positive strain of S. aureus.

The Prevalence of Pemphigus (Razi Hospital and Department of Oral Pathology, Tehran University of Medical Sciences

Directory of Open Access Journals (Sweden)

Eshghyar N

1999-12-01

Full Text Available The aim of this retrospective statistical study was to determine the prevalcence and frequency of"nage and sex distributions of pemphigus disease. Pemphigus disease classified as autoimmune bullous"ndermatoses which is a chronic mucocutaneous disease."nThis study was performed in Razi Hospital and department of oral pathology of dental school, Tehran"nUniversity of Medical Sciences. The most frequently effected area was buccal moucosa of oral cavity. The"nmost rate of recurrence was found in oral cavity which being more common in middle age females (25-44"nyears.

A case showing a blistering disorder in radiation dermatitis during radiation therapy

International Nuclear Information System (INIS)

Nonoshita, Takeshi; Nakamura, Katsumasa; Shioyama, Yoshiyuki

2007-01-01

We experienced a case showing a blistering disorder in radiation dermatitis during radiation therapy for thymic cancer. Application of steroid to the lesion improved blisters. The literature on bullous eruption including radiation-induced bullous pemhigoid was critically reviewed. (author)

TISSUE INHIBITOR OF METALLOPROTEINASE 1, MATRIX METALLOPROTEINASE 9, ALPHA-1 ANTITRYPSIN, METALLOTHIONEIN AND UROKINASE TYPE PLASMINOGEN ACTIVATOR RECEPTOR IN SKIN BIOPSIES FROM PATIENTS AFFECTED BY AUTOIMMUNE BLISTERING DISEASES

Directory of Open Access Journals (Sweden)

Ana Maria Abreu Velez

2013-07-01

Full Text Available Introduction: Proteinases and proteinase inhibitors have been described to play a role in autoimmune skin blistering diseases. We studied skin lesional biopsies from patients affected by several autoimmune skin blistering diseases for proteinases and proteinase inhibitors. Methods: We utilized immunohistochemistry to evaluate biopsies for alpha-1-antitrypsin, human matrix metalloproteinase 9 (MMP9, human tissue inhibitor of metalloproteinases 1 (TIMP-1, metallothionein and urokinase type plasminogen activator receptor (uPAR. We tested 30 patients affected by endemic pemphigus, 30 controls from the endemic area, and 15 normal controls. We also tested 30 biopsies from patients with bullous pemphigoid (BP, 20 with pemphigus vulgaris (PV, 8 with pemphigus foliaceus, and 14 with dermatitis herpetiformis (DH. Results: Contrary to findings in the current literature, most autoimmune skin blistering disease biopsies were negative for uPAR and MMP9. Only some chronic patients with El Bagre-EPF were positive to MMP9 in the dermis, in proximity to telocytes. TIMP-1 and metallothionein were positive in half of the biopsies from BP patients at the basement membrane of the skin, within several skin appendices, in areas of dermal blood vessel inflammation and within dermal mesenchymal-epithelial cell junctions.

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.

Pseudoporfyri er et symptomkompleks

DEFF Research Database (Denmark)

Velander, Marie Juul; Þorsteinsdóttir, Sunna; Bygum, Anette

2015-01-01

Pseudoporphyria is a photosensitive bullous disease, which resembles porphyria cutanea tarda. Normal porphyrin levels in urine, stool and blood define pseudoporphyria. Pseudoporphyria is associated with chronic renal failure, haemodialysis, a variety of drugs (e.g. naproxen, nabumetone, furosemid......, ciprofloxacin, voriconazole, acitretin), tanning beds and UVA exposure. Treatment consists of UV protection and cessation of suspected agents. Patients in haemodialysis can benefit from treatment with N-acetylcysteine or glutathione....

(3)He pO2 mapping is limited by delayed-ventilation and diffusion in chronic obstructive pulmonary disease.

Science.gov (United States)

Marshall, Helen; Parra-Robles, Juan; Deppe, Martin H; Lipson, David A; Lawson, Rod; Wild, Jim M

2014-03-01

Lung pO2 mapping with (3)He MRI assumes that the sources of signal decay with time during a breath-hold are radiofrequency depolarization and oxygen-dependent T1 relaxation, but the method is sensitive to other sources of spatio-temporal signal change such as diffusion. The purpose of this work was to assess the use of (3)He pO2 mapping in patients with chronic obstructive pulmonary disease. Ten patients with moderate to severe chronic obstructive pulmonary disease were scanned with a 3D single breath-hold pO2 mapping sequence. Images showed signal increasing over time in some lung regions due to delayed ventilation during breath-hold. Regions of physically unrealistic negative pO2 values were seen in all patients, and regional mean pO2 values of -0.3 bar were measured in the two patients most affected by delayed ventilation (where mean time to signal onset was 3-4 s). Movement of gas within the lungs during breath-hold causes regional changes in signal over time that are not related to oxygen concentration, leading to erroneous pO2 measurements using the linear oxygen-dependent signal decay model. These spatio-temporal sources of signal change cannot be reliably separated at present, making pO2 mapping using this methodology unreliable in chronic obstructive pulmonary disease patients with significant bullous emphysema or delayed ventilation. Copyright © 2013 Wiley Periodicals, Inc.

Marijuana and Lung Disease.

Science.gov (United States)

Tashkin, Donald P

2018-05-17

As marijuana smoking prevalence increases in the U.S. concern regarding its potential risks to lung health has also risen, given the general similarity in the smoke contents between marijuana and tobacco. Most studies have found a significant association between marijuana smoking and chronic bronchitis symptoms after adjustment for tobacco. While reports are mixed regarding associations between marijuana smoking and lung function, none has shown a relationship to decrements in forced expired volume in 1 sec (FEV1) and few have found a relationship to a decreased ratio of FEV1 to forced vital capacity (FVC), possibly related to an association between marijuana and an increased FVC. A few studies have found a modest reduction in specific airway conductance in relation to marijuana, probably reflecting endoscopic evidence of bronchial mucosal edema among habitual marijuana smokers. Diffusing capacity in marijuana smokers has been normal and two studies of thoracic high-resolution computed tomography (HRCT) have not shown any association of marijuana smoking with emphysema. Although bronchial biopsies from habitual marijuana smokers have shown precancerous histopathological changes, a large cohort study and a pooled analysis of six well-designed case-control studies have not found evidence of a link between marijuana smoking and lung cancer. The immunosuppressive effects of delta-9 tetrahydrocannabinol raise the possibility of an increased risk of pneumonia, but further studies are needed to evaluate this potential risk. Several cases series have demonstrated pneumothoraces/pneumomediastinum, as well as bullous lung disease, in marijuana smokers, but these associations require epidemiologic studies for firmer evidence of possible causality. Copyright © 2018. Published by Elsevier Inc.

Eritema multiforme ampollar extenso asociado a infección por virus herpes simplex Extended Bullous Erythema Multiforme Associated To Herpes Simplex Virus Infection

Directory of Open Access Journals (Sweden)

A Elgueta-Noy

2009-12-01

Full Text Available El Eritema Multiforme (EM es una reacción cutánea aguda generalmente benigna y autolimitada, asociada a la infección por Virus Herpes Simplex (HSV. Se caracteriza por lesiones polimorfas y tipo diana en extremidades y mucosas. Presentamos un paciente de 22 años con pápulas, vesículas y ampollas, que evoluciona con un 90% de la superficie corporal comprometida en tres semanas. Se realizó una reacción de polimerasa en cadena para HSV, resultando positiva en una costra. La biopsia de piel y la tinción de inmunohistoquímica positiva para linfocitos T CD4, fueron compatibles con EM ampollar asociado a HSV. Destacamos la importancia de la correlación clínico patológica, apoyada por el estudio virológico, en el diagnóstico de este caso de presentación atípica. Los hallazgos de laboratorio confirmaron lo descrito en la literatura respecto de la patogenia del EM asociado a HSV.Erythema Multiforme (EM is a generally benign and self-limited acute cutaneous reaction, associated with Herpes Simplex Virus (HSV infection. It is characterized by polymorphic "target" lesions in extremities and mucosal tissues. We report a 22-year old patient with papules, vesicles and blisters, which evolved to cover 90% of the body in three weeks. We performed a PCR study for HSV, which was positive in a crust. A skin biopsy and positive immunohistochemical stain for LT CD4+ were compatible with bullous EM associated with HSV. We underline the importance of pathological clinical correlation, reinforced by a virological study, in the diagnosis of this case with atypical symptoms. The laboratory findings confirmed literature descriptions with respect to the pathogenicity of EM associated with HSV.

Emphysema Is Common in Lungs of Cystic Fibrosis Lung Transplantation Patients: A Histopathological and Computed Tomography Study.

Directory of Open Access Journals (Sweden)

Onno M Mets

Full Text Available Lung disease in cystic fibrosis (CF involves excessive inflammation, repetitive infections and development of bronchiectasis. Recently, literature on emphysema in CF has emerged, which might become an increasingly important disease component due to the increased life expectancy. The purpose of this study was to assess the presence and extent of emphysema in endstage CF lungs.In explanted lungs of 20 CF patients emphysema was semi-quantitatively assessed on histology specimens. Also, emphysema was automatically quantified on pre-transplantation computed tomography (CT using the percentage of voxels below -950 Houndfield Units and was visually scored on CT. The relation between emphysema extent, pre-transplantation lung function and age was determined.All CF patients showed emphysema on histological examination: 3/20 (15% showed mild, 15/20 (75% moderate and 2/20 (10% severe emphysema, defined as 0-20% emphysema, 20-50% emphysema and >50% emphysema in residual lung tissue, respectively. Visually upper lobe bullous emphysema was identified in 13/20 and more diffuse non-bullous emphysema in 18/20. Histology showed a significant correlation to quantified CT emphysema (p = 0.03 and visual emphysema score (p = 0.001. CT and visual emphysema extent were positively correlated with age (p = 0.045 and p = 0.04, respectively.In conclusion, this study both pathologically and radiologically confirms that emphysema is common in end-stage CF lungs, and is age related. Emphysema might become an increasingly important disease component in the aging CF population.

Rola limfocytów T regulatorowych w patogenezie wybranych chorób pęcherzowych

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Elżbieta Waszczykowska

2011-07-01

Full Text Available Regulatory T cells (Treg are a phenotypically heterogeneous group ofcells, playing an important role in regulation of the immune response.To date, several subtypes of regulatory cells have been identified. Themost important of them are: lymphocytes CD4+CD25+, Tr1, Th3 andNK cells. Regulatory T lymphocytes act by secretion of suppressivecytokines (IL-10, TGF-β or direct cell-to-cell interaction. Functionalabnormalities of regulatory T cells are important in pathogenesis ofmany diseases, especially autoimmune ones. Based on published studies,the review discusses the role of regulatory T cells in pathogenesisof selected bullous skin diseases.

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

Epidemiological investigation of nosocomial outbreak of staphylococcal skin diseases in neonatal ward.

Science.gov (United States)

Kurlenda, J; Grinholc, M; Krzysztoń-Russjan, J; Wiśniewska, K

2009-05-01

During a 1-month period, eight neonates developed staphylococcal skin disease diagnosed as a bullous impetigo in the maternity unit of the Provincial Hospital in Gdansk. An epidemiological investigation based on phenotyping and genotyping methods was performed. All neonates involved in the outbreak, their mothers and 15 staff members were screened for carriage of Staphylococcus aureus by nasal swabs. Isolated strains were compared with strains cultured from affected skin and purulent conjunctiva of infected newborns. Isolates were analyzed for the presence of the etA and etB genes using polymerase chain reaction and genotyped by pulsed-field gel electrophoresis (PFGE) and coa gene polymorphism. The analyzed S. aureus strains were methicillin-sensitive and could be divided into two groups according to antibiotyping, phage typing, coa polymorphism and PFGE pattern. The first group consisted of etA and etB negative strains, and the second one involved only the etB positive ones. Our results have shown that there were two different clusters of infection caused by two populations of S. aureus strains. Among the 15 medical staff members screened we have found seven carriers. However, phage typing revealed that distinct strains unrelated to the outbreak isolates were carried. Although we have not been able to establish the source of bacteria involved in the outbreak, our results suggest that for both groups, mothers could be the source of the infecting strains.

An autoradiographic study on the mechanism of mast cell hyperplasia evoked by a carcinogen, 20-methylcholanthrene

International Nuclear Information System (INIS)

Tada, Narashi

1974-01-01

A reactive increase of mast cells was studied by means of 3 H-thymidine autoradiography. Mice were painted on the back skin with 20-methylcholanthrene twice a week for two to eight weeks. No labeled mast cells were found with flash labeling or fourty-eight hours' cumulative labeling immediately before the sacrifice at the end of varying periods of painting. Subsequently, a cumulative chase method was performed in order to obtain labeled mast cells. Mice were painted with 20-methylcholanthrene for thirty-one days. Twenty-four hours' cumulative labelings were performed 31 (i.e. at the beginning of painting), 21, 14, 7, 5 and 3 days before the sacrifice. In each of these labeling experiments, labeled mast cells were observed. From these results it was concluded that the increase of mast cells in response to an irritation is not due to the proliferation of mast cells themselves but to the proliferation of undifferentiated precursor cells and their differentiation into the mast cells. A histogram of the labeling indices of mast cells in the subepidermal, dermal and subcutaneous layers indicated that, in the early period of painting, a peak of labeling indices of mast cells appeared in the subcutaneous layer, and that, in the late period of painting, a peak of indices appeared in the subepidermal layer. This suggests that mast cells are produced in the subepidermal layer and migrate into the deep layers of the skin. (author)

The Role of PTHrP in Mammary Gland Development and Tumorigenesis

National Research Council Canada - National Science Library

Wysolmerski, John

1999-01-01

.... The PTHrP receptor is expressed throughout the sub-epidermal mesenchyme. In those mesenchymal cells closest to the mammary epithelial bud, PTHrP induces a change in cell fate allowing those cells to become functional mammary mesenchymal cells...

Evaluation of four non-invasive methods for examination and characterization of pressure ulcers

DEFF Research Database (Denmark)

Andersen, E.S.; Karlsmark, T.

2008-01-01

Background: Pressure ulcers are globally of major concern and there is need for research in the pathogenesis for early intervention. Early studies have suggested existence of a hypo-echogenic subepidermal layer at the location of pressure ulcers, visualized by ultrasound scans. As a continuation......, we here report on usability of four non-invasive techniques for evaluation of pressure ulcers. Methods: Fifteen pressure ulcers in stage 0-IV were examined using four different non-invasive techniques [redness index, skin temperature, skin elasticity (i.e. retraction time), and ultrasound scanning...... at all pressure ulcers, but none at the reference points. The skin retraction time was often higher at the location of a pressure ulcer than at the reference location. We found no correlation between the stage of the ulcers and temperature, redness index, subepidermal layer thickness, or retraction time...

Treatment of Large Bulla Formation after Tattoo Removal with a Q-Switched Laser

Science.gov (United States)

Kartono, Francisca; Desai, Alpesh; Kaur, Ravneet R.; Desai, Tejas

2010-01-01

Widely considered the gold standard treatment option for tattoo removal, the use of Q-switched lasers may very rarely result in the formation of large bulla. While very disconcerting to patients, these lesions are easily managed and, with proper care, heal quickly with no long-term consequences. The authors present three cases of patients who had bullous reactions shortly after receiving Q-switched laser treatment of tattoo ink. Bullous formation in all three patients was treated successfully. PMID:20725537

Angioma serpiginosum with oesophageal papillomatosis is an X-linked dominant condition that maps to Xp11.3-Xq12

NARCIS (Netherlands)

Blinkenberg, Ellen O.; Brendehaug, Atle; Sandvik, Arne K.; Vatne, Oystein; Hennekam, Raoul C. M.; Houge, Gunnar

2007-01-01

We report on a four-generation family with localized subepidermal telangiectasias following Blaschko's lines (angioma serpiginosum). The vascular streaks are present at birth and progress slowly thereafter. In several family members papillomatosis of the entire oesophagus was found to be part of the

"EB, or Not EB?" Neonatal Desquamative Impetigo in a Degloving Pattern.

Science.gov (United States)

Nguyen, Tuyet A; Wang, Audrey S; Eichenfield, Lawrence F; Barrio, Victoria

2016-01-01

We present the case of a 7-day-old boy with significant, rapidly spreading blistering and desquamation in a "degloving" pattern on the hands that mimicked epidermolysis bullosa but was ultimately diagnosed as bullous impetigo caused by a clinically aggressive strain of Staphylococcus aureus. Bullous impetigo is a desquamating condition caused by local release of S. aureus exfoliative toxin A and is more commonly seen in children. This case highlights the fragility of newborn skin and reviews the major diagnoses that should be considered in an infant with significant blistering. © 2016 Wiley Periodicals, Inc.

Dermatitis Herpetiformis and Vitiligo

Directory of Open Access Journals (Sweden)

Ozlem Karabudak

2007-11-01

Full Text Available Dermatitis herpetiformis (DH is a rare immunobullous disorder of the skin that is associated with gluten hypersensitivity. Subepidermal IgA-type antibody deposition against tissue transglutaminase leads to dense neutrophilic microabscess and eventually into vesicles in dermal papillae, which may occasionally merge into bullae. Being a subepidermal vesiculobullous disorder, DH is frequently associated with postinflammatory pigmentary changes, particularly hypopigmentation. However, the association of DH with true vitiligo is extremely rare. Here, we report a 21-year-old male with vitiligo and comorbid DH, and review the literature. This new case had severely pruritic, papular and papulovesicular lesions that were localized symmetrically and partly confined to the pre-existing vitiliginous areas. The skin biopsy specimen taken from an erythematous papule on the elbow showed characteristic findings of DH and vitiligo. Direct immunofluorescence microscopy of the perilesional skin revealed granular IgA deposition of dermal papillae. There are only 10 reports in the literature of DH and vitiligo comorbidity.

IMMUNOHISTOCHEMISTRY VERSUS IMMUNOFLUORESENCE IN THE DIAGNOSIS OF AUTOIMMUNE BLISTERING DISEASES

Directory of Open Access Journals (Sweden)

Ana Maria Abreu Velez

2013-11-01

Full Text Available Introduction: In patients with autoimmune skin blistering diseases (ABDs, the diagnostic gold standard has classically been direct and indirect immunofluorescence (DIF and IIF, despite inherent technical problems of autofluorescence. Aim: We sought to overcome autofluorescence issues and compare the reliability of immunofluorescence versus immunohistochemistry (IHC staining in the diagnoses of these diseases. Methods: We tested via IHC for anti-human IgG, IgM, IgA, IgD, IgE, Kappa light chains, Lambda light chains, Complement/C3c, Complement/C1q, Complement/C3d, albumin and fibrinogen in 30 patients affected by a new variant of endemic pemphigus foliaceus in El Bagre, Colombia (El Bagre-EPF, and 30 control biopsies from the endemic area. We also tested archival biopsies from patients with ABDs whose diagnoses were made clinically, histopathologically and by DIF/IIF studies from 2 independent dermatopathology laboratories in the USA. Specifically, we tested 34 patients with bullous pemphigoid (BP, 18 with pemphigus vulgaris (PV, 8 with pemphigus foliaceus (PF, 14 with dermatitis herpetiformis (DH and 30 control skin samples from plastic esthetic surgery reduction surgeries. Results: The diagnostic correlation between IHC and DIF-IIF was almost 98% in most cases. IHC revealed evidence of autofluorescence around dermal blood vessels, dermal eccrine glands and neurovascular packages feeding skin appendices in ABDs; this autofluorescence may represent a non-specific immune response. Strong patterns of positivity were seen also in endothelial-mesenchymal cell junction-like structures, as well as between dermal fibrohistiocytic cells. In PV, we noted strong reactivity to neurovascular packages supplying sebaceous glands, as well as apocrine glands with edematous changes. Conclusions: We suggest that IHC is as reliable as DIF or IIF for the diagnosis of ABDs; our findings further suggest that what has previously been considered DIF/IIF autofluorescence

Macrolides in Chronic Inflammatory Skin Disorders

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Abdullateef A. Alzolibani

2012-01-01

Full Text Available Long-term therapy with the macrolide antibiotic erythromycin was shown to alter the clinical course of diffuse panbronchiolitis in the late 1980s. Since that time, macrolides have been found to have a large number of anti-inflammatory properties in addition to being antimicrobials. These observations provided the rationale for many studies performed to assess the usefulness of macrolides in other inflammatory diseases including skin and hair disorders, such as rosacea, psoriasis, pityriasis rosea, alopecia areata, bullous pemphigoid, and pityriasis lichenoides. This paper summarizes a collection of clinical studies and case reports dealing with the potential benefits of macrolides antibiotics in the treatment of selected dermatoses which have primarily been classified as noninfectious and demonstrating their potential for being disease-modifying agents.

Macrolides in Chronic Inflammatory Skin Disorders

Science.gov (United States)

Alzolibani, Abdullateef A.; Zedan, Khaled

2012-01-01

Long-term therapy with the macrolide antibiotic erythromycin was shown to alter the clinical course of diffuse panbronchiolitis in the late 1980s. Since that time, macrolides have been found to have a large number of anti-inflammatory properties in addition to being antimicrobials. These observations provided the rationale for many studies performed to assess the usefulness of macrolides in other inflammatory diseases including skin and hair disorders, such as rosacea, psoriasis, pityriasis rosea, alopecia areata, bullous pemphigoid, and pityriasis lichenoides. This paper summarizes a collection of clinical studies and case reports dealing with the potential benefits of macrolides antibiotics in the treatment of selected dermatoses which have primarily been classified as noninfectious and demonstrating their potential for being disease-modifying agents. PMID:22685371

Behçet's syndrome with pyoderma-gangrenosum-like lesions treated successfully with dapsone monotherapy.

Science.gov (United States)

Joshi, Arun; Mamta

2004-10-01

Behçet's syndrome (BS) is a rare multisystem disorder belonging to a group of neutrophilic dermatoses. We report a 65-year-old male patient who had suffered from recurrent painful orogenital ulcers for 50 years from the age of 15 and started developing pustular and bullous lesions evolving into non-healing ulcers similar to those seen in pyoderma gangrenosum (PG) two months prior to presenting to us. There was no evidence of systemic disease or malignancy. Routine baseline investigations were within normal limits. The patient was treated successfully with dapsone, antibiotics, and local wound care.

Bulløs pemfigoid rammer også yngre patienter

DEFF Research Database (Denmark)

Madsen, Jens Otto Broby; Lauritzen, Anne Falensteen; Lei, Ulrikke

2015-01-01

Bullous pemphigoid is most commonly seen in elderly patients, however, an increasing number of cases in children have been reported. In this article we present a case of a five-month-old boy who was admitted with red annular plaques over his entire body and vesicles and bullae on his hands and feet....... Correct diagnosis was made by skin biopsy and after reviewing the literature high dose oral steroids therapy (1 mg/kg/day) was successfully initiated. The cause of the disease remains unknown. With correct treatment the prognosis is good with documented ten-year follow-up without relapse....

Catarata subcapsular posterior em pacientes com pênfigo e penfigóide tratados com corticoterapia oral Posterior subcapsular cataract in patients with pemphigus and pemphigoid using oral corticosteroid

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Viviane Reggiani

2004-10-01

Full Text Available FUNDAMENTO: O tratamento mais utilizado para pacientes com dermatoses bolhosas crônicas é a corticoterapia oral, muitas vezes em altas doses e por períodos prolongados. Como efeitos colaterais dessa terapêutica, freqüentemente ocorrem: hipertensão arterial, diabete, osteoporose, infecções e distúrbios hidroeletrolíticos. A catarata subcapsular posterior é raramente citada na literatura como efeito colateral da corticoterapia em pacientes com doenças bolhosas. OBJETIVO: Avaliar a prevalência de catarata subcapsular posterior como efeito colateral da corticoterapia oral. MÉTODOS: Trata-se de um estudo retrospectivo incluindo 49 pacientes com dermatoses bolhosas crônicas em uso de corticoterapia acompanhados entre janeiro de 1987 e dezembro de 1997 no ambulatório de Dermatoses Bolhosas do Departamento de Dermatologia da Unifesp/EPM e regularmente submetidos à avaliação oftalmológica em busca de catarata cortisônica. RESULTADOS: No período de 1987 a 1997, 49 pacientes foram avaliados, tendo apresentado prevalência de catarata subcapsular posterior como efeito colateral da corticoterapia oral de 28,57%. CONCLUSÕES: 1 a prevalência de catarata foi de 28,57%; 2 o tempo médio de tratamento até o surgimento de catarata foi de 45,71 meses; 3 a média da dose máxima de corticosteróide utilizada pelos pacientes foi de 78,57mg/dia.BACKGROUND: Oral corticosteroid is the treatment most used for patients with chronic bullous dermatosis, often at high dosages and over long periods of time. Arterial hypertension, diabetes, osteoporosis, infections and hydro-electrolytic disturbances frequently occur as side effects of this therapy. Posterior subcapsular cataract is rarely mentioned in the literature as a side effect of corticosteroid in patients with bullous diseases. OBJECTIVES: To evaluate the incidence of posterior subcapsular cataract as a side effect of oral corticosteroid. METHODS: A retrospective study: 49 patients with chronic

Exaggerated Arthropod Bite: A Case Report and Review of the Mimics

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Sagah Ahmed

2018-01-01

Full Text Available Exaggerated arthropod bite reactions causing hemorrhagic or necrotic bullous lesions can mimic other serious conditions such as cutaneous anthrax, brown recluse spider bite, and tularemia. A 55- year-old, healthy woman presented to the emergency department with a 3.5-centimeter painless, collapsed hemorrhagic bulla at the left costal margin. She was afebrile and had no systemic symptoms. Laboratory evaluation was unremarkable. She was prescribed silver sulfadiazine cream and mupirocin ointment. The area denuded two days later and the lesion completely healed. This case illustrates the broad differential to be considered when evaluating patients with hemorrhagic bullous lesions.

Modified osteo-odonto-keratoprosthesis for treatment of corneal blindness: long-term anatomical and functional outcomes in 181 cases.

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Falcinelli, Giancarlo; Falsini, Benedetto; Taloni, Maurizio; Colliardo, Paolo; Falcinelli, Giovanni

2005-10-01

To evaluate long-term anatomical and functional outcomes of a modified osteo-odonto-keratoprosthesis (OOKP) technique for treatment of corneal blindness from various etiologies. Two-hundred three patients (224 eyes) underwent modified OOKP surgery between 1973 and 1999. Of the original cohort, 181 patients (98 men and 83 women; mean [SD], age 54.3 [15] years) in whom a standardized 2-step surgical procedure was performed were included in the study. Preoperative diagnoses were dry eye (n = 70) due to ocular pemphigoid (n = 39), Sjögren syndrome (n = 11), trachoma (n = 8), Lyell syndrome (n = 6), Stevens-Johnson syndrome (n = 4), and graft-vs-host disease (n = 1) and congenital lid coloboma (n = 1), severe corneal burns (n = 68), bullous keratopathy (n = 13), keratitis sequelae (n = 15), and bullous keratopathy secondary to antiglaucoma surgery (n = 15). Several innovations were made to the original Strampelli technique. Median follow-up duration was 12 years (range, 1-25 years). Anatomical complications leading to OOKP loss were found in 11 (6.07%) of 181 patients. Survival analysis estimated that 18 years after surgery, the probability of retaining an intact OOKP was 85% (95% confidence interval, 79.3%-90.7%). Pooling patient groups, mean (SD) best postoperative visual acuity was 0.76 (0.34). Mean (SD) final acuity at the end of follow-up declined slightly (0.69 [0.39]) but significantly (P<.01). In individual diagnostic groups, mean acuity decline reached statistical significance (P<.05) only in the pemphigoid (1 line), trachoma (1 line), and bullous keratopathy secondary to antiglaucoma surgery (2 lines) groups. Survival analysis estimated that 18 years after surgery, the probability of retaining best postoperative visual acuity (within 2 lines) was mean (SD) 55.5% (12.9%). Modified OOKP surgery for corneal blindness of different etiologies may provide, in the long-term, anatomically stable corneal prosthesis as well as an effective, rehabilitating recovery in

Hidradenitis suppurativa/acne inversa: bilocated epithelial hyperplasia with very different sequelae.

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von Laffert, M; Stadie, V; Wohlrab, J; Marsch, W C

2011-02-01

Hidradenitis suppurativa/acne inversa is a chronic, inflammatory, scarring disease in the terminal hair follicle and apocrine gland-bearing areas (skin folds). There is considerable histological evidence that perifolliculitis and follicular hyperkeratosis precede the rupture of the follicle. The timing of the epithelial hyperplasia at the infundibula of inflamed terminal follicles has not yet been clarified. To clarify the early histopathological life of lesions ('chronology') in hidradenitis suppurativa/acne inversa, focusing on the terminal follicle structure and its surrounding tissue (hyperkeratosis, hyperplasia of follicular epithelium, perifolliculitis and rupture). In total, 485 operative specimens obtained from 128 patients with diagnosed hidradenitis suppurativa/acne inversa (all surgically treated by wide excision) were examined histologically. Two to five histological preparations (total 485) per operation area (total 196) were prepared by multiple slicing. Hidradenitis suppurativa/acne inversa showed a heterogeneous histological pattern: hyperkeratosis of the terminal follicles (89%), hyperplasia of follicular epithelium (80%), pronounced perifolliculitis (68%) and follicle rupture (24%). Perifolliculitis, follicular hyperkeratosis and hyperplasia occurred prior to the rupture of the follicle. Other histological criteria were: subepidermal cellular inflammatory infiltrate (82%), epidermal psoriasiform hyperplasia (56%), pronounced acute dermal inflammation (28%), pronounced chronic dermal inflammation (49%), and involvement of apocrine glands (52%) and subcutis (31%). Infundibular hyperkeratosis, hyperplasia of the follicular epithelium and perifolliculitis are major histopathological characteristics of hidradenitis suppurativa/acne inversa. These apparently precede rupture of the follicle. In particular, hyperplasia of the follicular epithelium probably marks the beginning of sinus formation, which usually spreads horizontally. Psoriasiform hyperplasia

[Anti-TNF alpha in dermatology].

Science.gov (United States)

Mahe, E; Descamps, V

2002-12-01

The discovery of the major role of TNF alpha in the physiopathology of certain inflammatory diseases and notably in rheumatoid arthritis and Crohn's disease has led to the development of anti-TNF alpha drugs. These new therapeutic arms issued from bio-technology have rapidly demonstrated their efficacy in the treatment of these two diseases. The anti-TNF alpha arsenal is currently dominated by etanercept, a fusion protein composed of a soluble TNF alpha receptor, and infliximab, a chimeric monoclonal antibody. However, new molecules will soon enrich this arsenal. TNF alpha is a major cytokine of inflammatory diseases of the skin. Many dermatological diseases will probably benefit from these new treatments. Two studies have already demonstrated their interest in cutaneous and articular psoriasis. Encouraging sporadic results suggest other potential indications (Behcet's disease, bullous dermatitis, neutrophilic dermatitis, toxic epidermal necrolysis, systemic vascularitis,.). These promising new treatments, although expensive, and with yet unknown long term side effects, justify rigorous assessment of their efficacy and tolerance in each indication. Here again the dermatologist has a major role to play in post-marketing pharmacovigilance.

Quantitative Fundus Autofluorescence in Best Vitelliform Macular Dystrophy: RPE Lipofuscin is not Increased in Non-Lesion Areas of Retina.

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Sparrow, Janet R; Duncker, Tobias; Woods, Russell; Delori, François C

2016-01-01

Since the lipofuscin of retinal pigment epithelial (RPE) cells has been implicated in the pathogenesis of Best vitelliform macular dystrophy, we quantified fundus autofluorescence (quantitative fundus autofluorescence, qAF) as an indirect measure of RPE lipofuscin levels. Mean non-lesion qAF was found to be within normal limits for age. By spectral domain optical coherence tomography (SD-OCT) vitelliform lesions presented as fluid-filled subretinal detachments containing reflective material. We discuss photoreceptor outer segment debris as the source of the intense fluorescence of these lesions and loss of anion channel functioning as an explanation for the bullous photoreceptor-RPE detachment. Unexplained is the propensity of the disease for central retina.

Digital subtraction cardiopulmonary angiography using FCR (Fuji computed radiography)

International Nuclear Information System (INIS)

Tanimura, Shigeo; Tomoyasu, Hiroshi; Banba, Jiro; Masaki, Mikio; Kanno, Yukio; Abe, Kazuo

1987-01-01

Digital subtraction cardiopulmonary angiography using FCR was performed on 46 patients including lung cancer, mediastinal tumor, giant bullous formation and others. The images of digital subtraction for pulmonary artery, pulmonary vein and thoracic aorta were studied by comparing to the conventional pulmonary angiogram. Good images of pulmonary artery due to digital subtraction were obtained in 80 % of the 45 cases. This method needed only half volume of contrast media compared to the conventional for obtaining good images and thus reduced side effect. Therefore this method seems to be an usefull pre-operative examination in various chest diseases, especially in case of lung cancer. (author)

Neovascularization of the iris in retinoschisis

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Geraldine R. Slean

2017-09-01

Conclusions and importance: Chronic bullous retinoschisis can be associated with anterior segment neovascularization such as rubeosis iridis, presumably due to non-perfusion within the retinoschisis cavity.

Skin: Major target organ of allergic reactions to small molecular weight compounds

International Nuclear Information System (INIS)

Merk, Hans F.; Baron, Jens M.; Neis, Mark M.; Obrigkeit, Daniela Hoeller; Karlberg, Ann-Therese

2007-01-01

Skin is a major target organ for allergic reactions to small molecular weight compounds. Drug allergic reactions may be life-threatening such as in the case of anaphylactic reactions or bullous drug reactions and occur in about 5% of all hospitalized patients. Allergic contact dermatitis has an enormous influence on the social life of the patient because it is the most frequent reason for occupational skin diseases and the treatment and prevention of this disease cost approximately Euro 3 billion per year in Germany. The different proposed pathophysiological pathways leading to a drug eruption are discussed in this paper. All major enzymes which are involved in the metabolism of xenobiotica were shown to be present in skin. Evidence supporting the role of metabolism in the development of drug allergy and allergic contact dermatitis is demonstrated in the example of sulphonamides and fragrances

Medical image of the week: aspergilloma

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Hsu W

2014-05-01

Full Text Available No abstract available. Article truncated after 150 words. A 69-year-old woman, a current smoker, with very severe chronic obstructive pulmonary disease and prior atypical mycobacterium, was found unresponsive by her family and intubated in the field by emergency medical services for respiratory distress. Her CT thorax showed severe emphysematous disease, apical bullous disease, and a large left upper lobe cavitation with debris (Figure 1. She was treated with broad-spectrum antibiotics and anti-fungal medications. Hemoptysis was never seen. Sputum cultures over a span of two weeks repeatedly showed Aspergillus fumigatus and outside medical records confirmed the patient had a known history of stable aspergilloma not requiring therapy. Aspergillomas usually arises in cavitary areas of the lung damaged by previous infections. The fungus ball is a combination of colonization by Aspergillus hyphae and cellular debris. Individuals with aspergillomas are usually asymptomatic or have mild symptoms (chronic cough and do not require treatment unless it begins to invade into the cavity ...

Connective tissue photodamage in the hairless mouse is partially reversible

International Nuclear Information System (INIS)

Kligman, L.H.

1987-01-01

Photodamaged connective tissue in animal and human skin is characterized by excessive accumulations of elastic fibers, loss of mature collagen, concomitant overproduction of new collagen, and greatly increased levels of glycosaminoglycans. Formerly considered irreversible changes, we recently showed in hairless mice, post irradiation, that a band of normal connective tissue was laid down subepidermally. The present studies focused on 2 aspects of this repair: whether repair would occur if animals were protected by sunscreens after dermal damage was induced and irradiation continued; whether retinoic acid could enhance the repair process. To examine the first aspect, albino hairless mice were irradiated with Westinghouse FS 20 sunlamps thrice weekly for 30 weeks. Sunscreens of high sun-protection factors were applied after 10 and 20 weeks. Not only was further damage prevented, but the damage incurred before sunscreen application was repaired. This appeared as subepidermal reconstruction zones containing normal, mature collagen and a network of fine elastic fibers. The second aspect was examined by applying 0.05% retinoic acid, topically, to animals preirradiated for 10 weeks. In contrast to controls treated with vehicle, the reconstruction zone was significantly wider in retinoic acid-treated mice. The enhanced repair was dose-related

Outcomes in bullous retinal detachment

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Sarah P. Read

2017-06-01

Conclusions and importance: GRTs are an uncommon cause of retinal detachment. While pars plana vitrectomy with tamponade is standard in GRT management, there is variability in the use of scleral buckling and PFO in these cases. This is in contrast to retinal dialysis where scleral buckle alone can yield favorable results. Though a baseball ocular trauma is common, retinal involvement is rare compared to other sports injuries such as those occurring with tennis, soccer and golf. Sports trauma remains an important cause of retinal injury and patients should be counseled on the need for eye protection.

Mucocutaneous manifestations in patients with chronic kidney disease: A cross-sectional study

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Rattan S Rashpa

2018-01-01

Full Text Available Background: Chronic kidney disease (CKD-associated mucocutaneous manifestations significantly impair the quality of life but often remain understudied. They may also vary across regions, socioeconomic and nutritional status, and racial differences. Objectives: To study the patterns of mucocutaneous disorders and their prevalence in CKD patients irrespective of clinical stage or dialysis status. Materials and Methods: 122 (M:F = 77:45 patients aged 21‒85 (Mean ± SD = 57.5 ± 14.0 years having CKD for 3 month to 5 years were studied for mucocutaneous manifestations. Fifty (41% patients were on hemodialysis for 1‒42 months. Detailed medical history, clinical and mucocutaneous examination, and lab investigations were performed. KOH mounts, skin biopsy, Gram's and Giemsa staining, bacterial or fungal cultures were performed as required. Results: Xerosis in 93 (76.2%, skin pallor in 61 (50%, pruritus in 57 (46.7%, pigmentation in 47 (38.5%, and purpura in 18 (14.8% patients were the major dermatoses. Bullous lesions and perforating folliculitis occurred in 3 (2.5% patients each. Major nail abnormalities were pallor (in 35.2%, absent lunula (in 23.8%, nail discoloration (in 18%, and “half-and-half nails” in 16.4% patients, respectively. Hair abnormalities included sparse scalp and body hairs (in 35.2% and 13.1%, respectively and lusterless hair in 12.3% patients. Coated tongue (in 14.8%, xerostomia (in 12.3%, and macroglossia with teeth indention (in 7.4% patients were the mucosal manifestations. Conclusions: Xerosis, pruritus, skin pallor/pigmentary changes, nail pallor, absent lunula, nail discoloration, sparse hairs, coated tongue, xerostomia, macroglossia, and infections were the most common mucocutaneous manifestations in the studied patients irrespective of hemodialysis status. Cold and dry climates might be additional aggravators for xerosis/pruritus. Lifelong follow-up may be needed to reduce the morbidity associated with CKD

Role of cutaneous surface fluid in frog osmoregulation

DEFF Research Database (Denmark)

Larsen, Erik Hviid; Ramløv, Hans

2013-01-01

are regulated by subepidermal exocrine gland secretion balanced by EWL into the atmosphere and ion reuptake by the epidermal epithelium. Previously discovered regulatory mechanisms of epithelial ion absorption, hitherto not ascribed a body function, fit well with a role in regulating turnover of CSF....... As a regulated external physiological compartment, CSF would be of importance for the immune defenses that amphibians employ in protecting their skin....

Beyond Psoriasis: Novel Uses for Biologic Response Modifiers in Pediatric Dermatology.

Science.gov (United States)

Bellodi-Schmidt, Fernanda; Shah, Kara N

2016-01-01

Dermatologists have witnessed the increasing availability of novel biologic response modifiers for the treatment of inflammatory and autoimmune diseases in recent years. The most common dermatologic indication for the use of biologic response modifiers in adults is psoriasis, but the U.S. Food and Drug Administration has not approved any of these agents for use in any dermatologic disease in children with the exception of omalizumab, and as such, use in this population is considered off-label. In this review, we focus on the use of these agents in children to treat inflammatory skin diseases other than psoriasis, including atopic dermatitis, hidradenitis suppurativa, pemphigus vulgaris, bullous pemphigoid, and toxic epidermal necrolysis, with an emphasis on the use of etanercept, infliximab, rituximab, omalizumab, and ustekinumab. By highlighting novel uses of these agents, particularly for the treatment of dermatologic conditions for which optimal therapies are yet to be established, we hope to raise awareness of the potential use of this class of medications to treat inflammatory skin diseases in children. © 2015 Wiley Periodicals, Inc.

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

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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

Risk of progression in macula-on rhegmatogenous retinal detachment.

Science.gov (United States)

Callizo, Josep; Pfeiffer, Sebastian; Lahme, Eva; van Oterendorp, Christian; Khattab, Mohammed; Bemme, Sebastian; Kulanga, Miroslav; Hoerauf, Hans; Feltgen, Nicolas

2017-08-01

To identify factors that may lead to a rapid progression in macula-on rhegmatogenous retinal detachment (RRD), in particular, those that may lead to macular involvement. Observational, prospective, single-center study. Patients referred for surgery due to primary rhegmatogenous retinal detachment with the macula on between 2009 and 2013 were included. Relevant factors analyzed included age, time delay until surgery, lens status, myopia, the detachment's location and configuration as well as number, size and type of retinal breaks. Eyes underwent optical coherence tomography to detect macular detachment. A multivariate analysis was performed to investigate the effect of several factors in the progression of retinal detachment. A total of 116 eyes of 116 patients were included. Mean time delay between admission and surgery was 1.8 ± 1.4 days. Progression was observed in 19.8% of the eyes. Of those, 47.8% presented macular detachment. Ten of the 11 (90.9%) eyes presenting progression involving the macula also exhibited a bullous configuration, which was the only parameter that correlated significantly with detachment progression in patients with (p = 0.0036) and without (p = 0.0014) macular involvement. For the first time in a prospective trial, a bullous configuration was found to be a highly significant predictor for progression in macula-on detachments. Our data support prompt surgery in patients diagnosed with bullous macula-on RRD.

Adverse drug events in the oral cavity.

Science.gov (United States)

Yuan, Anna; Woo, Sook-Bin

2015-01-01

Adverse reactions to medications are common and may have a variety of clinical presentations in the oral cavity. Targeted therapies and the new biologic agents have revolutionized the treatment of cancers, autoimmune diseases, and inflammatory and rheumatologic diseases but have also been associated with adverse events in the oral cavity. Some examples include osteonecrosis, seen with not only bisphosphonates but also antiangiogenic agents, and the distinctive ulcers caused by mammalian target of rapamycin inhibitors. As newer therapeutic agents are approved, it is likely that more adverse drug events will be encountered. This review describes the most common clinical presentations of oral mucosal reactions to medications, namely, xerostomia, lichenoid reactions, ulcers, bullous disorders, pigmentation, fibrovascular hyperplasia, white lesions, dysesthesia, osteonecrosis, infection, angioedema, and malignancy. Oral health care providers should be familiar with such events, as they will encounter them in their practice. Copyright © 2015 Elsevier Inc. All rights reserved.

Impetigo

Science.gov (United States)

... it look like it has been coated with honey or brown sugar. Bullous impetigo causes larger fluid- ... or showers regularly. Pay special attention to skin injuries (cuts, scrapes, bug bites, etc.), areas of eczema, ...

Comparative anatomy of leaves of Kalanchoe pinnata and K. crenata in sun and shade conditions, as a support for their identification

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Nattacha S. Moreira

2012-04-01

Full Text Available Kalanchoe pinnata (Lam. Pers. and K. crenata (Andrews Haw., Crassulaceae, are popularly used in the treatment of many diseases. Their biological activities, such as anti-leishmaniasis and analgesic, can be useful in phytotherapy. Both species are often misidentified as the other, because of their similar popular uses and names, and the similar external morphology of the leaves. We investigated the existence of anatomical characters that will permit correct identification of the species grown in shade and in sun conditions. We also contribute with new observations on the leaf anatomy of K. pinnata and K. crenata. Fixed (FAA70 leaves were used, and their sections were embedded in Leica historesin. Hydathodes were observed in both species, and for the first time were anatomically described in K. crenata. The species showed anatomical differences in relation to the presence of epidermal idioblasts only in K. crenata, the different pattern of distribution of subepidermal idioblasts, and the presence of leaf buds only in K. pinnata.

Comparative anatomy of leaves of Kalanchoe pinnata and K. crenata in sun and shade conditions, as a support for their identification

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Nattacha S. Moreira

2012-10-01

Full Text Available Kalanchoe pinnata (Lam. Pers. and K. crenata (Andrews Haw., Crassulaceae, are popularly used in the treatment of many diseases. Their biological activities, such as anti-leishmaniasis and analgesic, can be useful in phytotherapy. Both species are often misidentified as the other, because of their similar popular uses and names, and the similar external morphology of the leaves. We investigated the existence of anatomical characters that will permit correct identification of the species grown in shade and in sun conditions. We also contribute with new observations on the leaf anatomy of K. pinnata and K. crenata. Fixed (FAA70 leaves were used, and their sections were embedded in Leica historesin. Hydathodes were observed in both species, and for the first time were anatomically described in K. crenata. The species showed anatomical differences in relation to the presence of epidermal idioblasts only in K. crenata, the different pattern of distribution of subepidermal idioblasts, and the presence of leaf buds only in K. pinnata.

A novel mutation in the L12 domain of keratin 1 is associated with mild epidermolytic ichthyosis

NARCIS (Netherlands)

Bolling, M. C.; Bladergroen, R. S.; van Steensel, M. A. M.; Willemsen, M.; Jonkman, M. F.; van Geel, M.

P>Background Epidermolytic ichthyosis (EI), previously termed bullous congenital ichthyosiform erythroderma or epidermolytic hyperkeratosis, is a clinically heterogeneous genodermatosis caused by mutations in the genes encoding the suprabasal keratins 1 and 10. Classical EI is clinically

'An eye, for eyes - mission' - From dream to reality

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Parikumar P

2013-10-01

Full Text Available Corneal transplantation has been in routine practice to treat corneal endothelial diseases like Bullous Keratopathy, in which either the whole cornea or the partial cornea (the endothelium alone is transplanted from the cadaver donor to the recipient with the endothelial disease [1]. In whole corneal or partial corneal transplant one cadaver donor's cornea can be used to treat one recipient cornea only, which leads to a huge global shortage of donor corneas [2]. At this juncture Yokoo et al isolated and expanded corneal endothelial precursors using the sphere forming assay in vitro [3] and demonstrated the in vivo transplantation of these corneal endothelial precursors in a rabbit model of bullous keratopathy [4]. Following this, we studied the transportation of cadaver donor derived corneal endothelial tissue (CET from human cadaver donors in a thermoreversible gelation polymer (TGP (4 based transportation cocktail without cool preservation and demonstrated the viability of human corneal endothelial precursor (HCEP cells isolated from these CETs even after 72 hours of transportation without cool preservation [5]. This was done to suit the conditions existing in developing nations like India where hospitals might be located far from eye banks and maintaining cold chain preservation is relatively difficult. Further, these HCEPs were expanded in vitro using a polymer based expansion protocol [5]. This was the first step in the realisation of the dream of 'Eye for eyes' in a manner suitable for Indian conditions. Corneal transplantation has been in routine practice to treat corneal endothelial diseases like Bullous Keratopathy, in which either the whole cornea or the partial cornea (the endothelium alone is transplanted from the cadaver donor to the recipient with the endothelial disease [1]. In whole corneal or partial corneal transplant one cadaver donor's cornea can be used to treat one recipient cornea only, which leads to a huge global

IN SITU IMMUNE RESPONSE EVALUATION VIA IMMUNOHISTOCHEMISTRY IN SKIN BIOPSIES FROM PATIENTS AFFECTED BY AUTOIMMUNE BLISTERING DISEASES

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Ana Maria Abreu Velez

2013-11-01

Full Text Available Introduction: The in situ immune response in skin biopsies from patients affected by autoimmune skin blistering diseases (ABD is not well characterized. Aim: Our investigation attempts to immunophenotype cells in lesional skin in several ABD, utilizing immunohistochemistry (ICH. Methods: We tested by IHC for CD4, CD8, CD19, CD20, CD45, CD56/NCAM, PAX-5, granzyme B, myeloperoxidase, neutrophil elastase, LAT and ZAP-70 in patients affected by ABD. We tested 30 patients with endemic pemphigus foliaceus (EPF, 15 controls from the EPF endemic area, and 15 biopsies from healthy controls from the USA. We also tested archival biopsies from patients with selected ABD, including 30 patients with bullous pemphigoid, 20 with pemphigus vulgaris, 8 with pemphigus foliaceus and 14 with dermatitis herpetiformis. Results: We found a predominantly CD8 positive/CD45 positive T cell infiltrate in all ABD. Our skin biopsies demonstrated consistently positive staining for myeloperoxidase, but negative staining for neutrophil elastase. Most ABD biopsies displayed negative staining for CD4 and B cell markers; natural killer cell markers were also rarely seen. ZAP-70 and LAT were frequently detected. In El Bagre-EPF, a significant fragmentation of T cells in lesional skin was noted, as well as autoreactivity to lymph nodes. Conclusions: The documented T cell and myeloperoxidase staining are indicative of the role of T lymphocytes and neutrophils in lesional biopsies in patients with ABD, in addition to previously documented deposition of B cells, immunoglobulins and complement in situ. In El Bagre-EPF, T cells could also target lymph nodes; however, further studies are needed to confirm this possibility.

Degree of skin denervation and its correlation to objective thermal sensory test in leprosy patients.

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Ismael Alves Rodrigues Júnior

Full Text Available BACKGROUND: Leprosy is an infectious disease affecting skin and peripheral nerves resulting in increased morbidity and physical deformities. Early diagnosis provides opportune treatment and reduces its complications, relying fundamentally on the demonstration of impaired sensation in suggestive cutaneous lesions. The loss of tactile sensitivity in the lesions is preceded by the loss of thermal sensitivity, stressing the importance of the thermal test in the suspicious lesions approach. The gold-standard method for the assessment of thermal sensitivity is the quantitative sensory test (QST. Morphological study may be an alternative approach to access the thin nerve fibers responsible for thermal sensitivity transduction. The few studies reported in leprosy patients pointed out a rarefaction of thin dermo-epidermal fibers in lesions, but used semi-quantitative evaluation methods. METHODOLOGY/PRINCIPAL FINDINGS: This work aimed to study the correlation between the degree of thermal sensitivity impairment measured by QST and the degree of denervation in leprosy skin lesions, evaluated by immunohistochemistry anti-PGP 9.5 and morphometry. Twenty-two patients were included. There were significant differences in skin thermal thresholds among lesions and contralateral skin (cold, warm, cold induced pain and heat induced pain. The mean reduction in the density of intraepidermal and subepidermal fibers in lesions was 79.5% (SD = 19.6 and 80.8% (SD = 24.9, respectively. CONCLUSIONS/SIGNIFICANCE: We observed a good correlation between intraepidermal and subepidermal fibers deficit, but no correlation between these variables and those accounting for the degree of impairment in thermal thresholds, since the thin fibers rarefaction was homogeneously intense in all patients, regardless of the degree of sensory deficit. We believe that the homogeneously intense denervation in leprosy lesions should be objective of further investigations focused on its

Genetic reversion of inherited skin disorders

Energy Technology Data Exchange (ETDEWEB)

Magnaldo, Thierry; Sarasin, Alain

2002-11-30

Human epidermis is a squamous stratified epithelium whose integrity relies on balanced processes of cell attachment, proliferation, and differentiation. In monogenic skin dermatoses, such as mecano-bullous diseases, or DNA repair deficiencies such as the xeroderma pigmentosum (XP), alterations of skin integrity may have devastating consequences as illustrated by the extremely high epidermal cancer proneness of XP patients. The lack of efficient pharmacological treatments, the easy accessibility of skin, and the possibility of long term culture and genetic manipulations ex vivo of epidermal keratinocytes, have encouraged approaches toward gene transfer and skin therapy prospects. We review here some of the human genetic disorders that exhibit major traits in skin, as well as requirements and difficulties inherent to approaches aimed at stable phenotypic correction.

Association Between Inflammatory Skin Disease and Cardiovascular and Cerebrovascular Co-Morbidities in US Adults: Analysis of Nationwide Inpatient Sample Data.

Science.gov (United States)

Kwa, Michael C; Silverberg, Jonathan I

2017-12-01

Psoriasis, atopic dermatitis or eczema (AD-E), pemphigus, bullous pemphigoid (BP), and hidradenitis are chronic inflammatory skin disorders associated with systemic immune activation, considerable symptom burden, stigma, functional disturbances, and mental health symptoms. All of these might increase cardiovascular risk. The objective of this study was to determine whether these inflammatory skin diseases are associated with increased cardiovascular/cerebrovascular risk and/or disease. We analyzed data from the 2002-2012 National Inpatient Sample, including a representative 20% sample of all US hospitalizations (n = 72,108,077 adults). In multivariate logistic regression models with propensity score matching, patients hospitalized with versus without a diagnosis the inflammatory skin diseases examined had higher odds of obesity (odds ratio [95% confidence interval] for pemphigus: 1.16 [1.05-1.29]; BP 1.14 [1.06-1.23]; AD-E: 1.82 [1.79-1.86]; psoriasis: 2.36 [2.32-2.41]; hidradenitis: 2.79 [2.59-3.01]). Inflammatory skin disease was also associated with significantly higher odds of different cardiovascular risk factors, including hypertension (pemphigus: 1.39 [1.31-1.48]; BP 1.96 [1.88-2.05]; AD-E: 1.19 [1.17-1.21]; psoriasis: 1.61 [1.59-1.64]), and diabetes mellitus with complications (pemphigus: 1.34 [1.18-1.52]; BP: 2.06 [1.90-2.24]; AD-E: 1.13 [1.10-1.17]; psoriasis: 1.39 [1.35-1.44]), as well as vascular, cardiovascular, and cerebrovascular disease, including peripheral vascular disease (pemphigus: 1.14 [1.00-1.30]; BP: 1.83 [1.69-1.98]; AD-E: 1.18 [1.14-1.22]; psoriasis: 1.32 [1.28-1.35]), peripheral and visceral atherosclerosis (BP: 1.67 [1.53-1.81]; AD-E: 1.16 [1.12-1.20]; psoriasis: 1.27 [1.24-1.30]), pulmonary circulation disorders (pemphigus: 1.67 [1.39-2.01]; BP: 2.17 [1.92-2.45]; AD-E: 1.39 [1.33-1.45]; psoriasis: 1.37 [1.31-1.43]), congestive heart failure (pemphigus: 1.75 [1.60-1.90]; BP: 2.82 [2.68-2.98]; AD-E: 1.10 [1.07-1.13]; psoriasis: 1.05 [1

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

NARCIS (Netherlands)

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

2015-01-01

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

Clinical Immunology Review Series: an approach to the patient with recurrent orogenital ulceration, including Behçet's syndrome.

LENUS (Irish Health Repository)

Keogan, M T

2009-04-01

Patients presenting with recurrent orogenital ulcers may have complex aphthosis, Behçet\\'s disease, secondary complex aphthosis (e.g. Reiter\\'s syndrome, Crohn\\'s disease, cyclical neutropenia) or non-aphthous disease (including bullous disorders, erythema multiforme, erosive lichen planus). Behçet\\'s syndrome is a multi-system vasculitis of unknown aetiology for which there is no diagnostic test. Diagnosis is based on agreed clinical criteria that require recurrent oral ulcers and two of the following: recurrent genital ulcers, ocular inflammation, defined skin lesions and pathergy. The condition can present with a variety of symptoms, hence a high index of suspicion is necessary. The most common presentation is with recurrent mouth ulcers, often with genital ulcers; however, it may take some years before diagnostic criteria are met. All patients with idiopathic orogenital ulcers should be kept under review, with periodic focused assessment to detect evolution into Behçet\\'s disease. There is often a delay of several years between patients fulfilling diagnostic criteria and a diagnosis being made, which may contribute to the morbidity of this condition. Despite considerable research effort, the aetiology and pathogenesis of this condition remains enigmatic.

Severity of nonbullous Staphylococcus aureus impetigo in children is associated with strains harboring genetic markers for exfoliative toxin B, Panton-Valentine leukocidin, and the multidrug resistance plasmid pSK41

NARCIS (Netherlands)

S. Koning (Sander); A.F. van Belkum (Alex); S.V. Snijders (Susan); J.L. Nouwen (Jan); M. op 't Veld; J.C. van der Wouden (Hans); C.M. Verduin (Cees); H.A. Verbrugh (Henri); W.B. van Leeuwen (Willem); L.W.A. van Suijlekom-Smit (Lisette)

2003-01-01

textabstractNonbullous impetigo is a common skin infection in children and is frequently caused by Staphylococcus aureus. Staphylococcal toxins and especially exfoliative toxin A are known mediators of bullous impetigo in children. It is not known whether this is also true for

Changing indications and techniques for corneal transplantations at a tertiary referral center in Turkey, from 1995 to 2014

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Altay Y

2016-06-01

Full Text Available Yesim Altay, Ayse Burcu, Gozde Aksoy, Evin Singar Ozdemir, Firdevs Ornek Department of Ophthalmology, Ankara Training and Research Hospital, Ankara, Turkey Background: Indications for corneal transplantation in developed and developing nations differ according to the different spectrum of corneal disease in each country. Objective: The purpose of this study is to analyze the changing indications and surgical techniques for corneal transplantation over the past 20 years at a tertiary referral center in Turkey. Methods: We retrospectively reviewed the records of patients who underwent keratoplasty from January 1995 to December 2014 (between 1995 and 2004, period 1, and between 2005 and 2014, period 2. Patients’ demographic data, indications for corneal transplantation, and the type of surgery were recorded. Results: The number of keratoplasties performed ranged from 548 in period 1 to 782 in period 2. Between 1995 and 2004, the leading indications were keratoconus (34.1%, bullous keratopathy (17%, and non-herpetic corneal scar (13.3%, and between 2005 and 2014, they were keratoconus (33.8%, corneal stromal dystrophy (14.2%, and bullous keratopathy (12.7%. All the keratoplasties performed in the 1995–2004 period were penetrating keratoplasty (PKP. During the period 2005–2014, PKP accounted for 93%, automated lamellar keratoplasty 5.8%, and deep anterior lamellar keratoplasty 1.2% of all corneal transplantations. Conclusion: Keratoconus was the leading indication for keratoplasty in both periods. In the 2005–2014 period, corneal stromal dystrophy increased significantly. All the keratoplasties performed in period 1 and 93% of all keratoplasties performed in period 2 were PKP. Keywords: corneal transplantation, indication, keratoconus, keratoplasty technique, pene­trating keratoplasty

Histopathological detection of entry and exit holes in human skin wounds caused by firearms.

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Baptista, Marcus Vinícius; d'Ávila, Solange C G P; d'Ávila, Antônio Miguel M P

2014-07-01

The judiciary needs forensic medicine to determine the difference between an entry hole and an exit hole in human skin caused by firearms for civilian use. This important information would be most useful if a practical and accurate method could be done with low-cost and minimal technological resources. Both macroscopic and microscopic analyses were performed on skin lesions caused by firearm projectiles, to establish histological features of 14 entry holes and 14 exit holes. Microscopically, in the abrasion area macroscopically observed, there were signs of burns (sub-epidermal cracks and keratinocyte necrosis) in the entrance holes in all cases. These signs were not found in three exit holes which showed an abrasion collar, nor in other exit holes. Some other microscopic features not found in every case were limited either to entry holes, such as cotton fibres, grease deposits, or tattooing in the dermis, or to exit holes, such as adipose tissue, bone or muscle tissue in the dermis. Coagulative necrosis of keratinocytes and sub-epidermal cracks are characteristic of entry holes. Despite the small sample size, it can be safely inferred that this is an important microscopic finding, among others less consistently found, to define an entry hole in questionable cases. Copyright © 2014 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

The Pea SAD Short-Chain Dehydrogenase/Reductase: Quinone Reduction, Tissue Distribution, and Heterologous Expression1[W][OA

Science.gov (United States)

Scherbak, Nikolai; Ala-Häivälä, Anneli; Brosché, Mikael; Böwer, Nathalie; Strid, Hilja; Gittins, John R.; Grahn, Elin; Eriksson, Leif A.; Strid, Åke

2011-01-01

The pea (Pisum sativum) tetrameric short-chain alcohol dehydrogenase-like protein (SAD) family consists of at least three highly similar members (SAD-A, -B, and -C). According to mRNA data, environmental stimuli induce SAD expression. The aim of this study was to characterize the SAD proteins by examining their catalytic function, distribution in pea, and induction in different tissues. In enzyme activity assays using a range of potential substrates, the SAD-C enzyme was shown to reduce one- or two-ring-membered quinones lacking long hydrophobic hydrocarbon tails. Immunological assays using a specific antiserum against the protein demonstrated that different tissues and cell types contain small amounts of SAD protein that was predominantly located within epidermal or subepidermal cells and around vascular tissue. Particularly high local concentrations were observed in the protoderm of the seed cotyledonary axis. Two bow-shaped rows of cells in the ovary and the placental surface facing the ovule also exhibited considerable SAD staining. Ultraviolet-B irradiation led to increased staining in epidermal and subepidermal cells of leaves and stems. The different localization patterns of SAD suggest functions both in development and in responses to environmental stimuli. Finally, the pea SAD-C promoter was shown to confer heterologous wound-induced expression in Arabidopsis (Arabidopsis thaliana), which confirmed that the inducibility of its expression is regulated at the transcriptional level. PMID:21343423

The pea SAD short-chain dehydrogenase/reductase: quinone reduction, tissue distribution, and heterologous expression.

Science.gov (United States)

Scherbak, Nikolai; Ala-Häivälä, Anneli; Brosché, Mikael; Böwer, Nathalie; Strid, Hilja; Gittins, John R; Grahn, Elin; Eriksson, Leif A; Strid, Åke

2011-04-01

The pea (Pisum sativum) tetrameric short-chain alcohol dehydrogenase-like protein (SAD) family consists of at least three highly similar members (SAD-A, -B, and -C). According to mRNA data, environmental stimuli induce SAD expression. The aim of this study was to characterize the SAD proteins by examining their catalytic function, distribution in pea, and induction in different tissues. In enzyme activity assays using a range of potential substrates, the SAD-C enzyme was shown to reduce one- or two-ring-membered quinones lacking long hydrophobic hydrocarbon tails. Immunological assays using a specific antiserum against the protein demonstrated that different tissues and cell types contain small amounts of SAD protein that was predominantly located within epidermal or subepidermal cells and around vascular tissue. Particularly high local concentrations were observed in the protoderm of the seed cotyledonary axis. Two bow-shaped rows of cells in the ovary and the placental surface facing the ovule also exhibited considerable SAD staining. Ultraviolet-B irradiation led to increased staining in epidermal and subepidermal cells of leaves and stems. The different localization patterns of SAD suggest functions both in development and in responses to environmental stimuli. Finally, the pea SAD-C promoter was shown to confer heterologous wound-induced expression in Arabidopsis (Arabidopsis thaliana), which confirmed that the inducibility of its expression is regulated at the transcriptional level.

Chloroplasts in anther endothecium of Zea mays (Poaceae).

Science.gov (United States)

Murphy, Katherine M; Egger, Rachel L; Walbot, Virginia

2015-11-01

Although anthers of Zea mays, Oryza sativa, and Arabidopsis thaliana have been studied intensively using genetic and biochemical analyses in the past 20 years, few updates to anther anatomical and ultrastructural descriptions have been reported. For example, no transmission electron microscopy (TEM) images of the premeiotic maize anther have been published. Here we report the presence of chloroplasts in maize anthers. TEM imaging, electron acceptor photosynthesis assay, in planta photon detection, microarray analysis, and light and fluorescence microscopy were used to investigate the presence of chloroplasts in the maize anther. Most cells of the maize subepidermal endothecium have starch-containing chloroplasts that do not conduct measurable photosynthesis in vitro. The maize anther contains chloroplasts in most subepidermal, endothecial cells. Although maize anthers receive sufficient light to photosynthesize in vivo and the maize anther transcribes >96% of photosynthesis-associated genes found in the maize leaf, no photosynthetic light reaction activity was detected in vitro. The endothecial cell layer should no longer be defined as a complete circle viewed transversely in anther lobes, because chloroplasts are observed only in cells directly beneath the epidermis and not those adjacent to the connective tissue. We propose that chloroplasts be a defining characteristic of differentiated endothecial cells and that nonsubepidermal endothecial cells that lack chloroplasts be defined as a separate cell type, the interendothecium. © 2015 Botanical Society of America.

The influence of maize kernel moisture on the sterilizing effect of gamma rays

International Nuclear Information System (INIS)

Khanymova, T.; Poloni, E.

1980-01-01

The influence of 4 levels of maize kernel moisture (16, 20, 25 and 30%) on gamma-ray sterilizing effect was studied and the after-effect of radiation on the microorganisms at short term storage was followed up. Maize kernels of the hybrid Knezha-36 produced in 1975 were used. Gamma-ray treatment of the kernels was effected by GUBEh-4000 irradiator at doses of 0.2 and 0.3 Mrad and after that they were stored for a month at 12 deg and 25 deg C and controlled moisture conditions. Surface and subepidermal infection of the kernels was determined immediately post irradiation and at the end of the experiment. Non-irradiated kernels were used as controls. Results indicated that the initial kernel moisture has a considerable influence on the sterilizing effect of gamma-rays at the rates used in the experiment and affects to a considerable extent the post-irradiation recovery of organisms. The speed of recovery was highest in the treatment with 30% moisture and lowest in the treatment with 16% kernel moisture. Irradiation of the kernels causes pronounced changes on the surface and subepidermal infection. This was due to the unequal radio resistance to the microbial components and to the modifying effect of the moisture holding capacity. The useful effect of maize kernel irradiation was more prolonged at 12 deg C than at 25 deg C

Congenital anomalies in black South African liveborn neonates at ...

African Journals Online (AJOL)

hypospadias the commonest urogenital anomalies (Table VI). The incidence of cleft lip/palate was only 0,23 per 1 000. Iivebirths (Table V). The integumentary conditions seen included congenital ichthyosis, epidermolysis bullosa, both disorders of. Mendelian inheritance, an unclassifiable bullous dermatitis and a naevus.

A survey of poplar (populus nigra rust and identification of fungal agent species with conventional and molecular approaches in Maragheh area of Iran.

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Seyed Mohsen Damadi

2014-12-01

Full Text Available In a survey for rust diseases in Maragheh area rust symptoms were observed on poplars (Populus nigra in Maragheh city parks and orchards near the city. Uredinia and urediniospores typical of Melampsora were present on the underside of leaves. Paraphyses were clavate with walls evenly thick and Telia, formed on the leaves early in the autumn, were epiphyllous and subepidermal. DNA was extracted from urediniospores and the primers ITS1 and ITS4 were used to amplify the internal transcribed spacer region of the ribosomal DNA. Based on the key provided by Bagyanarayana and the species description by Pei and Shang, and the result of sequencing, the causal agent was identified as Melampsora allii-populina Kelb. Melampsora species are mainly determined based on their morphology, alternate hosts and telial host range. However, in most cases, only one or two spore stages could be found at the time of observation and there is no information of the alternate hosts. This is the first study of poplar rust disease to the species level in the area. Rust disease is likely to be the most important disease on poplar in this area. As poplar rust can cause severe damages to nursery plants and young trees, there must be further research to understand the epidemiology of the rust disease. A key question to be answered is whether the rust goes through a full life-cycle, possible via known alternate host Allium spp. or only uredinial and telial stages are present in the studied area.

[Evaluation of Iris Morphology Viewed through Stromal Edematous Corneas by Infrared Camera].

Science.gov (United States)

Kobayashi, Masaaki; Morishige, Naoyuki; Morita, Yukiko; Yamada, Naoyuki; Kobayashi, Motomi; Sonoda, Koh-Hei

2016-02-01

We reported that the application of infrared camera enables us to observe iris morphology in Peters' anomaly through edematous corneas. To observe the iris morphology in bullous keratopathy or failure grafts with an infrared camera. Eleven bullous keratopathy or failure grafts subjects (6 men and 5 women, mean age ± SD; 72.7 ± 13.0 years old) were enrolled in this study. The iris morphology was observed by applying visible light mode and near infrared light mode of infrared camera (MeibomPen). The detectability of pupil shapes, iris patterns and presence of iridectomy was evaluated. Infrared mode observation enabled us to detect the pupil shapes in 11 out of 11 cases, iris patterns in 3 out of 11 cases, and presence of iridetomy in 9 out of 11 cases although visible light mode observation could not detect any iris morphological changes. Applying infrared optics was valuable for observation of the iris morphology through stromal edematous corneas.

Occurence of Cryptocline taxicola (All. Petr. on needles of Taxus spp. – symptoms and morphological features

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Ivana Šafránková

2008-01-01

Full Text Available In comparison with other European trees species Taxus spp. is not often prone to phytopathological problems. The fungus Cryptocline taxicola (All. Petr was found on needles of cultivars of Yet (T. baccata L., T. cuspidata S. & Z., Taxus × media Rehd. in Brno in the summer and the autumn of 2007. The article attemps to describe the disease symptoms and morphological features. Infected needles and whole shoots turn brown. Fruiting bodies were observed on upper and lower surfaces necrotic and chlorotic needles. Acervuli of the fungus were black, subepidermal, ∅ 160–370 × 90–145 μm, surrounded by brown circles epidermis, and the epidermis ruptures as they brake through. Conidia were smooth-walled and hyaline, without septa, ellipsoid to oval, 10–18 × 4,5–8,4 μm. Conidiophores were hyaline, cylindrical, with phyalides, 12–18 × 2,8–3,4 μm. The fungus forms dark colonies wish greyish aerial mycelium on potato-dextrose agar (PDA.

Dermatitis herpetiformis misdiagnosed and treated as tinea cutis glabrae

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Marta Stawczyk-Macieja

2016-02-01

Full Text Available Introduction . Dermatitis herpetiformis is a rare bullous disorder. Autoimmunological disturbances associated with hypersensitivity to gluten play the main role in the etiopathogenesis of the disease. Clinical manifestations include polymorphic skin lesions which may cause diagnostic difficulties. Objective. To present a case of dermatitis herpetiformis diagnosed and treated without any clinical improvement as tinea cutis glabrae. Case report . A 20-year-old male patient was admitted for the evaluation of polymorphic skin lesions of 7-month duration distributed symmetrically on the skin of elbows, knees, face and the gluteal and sacral region. The patient was previously treated with systemic and topical antimycotic drugs. Due to a typical distribution of skin lesions as well as symptoms reported by the patient, we started to suspect Duhring’s disease. The diagnosis was confirmed by direct immunofluorescence of skin biopsy and serological tests. Systemic treatment with dapsone and a gluten-free diet led to clinical improvement. Conclusions . The morphology and localization of skin lesions in Duhring’s disease may be similar to the clinical presentation of fungal infection of the skin, which in equivocal cases should be excluded by a mycological test.

Radiographic and radionuclide lung perfusion imaging in healthy calves and calves naturally infected with bovine respiratory syncytial virus

International Nuclear Information System (INIS)

Verhoeff, J.; Brom, W.E. van den; Ingh, T.S.G.A.M. van den

1992-01-01

Nine calves between three and 18 weeks old with serologically confirmed natural bovine respiratory syncytial virus infection were examined clinically, radiographically and by radionuclide lung perfusion imaging. The results were compared with those from seven healthy calves. The diseased calves were euthanased and examined pathologically, virologically and bacteriologically. The clinical signs indicated that the disease was in an acute stage. Radiography of the diseased animals revealed cysts, corresponding morphologically with bullous emphysema, and infiltrations roughly corresponding in distribution with atelectatic and, or, pneumonic areas. Radionuclide lung perfusion imaging revealed no perfusion shifts between the left and right lungs and a normal perfusion pattern in five of the nine diseased calves. The abnormalities in the perfusion patterns of three calves were probably caused by anatomical disorders such as cysts and pleural adhesions, but no cause of the abnormality could be found in one calf. These findings suggest that in calves infected with bovine respiratory syncytial virus, the normal perfusion pattern is maintained until anatomical disorders occur. The pathological examination and radiography revealed that the cranioventral lung fields were particularly poorly ventilated. This finding and the normal perfusion pattern indicate that these parts of the lungs are probably the sites where shuntings and perfusion-ventilation mismatchings occur

Clinical optical coherence tomography combined with multiphoton tomography for evaluation of several skin disorders

Science.gov (United States)

König, Karsten; Speicher, Marco; Bückle, Rainer; Reckfort, Julia; McKenzie, Gordon; Welzel, Julia; Koehler, Martin J.; Elsner, Peter; Kaatz, Martin

2010-02-01

The first clinical trial of optical coherence tomography (OCT) combined with multiphoton tomography (MPT) and dermoscopy is reported. State-of-the-art (i) OCT systems for dermatology (e.g. multibeam swept source OCT), (ii) the femtosecond laser multiphoton tomograph DermaInspectTM, and (iii) digital dermoscopes were applied to 47 patients with a diversity of skin diseases and disorders such as skin cancer, psoriasis, hemangioma, connective tissue diseases, pigmented lesions, and autoimmune bullous skin diseases. Dermoscopy, also called 'epiluminescent microscopy', provides two-dimensional color images of the skin surface. OCT imaging is based on the detection of optical reflections within the tissue measured interferometrically whereas nonlinear excitation of endogenous fluorophores and the second harmonic generation are the bases of MPT images. OCT cross sectional "wide field" image provides a typical field of view of 5 x 2 mm2 and offers fast information on the depth and the volume of the investigated lesion. In comparison, multiphoton tomography presents 0.36 x 0.36 mm2 horizontal or diagonal sections of the region of interest within seconds with submicron resolution and down to a tissue depth of 200 μm. The combination of OCT and MPT provides a synergistic optical imaging modality for early detection of skin cancer and other skin diseases.

Trauma-induced pemphigus: a case series of 36 patients.

Science.gov (United States)

Daneshpazhooh, Maryam; Fatehnejad, Mina; Rahbar, Ziba; Balighi, Kamran; Ghandi, Narges; Ghiasi, Maryam; Abedini, Robabeh; Lajevardi, Vahideh; Chams-Davatchi, Cheyda

2016-02-01

Pemphigus is a group of autoimmune diseases characterized by intraepidermal acantholytic blisters. Isomorphic responses, or Koebner phenomenon (KP), defined as the appearance of typical lesions of a disease following trauma are rarely reported in pemphigus. Our aim was to present patients who developed new pemphigus lesions as a result of skin trauma. The medical files of pemphigus patients from the Autoimmune Bullous Diseases Research Center, who had a history of trauma before the onset or flare of their disease, between 1999 and 2013 were reviewed. Thirty-six pemphigus vulgaris (PV) patients had a history of trauma. Thirteen patients developed new-onset PV and the other 23 had previously been diagnosed with PV. Pemphigus lesions developed most often following major surgeries including abdominal, orthopedic, and chest surgeries as well as dental procedures, blunt physical trauma, and skin surgeries. Moreover, post-cataract laser surgery, burns, radiation therapy, and physiotherapy were also shown to induce pemphigus. Mean time between trauma and lesions was 4.7 weeks for recurrent PV and 15.0 weeks for new-onset PV. Unnecessary surgery and blunt trauma should be avoided in pemphigus patients. Furthermore, posttraumatic pemphigus should be suspected in poorly healing surgical wounds and confirmatory biopsies are mandatory. © 2016 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.

Effect of cannabis smoking on lung function and respiratory symptoms: a structured literature review

Science.gov (United States)

Ribeiro, Luis IG; Ind, Philip W

2016-01-01

As cannabis use increases, physicians need to be familiar with the effects of both cannabis and tobacco on the lungs. However, there have been very few long-term studies of cannabis smoking, mostly due to legality issues and the confounding effects of tobacco. It was previously thought that cannabis and tobacco had similar long-term effects as both cause chronic bronchitis. However, recent large studies have shown that, instead of reducing forced expiratory volume in 1 s and forced vital capacity (FVC), marijuana smoking is associated with increased FVC. The cause of this is unclear, but acute bronchodilator and anti-inflammatory effects of cannabis may be relevant. Bullous lung disease, barotrauma and cannabis smoking have been recognised in case reports and small series. More work is needed to address the effects of cannabis on lung function, imaging and histological changes. PMID:27763599

Application of Reflectance Confocal Microscopy in Dermatology Practice

Directory of Open Access Journals (Sweden)

Ayşe Esra Koku Aksu

2015-03-01

Full Text Available In vivo reflectance confocal microscopy (RCM is a non-invasive method, imaging cellular structures in living skin at a level close to the histological resolution. It is easier to diagnose melanocytic and non-melanocytic skin tumors especially in difficult cases when RCM features have been identified. Determination of the cellular features, presence of cellular and structural atypia with RCM allows the discrimination of benign and malignant lesions. Preoperative differential diagnosis of malignant lesions, determining preoperative lesion borders in complicated cases, identification of local recurrence after excision of malignant lesions, monitoring the treatment efficacy in patients using topical treatment and who can not be operated, are the main areas of RCM in tumoral lesions. Besides, RCM is helpful in the establishing the diagnosis of inflammatory disease like psoriasis, contact dermatitis, lichen planus and in evaluation of therapeutic efficacy, detecting of infestation like tinea, skabiyes, demodicosis and determining the level of bullae in bullous disease. Due to being noninvasive, RCM is preferred in cosmetology, in clinical research and practice for the evaluation of the effectiveness of cosmetic products and cosmetic procedures.

Prevalence of oral lichen planus in Iranian children and adolescents: a 12-year retrospective study.

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Bakhtiari, S; Taheri, J B; Toossi, P; Azimi, S; Kawosi Nezhad, Sh

2017-12-01

To investigate the prevalence of oral lichen planus in patients younger than 18 years, referred to a dermatology centre in Iran during 2002-2014. Lichen planus is a chronic inflammatory, immune-mediated disease that could affect the oral mucosa and is a pre-cancerous condition. The disease usually develops in middle age with female predominance and is rare in children. In this retrospective study, cases with definitive histopathologic diagnosis of lichen planus, over a 12-year period from 2002 to 2014 from a dermatologic hospital archive were evaluated. The prevalence of both cutaneous and oral lichen planus, the male:female ratio and site of involvement were calculated using SPSS version 21. Thirty-six of 564 patients younger than 18 years old diagnosed with lichen planus. Two females (0.4%) had oral lichen planus. One patient had erosive, and one had bullous, oral lichen planus. Oral lichen planus had a very low frequency in Iranian population younger than 18 years old, identifying these patients is recommended for long-term follow-up.

Combined treatment with immunoadsorption and rituximab leads to fast and prolonged clinical remission in difficult-to-treat pemphigus vulgaris.

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Behzad, M; Möbs, C; Kneisel, A; Möller, M; Hoyer, J; Hertl, M; Eming, R

2012-04-01

Pemphigus vulgaris (PV) is a potentially life-threatening autoimmune bullous disorder which is characterized by blisters and erosions of the skin and mucous membranes. A frequently applied first-line therapy for PV consists of systemic corticosteroids (CS) combined with immunosuppressive agents. In refractory cases, novel therapeutic strategies such as immunoadsorption (IA) and the anti-CD20 antibody rituximab (Rtx) aim at directly interfering with pathogenic autoantibodies (auto-Abs). To investigate the long-term efficacy of IA in combination with Rtx in patients with difficult-to-treat PV, we assessed the clinical response to treatment by monitoring the Autoimmune Bullous Skin Disorder Intensity Score, IgG auto-Abs against desmoglein 1 and 3 (Dsg1 and Dsg3) and the dose of systemic CS. We retrospectively analysed clinical and serological parameters of 10 patients with difficult-to-treat PV who received IA at 4-week intervals, followed by Rtx either twice at 1000 mg or four times at 375mg m(-2) . During a 12-month follow-up period, CS were tapered according to the individual clinical status. Six months after the first IA treatment eight of 10 patients were in complete remission on therapy while one patient showed a partial response and one patient was unresponsive to the treatment. At 12 months, six of eight patients were in complete remission on therapy, one patient showed stable disease and one patient had relapsed. Overall, anti-Dsg3 IgG and anti-Dsg1 IgG auto-Abs correlated well with the clinical activity and systemic CS were tapered gradually. The present findings show that the combination of IA and Rtx induces rapid clinical remission and long-term control in difficult-to-treat pemphigus. © 2011 The Authors. BJD © 2011 British Association of Dermatologists.

Common pediatric and adolescent skin conditions.

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Sanfilippo, Angela M; Barrio, Victoria; Kulp-Shorten, Carol; Callen, Jeffrey P

2003-10-01

Skin lesions are encountered in all areas of medicine, and it is therefore important for physicians to understand the fundamentals of explaining and diagnosing common skin conditions. This article begins with a discussion of description and documentation of skin lesions based on color, size, morphology, and distribution. Pigmentation disorders such as vitiligo are depicted. Cutaneous growths that are found in the pediatric and adolescent population include acrochordons, dermatofibromas, keloids, milia, neurofibromas, and pyogenic granulomas. Treatment of these growths usually involves observation or curettage with electrodessication.Psoriasis, atopic dermatitis, poison ivy, and eczema are comprised of scaling patches and plaques; poison ivy and atopic dermatitis may also present with bullous and vesicular changes. Therapy typically consists of topical emollients and corticosteroids; phototherapy is reserved for refractory cases. Acne vulgaris is the most common skin disease of the pediatric and adolescent population. This condition can be psychologically debilitating and, therefore, proper treatment is of paramount importance. Therapeutic options include topical as well as oral antibiotics and retinoids. Extreme caution must be used when prescribing retinoids to post-pubescent females, as these agents are teratogenic. Vascular anomalies are most commonly exemplified as port wine stains and hemangiomas. Port wine stains may be treated with pulsed dye laser or may be observed if they are not of concern to the patient or physician. Hemangiomas typically spontaneously regress by age ten; however, there has been recent concern that certain cases may need to be treated. Dermal rashes may be localized or generalized. Treatment of generalized drug eruptions involves elimination of the inciting agent, topical antipruritics, and systemic corticosteroids for severe reactions. Infectious etiologic agents of skin disease include bacteria, fungi, and viruses. Many sexually

Cardiomyopathy in Patients With Hereditary Bullous Epidermolysis.

Science.gov (United States)

Batalla, A; Vicente, A; Bartrons, J; Prada, F; Fortuny, C; González-Enseñat, M A

In recent decades, an association has been reported between epidermolysis bullosa (EB) and dilated cardiomyopathy (DC). DC is typically in an advanced phase when detected, leading to a poorer prognosis. Our objective was to determine the prevalence of DC in patients with EB seen in Hospital San Joan de Déu in Barcelona, Spain, between May 1986 and April 2015. This was a descriptive, cross-sectional chart-review study in which we recorded the type and main subtypes of EB and the presence or absence of DC. Fifty-seven patients with EB were found, 19 with EB simplex, 10 with junctional EB, 27 with dystrophic EB (14 dominant dystrophic and 13 recessive dystrophic), and just 1 with Kindler syndrome. DC was detected in only 2 patients with recessive dystrophic EB. Twenty-three patients had presented factors that could have had a causal relationship with the potential onset of DC. DC is a possible complication of EB, particularly in recessive dystrophic EB. Periodic follow-up should be performed to make an early diagnosis and start treatment. Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

The essential role of locoregional radiotherapy in the management of Merkel cell tumor

International Nuclear Information System (INIS)

Maeurer, J.; Busch, M.; Matthaei, D.; Duehmke, E.; Bergholz, M.; Halling, F.

1992-01-01

The Merkel cell tumor is becoming an increasingly diagnosed primary neoplasma of the skin. This subepidermal tumor is commonly located on the head and neck or extremities of elderly patients. Occassionally misinterpreted as cutaneous metastases, they show a high rate of lokal recurrence (27 to 52%) and distant metastatic spread (18 to 52%). The definitive diagnosis can be made with immunohistochemistry. Wide surgical excision with postoperative irradiation to the local site and regional lymphatics is the therapy of choice. In seven patients we describe management strategies and discuss their clinical results. (orig.) [de

Concurrent Presentation of Erythrodermic Lichen Planus and Squamous Cell Carcinoma: Coincidence or Malignant Transformation?

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Ali, Neema M; Bhat, Ramesh; Rao, Shwetha B

2015-01-01

Lichen planus is a common papulosquamous disorder affecting about 1-2% of the population, neoplastic transformation of cutaneous lichen planus lesions occurs very rarely. A 40 year old female patient presented with a 1 year history of developing multiple, itchy, pigmented lesions over both lower legs which gradually spread to involve the whole body. A few tense bullae were seen on the extremities. An erythematous fleshy lesion was seen on the upper aspect of the left buttock. Skin biopsy from a plaque on the right forearm showed features suggestive of lichen planus. Skin biopsy of a bullae showed a sub epidermal bulla filled with a mixed inflammatory infiltrate. Direct immunofluorescence revealed no immunoreactants along the basement membrane zone. A diagnosis of erythrodermic lichen planus with bullous lichen planus was made. Biopsy of fleshy lesion of left buttock revealed a moderately differentiated squamous cell carcinoma. Erythrodermic lichen planus with bullous lesions and secondary squamous cell carcinoma; these occurences in a single patient is extremely rare and has not been previously reported to the best of our knowledge.

Immunohistochemical localization of basement membrane components during hair follicle morphogenesis

DEFF Research Database (Denmark)

Westgate, G E; Shaw, D A; Harrap, G J

1984-01-01

Specific antisera were used to investigate the distributions of several basement membrane zone (BMZ) components, namely, bullous pemphigoid antigen (BPA), heparan sulfate proteoglycan (HSPG), laminin, and type IV collagen, during the development of hair follicles in late embryo rats. BPA was not ......Specific antisera were used to investigate the distributions of several basement membrane zone (BMZ) components, namely, bullous pemphigoid antigen (BPA), heparan sulfate proteoglycan (HSPG), laminin, and type IV collagen, during the development of hair follicles in late embryo rats. BPA...... of the elongating follicle. HSPG was associated with the basal cell layer prior to the appearance of hair follicle primordia and became BMZ-associated before birth but after follicle buds were first observed. HSPG was also found to be associated with the basal cell surfaces in the epidermis, but not in the hair...... follicle. Laminin and type IV collagen were continually present in epidermal and follicular BMZ both before and during development of hair follicles and were later present in the dermal papilla matrix. From these observations we conclude that (1) laminin and type IV collagen are functionally important...

Coma blisters in children: case report and review of the literature.

Science.gov (United States)

Bosco, Laura; Schena, Donatella; Colato, Chiara; Biban, Paolo; Girolomoni, Giampiero

2013-12-01

Coma-induced blisters is a rare condition associated with prolonged impairment of conscious level, which is relatively well-known in adults following overdose with barbiturates. However, it has been very rarely described in children. A case of coma-bullae occurring in an 11-year-old child with meningoencephalitis is herein reported. The bullous lesions occurred on the limbs and trunks, and evolved into necrotic ulcers in a few days. No correlation with any drug overdosage was found. A skin biopsy revealed epidermal and eccrine sweat gland necrosis with abundant neutrophils, and thrombosis of the vessels in the lower dermis. A comprehensive review of the literature showed that only 5 cases of coma-bullae in children have been published so far. Coma blistering resolves spontaneously within days or weeks. Diagnosis of coma-bullae may require careful clinical-pathologic correlation to exclude other blistering diseases in children.

HRCT findings in the lungs of non-smokers with neurofibromatosis

International Nuclear Information System (INIS)

Oikonomou, Anastasia; Vadikolias, Konstantinos; Birbilis, Theodosios; Bouros, Demosthenes; Prassopoulos, Panos

2011-01-01

Purpose: Interstitial lung disease in neurofibromatosis (NF) has been disputed and attributed to smoking-related changes. The aim of this study was to describe HRCT findings in the lungs of non-smokers with NF. Materials and methods: Six never-smokers with NF underwent lung HRCT. Two radiologists evaluated the HRCT scans and a final decision was reached by consensus. The HRCT scans were analyzed with regard to the number, size, location (upper, middle or lower lung zone) and distribution (peripheral and central) of lung cysts and the presence of ground-glass density centrilobular micronodules. Results: All patients with NF had small (2–18 mm) thin wall cysts and upper-lobe predominant patchy areas of ground-glass density centrilobular micronodules. In five cases, there were 3–17 cysts and in one there were numerous (>100). Lung cysts were central (1), subpleural (1) and in both locations (4). Conclusion: Interstitial lung disease in NF is not associated with smoking and may be entirely asymptomatic. HRCT may reveal small cysts, with barely perceptible walls therefore not representing emphysema and occasionally a minimal micronodular pattern of ground glass opacity. There was no radiologic evidence of lung fibrosis, honeycombing or severe bullous disease.

Is There a Relation between ABO Blood Groups and Clinical Outcome in Patients with Pemphigoid? A Case-Control Study.

Science.gov (United States)

Bakhtiari, Sedigheh; Toosi, Parviz; Azimi, Somayyeh; Esmaili, Nafiseh; Montazami, Ali; Rafieian, Nasrin

2016-01-01

Background. Relationship between blood groups and dermatologic diseases remains controversial and was not yet fully elucidated nor explained clearly. The aim of this study was to examine if any relation exists between different types of pemphigoid diseases and ABO blood group. Methods. In this case-control study, 159 pemphigoid patients and 152 healthy matched-controls were evaluated. All blood group (including Rh status) data for the study was obtained from the hospital medical records. Statistical comparisons were completed with chi-square test and logistic regression. Results. Blood group "O" was found in 32.9% of patients and 38.2% of control group. Blood group "A" was found among 30.8% of patients and 34.2% of control group, while group "B" was reported in 27.4% of cases and 21.1% of controls and "AB" was identified among 8.9% of patients and 6.6% of control group. 84.9% of patients were Rh positive, while in the control group 86.2% of patients were Rh positive. No significant differences were found regarding ABO blood groups (P = 0.46) or Rh (P = 0.76) between pemphigoid patients and control group. Also, older females had the higher risk of developing bullous pemphigoid. Conclusion. We found no relationship between ABO blood groups and pemphigoid disease.

Imaging of pulmonary emphysema: A pictorial review

Science.gov (United States)

Takahashi, Masashi; Fukuoka, Junya; Nitta, Norihisa; Takazakura, Ryutaro; Nagatani, Yukihiro; Murakami, Yoko; Otani, Hideji; Murata, Kiyoshi

2008-01-01

The term ‘emphysema’ is generally used in a morphological sense, and therefore imaging modalities have an important role in diagnosing this disease. In particular, high resolution computed tomography (HRCT) is a reliable tool for demonstrating the pathology of emphysema, even in subtle changes within secondary pulmonary lobules. Generally, pulmonary emphysema is classified into three types related to the lobular anatomy: centrilobular emphysema, panlobular emphysema, and paraseptal emphysema. In this pictorial review, we discuss the radiological – pathological correlation in each type of pulmonary emphysema. HRCT of early centrilobular emphysema shows an evenly distributed centrilobular tiny areas of low attenuation with ill-defined borders. With enlargement of the dilated airspace, the surrounding lung parenchyma is compressed, which enables observation of a clear border between the emphysematous area and the normal lung. Because the disease progresses from the centrilobular portion, normal lung parenchyma in the perilobular portion tends to be preserved, even in a case of far-advanced pulmonary emphysema. In panlobular emphysema, HRCT shows either panlobular low attenuation or ill-defined diffuse low attenuation of the lung. Paraseptal emphysema is characterized by subpleural well-defined cystic spaces. Recent topics related to imaging of pulmonary emphysema will also be discussed, including morphometry of the airway in cases of chronic obstructive pulmonary disease, combined pulmonary fibrosis and pulmonary emphysema, and bronchogenic carcinoma associated with bullous lung disease. PMID:18686729

Off-Label Uses of Omalizumab.

Science.gov (United States)

El-Qutob, David

2016-02-01

The off-label use of medicines is a common and extensive clinical practice. Omalizumab has been licensed for use in severe allergic asthma and chronic urticaria. Omalizumab dosing was based on body weight and baseline serum IgE concentration. All patients are required to have a baseline IgE between 30 and 700 IU/ml and body weight not more than 150 kg. The use of off-label drugs may lead to several problems including adverse effects and an increased risk/benefit balance. In this article, there are summarized off-label uses of omalizumab in the last recent years in diseases in which IgE maybe or certainly has a corner role such as allergic rhinitis, allergic bronchopulmonary aspergillosis, anaphylaxis, keratoconjunctivitis, food allergy, drug allergy, urticaria, angioedema, non-atopic asthma, atopic dermatitis, nasal polyps, Churg-Strauss syndrome, eosinophilic otitis media, chronic rhinosinusitis, bullous pemphigoid, contact dermatitis, and others. Use in pregnancy asthmatic women and pre-co-administration with specific immunotherapy will also be revised.

Bronchial and pulmonary scintigraphy with radioactively marked aerosols

International Nuclear Information System (INIS)

Wuerstle, T.

1982-01-01

In 97 patients with bronchitis, bronchial asthma, tuberculosis, sarcoidosis, pneumoconiosis, or tumors the mucociliary clearance and/or deposit pattern after inhalation of radioactively marked aerosols (1 mCi 99m Tc sulfur colloid) was studied. Normal values of the mucociliary 30 min. clearance for the central bronchial/lung periphery are 21%/15%. There was a decreased clearance with bronchitis (11/8%), bronchial asthma, emphysema, tuberculosis, sarcoidosis, trachiobronchial amyloidosis, pleural scarring or interstitial pneumona. Increased clearance (29/19%) was shown with pneumoconiosis. The correlation of deposit pattern and disease, for example, bronchitis, bronchial asthma, bullous emphysema, pleural scarring, partial lung resection, bronchopneumonia, or bronchial restriction, is described. In comparison of aerosol scintigraphy to perfusion scintigraphy and ventilation with gaseous xenon, the aerosol scintigraphy is superior to xenon for certain indications. The aerosol particles, which are larger in comparison to xenon, settle easier by obstructions or flow variations and thereby give better clinical indications of regional differences. (orig.) [de

Steven johnsons syndrome and toxic epidermal necrolysis: A review

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Sri ram Anne

2014-12-01

Full Text Available Toxic epidermal necrolysis (TEN and Stevens Johnson Syndrome (SJS are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. They are characterized by mucocutaneous tenderness and typically hemorrhagic erosions, erythema and more or less severe epidermal detachment presenting as blisters and areas of denuded skin. Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the etiology remains unknown. Several drugs are at "high" risk of inducing TEN/SJS including: Allopurinol, Trimethoprim-sulfamethoxazole and other sulfonamide-antibiotics, aminopenicillins, cephalosporins, quinolones, carbamazepine, phenytoin, phenobarbital and NSAID's of the oxicam-type. Differential diagnosis includes linear IgA dermatosis and paraneoplastic pemphigus, pemphigus vulgaris and bullous pemphigoid, acute generalized exanthematous pustulosis (AGEP, disseminated fixed bullous drug eruption and staphyloccocal scalded skin syndrome (SSSS. Due to the high risk of mortality, management of patients with SJS/TEN requires rapid diagnosis, identification and interruption of the culprit drug, specialized supportive care ideally in an intensive care unit, and consideration of immunomodulating agents such as high-dose intravenous immunoglobulin therapy.

Medicinudløst hudkløe, sår og blærer hos tre patienter med pseudoporfyri

DEFF Research Database (Denmark)

Þorsteinsdóttir, Sunna; Velander, Marie Juul; Bygum, Anette

2015-01-01

Pseudoporphyria cutanea tarda is a well described bullous skin disorder which resembles porphyria cutanea tarda. However, the levels of porphyrins in plasma, urine and faeces are normal. We present three cases of patients with classical symptoms of pseudoporhyria. Two of the patients developed ps...... pseudoporphyria after the combination of intensive sunbathing and medications well known to cause pseudoporphyria. The third case received haemodialysis and furosemide....

Medicinudløst hudkløe, sår og blærer hos tre patienter med pseudoporfyri

DEFF Research Database (Denmark)

Thorsteinsdóttir, Sunna; Velander, Marie Juul; Bygum, Anette

2015-01-01

Pseudoporphyria cutanea tarda is a well described bullous skin disorder which resembles porphyria cutanea tarda. However, the levels of porphyrins in plasma, urine and faeces are normal. We present three cases of patients with classical symptoms of pseudoporphyria. Two of the patients developed p...... pseudoporphyria after the combination of intensive sunbathing and medications well known to cause pseudoporphyria. The third case received haemodialysis and furosemide....

O papel da imunofluorescência direta na fisiopatologia e no diagnóstico diferencial da estomatite aftóide recorrente The role of immunoflorescence in the physiopathology and differential diagnosis of recurrent aphthous stomatitis

Directory of Open Access Journals (Sweden)

Niels Salles Willo Wilhelmsen

2008-06-01

diagnosis carried out with vesicobullous diseases, such as pemphigus vulgaris. AIM: Check the presence of immunocomplexes in the mucosa of patients with aphthous stomatitis and the usefulness of the differential diagnosis method with bullous skin diseases. MATERIALS AND METHODS: 23 patients with aphthous stomatitis were prospectively included in the study. There were all submitted to mucosa biopsy under local anesthesia for the removal of two fragments. One of these was sent to histology and, the other to direct immunofluorescence. RESULTS: The 23 samples from the histology exam revealed an ulcerated inflammatory process. The samples referred to immunofluorescence resulted negative and only one showed the presence of complement in the basal membrane. CONCLUSION: Based on our results, we conclude that the patients with RAS do not show deposits of immunocomplexes in their oral cavity mucosa and immunofluorescence is useful in the differential diagnosis between this disease and bullous skin diseases.

Reptured Epidermal Inclusion Cyst in the Axilla: A Case Report

International Nuclear Information System (INIS)

Kim, Kyu Soon; Kim, Hak Hee; Shin, Hee Jeong; Yang, Hye Rin; Sohn, Jeong Hee; Kwon, Gui Young; Gong, Gyung Yub

2006-01-01

Epidermal inclusion cysts, the most common type of simple epithelial cyst, are typically well-encapsulated, subepidermal and mobile nodules. They may occur anywhere, but are mostly found on the scalp, face, neck, trunk, and back. Less than 10% of epidermal inclusion cysts occur on the extremities, and even fewer are found on the palms, soles, and breasts. If epidermal inclusion cysts rupture, foreign body reaction, granulomatous reaction or abscess formation could follow. We described here the sonographic findings of ruptured epidermal inclusion cyst of the right axilla in a 33-year-old woman who presented with a palpable axillary mass forming an inflammatory abscess

Safety and potential efficacy of gemfibrozil as a supportive treatment for children with late infantile neuronal ceroid lipofuscinosis and other lipid storage disorders.

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Kim, Kyeongsoon; Kleinman, Hynda K; Lee, Hahn-Jun; Pahan, Kalipada

2017-06-17

Neuronal Ceroid Lipofuscinosis (NCL), also known as Batten disease, is a group of genetically distinct lysosomal disorders that mainly affect the central nervous system, resulting in progressive motor and cognitive decline primarily in children. Multiple distinct genes involved in the metabolism of lipids have been identified to date with various mutations in this family of diseases. There is no cure for these diseases but some new therapeutic approaches have been tested that offer more hope than the standard palliative care. Many of the therapeutic advances require invasive procedures but some progress in slowing the disease has been found and more options can be expected in the future. We also review the literature on children with disease/conditions other than NCL for the non-invasive use, safety, and tolerability of a lipid-lowering drug, gemfibrozil, as a potential treatment for NCLs. Gemfibrozil has shown efficacy in an animal model of NCL known as CLN2 (late infantile classic juvenile) and has been shown to be safe for lowering lipids in children. Among the 200 non-NCL children found in the published literature who were treated with gemfibrozil for NCL-related problems, only 3 experienced adverse events, including 2 with muscle pain and 1 with localized linear IgA bullous dermatitis. We conclude that gemfibrozil is safe for long-term use in children, causes minimal adverse events, is well tolerated, and may delay the progression of NCLs. Gemfibrozil may potentially be an alternative to more invasive therapeutic approaches currently under investigation and has the potential to be used in combination with other therapeutic approaches.

Skin rash and arthritis a simplified appraisal of less common associations.

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Cozzi, A; Doria, A; Gisondi, P; Girolomoni, G

2014-06-01

Skin and joint manifestations are part of the clinical spectrum of many disorders. Well-known associations include psoriatic arthritis and arthritis associated with autoimmune connective tissue diseases. This review focuses on less common associations where skin lesions can provide easily accessible and valuable diagnostic clues, and directly lead to the specific diagnosis or limit the list of possibilities. This may also affect health care resources as diagnostic tests are often low-specific, highly expensive and poorly available. This group of diseases can be divided into two subsets, based on the presence/absence of fever, and then further classified according to elementary skin lesions (macular, urticarial, maculo-papular, vesico-bullous, pustular, petechial and nodular). In most instances joint involvement occurs as peripheral migrating polyarthritis. Erythematosus macular or urticarial rashes occur in most febrile disorders such as monogenic autoinflammatory syndromes, Schnitzler's syndrome, Still's disease and rheumatic fever and afebrile diseases as urticarial vasculitis. Pustular rash may be observed in chronic recurrent multifocal osteomyelitis (CRMO) and pyogenic arthritis with pyoderma gangrenosum and acne (PAPA) syndrome (both febrile) as well as in Behcet's disease and Synovitis, acne, pustulosis, hyperostosis and osteitis syndrome (both non-febrile). Papular lesions are typical of secondary syphilis, sarcoidosis, interstitial granulomatous dermatitis, papular petechial of cutaneous small-vessel vasculitis and nodular lesions of polyarteritis nodosa and multicentric reticulohistiocytosis all of which are afebrile. Differential diagnosis includes infections and drug reactions which may mimic several of these conditions. To biopsy the right skin lesion at the right time it is essential to obtain relevant histological information. © 2013 European Academy of Dermatology and Venereology.

Acute haemorrhagic oedema of infancy with bullae and koebnerisation

OpenAIRE

Mohd Sazlly Lim, S; Shamsudin, N

2014-01-01

A 5-month-old Malay boy presented with purpuric papules and plaques on the face and extremities accompanied by fever, coryzal symptoms and bilateral lower limb oedema. There were also bullous linear purpuric lesions on the right upper limb. Blood and culture tests were normal. Histopathological tests showed leucocytoclastic vasculitis, confirming the diagnosis of acute haemorrhagic oedema of infancy. The patient achieved complete recovery after 2 weeks with no recurrence.

Early onset of Chanarin-Dorfman syndrome with severe liver involvement in a patient with a complex rearrangement of ABHD5 promoter

OpenAIRE

Missaglia, Sara; Valadares, Eugenia Ribeiro; Moro, Laura; Faguntes, Eleonora Druve Tavares; quintão Roque, Raquel; Giardina, Bruno; Tavian, Daniela

2014-01-01

Background α/β-hydrolase domain-containing protein 5 (ABHD5) plays an important role in the triacylglycerols (TAG) hydrolysis. Indeed, ABHD5 is the co-activator of adipose triglyceride lipase (ATGL), that catalyses the initial step of TAG hydrolysis. Mutations in ABHD5 gene are associated with the onset of Chanarin-Dorfman syndrome (CDS), a rare autosomal recessive lipid storage disorder, characterized by non-bullous congenital ichthyosiform erythroderma (NCIE), hepatomegaly and liver steatos...

Acute haemorrhagic oedema of infancy with bullae and koebnerisation

Directory of Open Access Journals (Sweden)

Norashikin Shamsudin

2014-08-01

Full Text Available A 5-month-old Malay boy presented with purpuric papules and plaques on the face and extremities accompanied by fever, coryzal symptoms and bilateral lower limb oedema. There were also bullous linear purpuric lesions on the right upper limb. Blood and culture tests were normal. Histopathological tests showed leucocytoclastic vasculitis, confirming the diagnosis of acute haemorrhagic oedema of infancy. The patient achieved complete recovery after 2 weeks with no recurrence.

Legalizing Cannabis: A physician's primer on the pulmonary effects of marijuana.

Science.gov (United States)

Lutchmansingh, Denyse; Pawar, Leena; Savici, Dana

2014-01-01

Habitual smoking of marijuana is associated with multiple respiratory symptoms such as cough, sputum production, and wheezing .These symptoms are not significantly different from those exhibited by tobacco smokers. Furthermore, endobronchial biopsies of habitual smokers of marijuana and /or tobacco have shown that both marijuana and cigarette smoking cause significant bronchial mucosal histopathology and that these effects are additive. Although marijuana smokers have minimal changes in pulmonary function studies as compared to tobacco smokers, they may develop bullous disease and spontaneous pneumothoraces. The relationship between marijuana smoking and lung cancer remains unclear due to design limitations of the studies published so far. These findings should warn individuals that marijuana smoking may result in serious short-term and long-term respiratory complications, and habitual marijuana use should be viewed with caution. The medical literature so far does not support routine evaluation by pulmonary function tests or imaging studies; until more definitive data is available, we do not recommend the regular use of these tests in the evaluation of habitual marijuana smokers.

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

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Michael P. Pender

2012-01-01

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

Anterior stromal puncture in the treatment of bullous keratopathy

OpenAIRE

Gomes, José Alvaro Pereira; Haraguchi, Daniel Keizo de Medeiros; Zambrano, Delbis Uzcátegui; Villavicencio, Luis Izquierdo; Cunha, Marcelo C.; Freitas, Denise de

2000-01-01

Objetivo: Avaliar o efeito terapêutico das punções do estroma anterior corneal em pacientes com ceratopatia bolhosa (CB). Métodos:Vinte e cinco pacientes com CB sintomáticos, com baixa visão, com e sem indicação de transplante de córnea, foram avaliados antes, uma, 4 e 12 semanas após punções estromais anteriores realizadas com agulha #25 à lâmpada de fenda. Em cada visita, os pacientes foram questionados sobre intensidade da dor, fotofobia, sensação de corpo estranho, além de serem submetido...

Increased risk of stroke in contact dermatitis patients

Science.gov (United States)

Chang, Wei-Lun; Hsu, Min-Hsien; Lin, Cheng-Li; Chan, Po-Chi; Chang, Ko-Shih; Lee, Ching-Hsiao; Hsu, Chung-Yi; Tsai, Min-Tein; Yeh, Chung-Hsin; Sung, Fung-Chang

2017-01-01

Abstract Dermatologic diseases are not traditional risk factors of stroke, but recent studies show atopic dermatitis, psoriasis, and bullous skin disease may increase the risk of stroke and other cardiovascular diseases. No previous studies have focused on the association between contact dermatitis and stroke. We established a cohort comprised of 48,169 contact dermatitis patients newly diagnosed in 2000–2003 and 96,338 randomly selected subjects without the disorder, frequency matched by sex, age, and diagnosis year, as the comparison cohort. None of them had a history of stroke. Stroke incidence was assessed by the end of 2011 for both cohorts. The incidence stroke was 1.1-fold higher in the contact dermatitis cohort than in the comparison cohort (5.93 vs 5.37 per 1000 person-years, P contact dermatitis cohort increased with age, from 1.14 (95% CI, 1.03–1.27) for 65 to 74 years; to 1.27 (95% CI, 1.15–1.42) for 75 years and older. The aHR of stroke were 1.16 (95% CI, 1.07–1.27) and 1.09 (95% CI, 1.00–1.18) for men and women, respectively. This study suggests that patients with contact dermatitis were at a modestly increased risk of stroke, significant for ischemic stroke but not for hemorrhagic stroke. Comorbidity, particularly hypertension, increased the hazard of stroke further. PMID:28272195

Evaluation of cases of pemphigus vulgaris and pemphigus foliaceus from a reference service in Pará state, Brazil.

Science.gov (United States)

Pires, Carla Andréa Avelar; Viana, Viviane Brito; Araújo, Fernando Costa; Müller, Silvia Ferreira Rodrigues; Oliveira, Miguel Saraty de; Carneiro, Francisca Regina Oliveira

2014-01-01

Pemphigusis a bullous, rare and chronic autoimmune disease. There are two major forms of pemphigus: vulgaris and foliaceus. Epidemiological data and clinical outcome in patients diagnosed in the Brazilian Amazon states are still rare. To study the occurrence of the disease during the study period and analyze the epidemiological profile of patients, the most common subtype of pemphigus, and the clinical evolution of patients. Retrospective analysis of medical records of hospitalized patients with pemphigus foliaceus and pemphigus vulgaris in the period from 2003 to 2010 in Dermatology Service of Hospital Fundação Santa Casa de Misericórdia do Pará, Belém, Northern Brazil. We found a total of 20 cases of pemphigus during the study period, 8 of which were of foliaceus pemphigus and 12 of vulgaris pemphigus. Pemphigus foliaceus had the predominance of male patients (75%), showed satisfactory clinical evolution, and was characterized by absence of pediatric cases. Pemphigus vulgaris affected more women (66.7%), showed mean hospital stay of 1 to 3 months (50%), and there were three cases of death (25%). The prescribed immunosuppressive drugs included prednisone with or without combination of azathioprine and/or dapsone. Sepsis was associated with 100% of the deaths. The occurrence of the disease is rare, there are no familiar/endemic outbreaks in the sample. Evolution is usually favorable, but secondary infection is associated with worse prognosis. The choice of best drugs to treat pemphigus remains controversial.

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

4th Pediatric Allergy and Asthma Meeting (PAAM)

OpenAIRE

Yavuz, S. Tolga; Koc, Ozan; Gungor, Ali; Gok, Faysal; Hawley, Jessica; O?Brien, Christopher; Thomas, Matthew; Brodlie, Malcolm; Michaelis, Louise; Mota, In?s; Gaspar, ?ngela; Piedade, Susana; Sampaio, Gra?a; Dias, Jos? Geraldo; Paiva, Miguel

2016-01-01

Table of contents WORKSHOP 4: Challenging clinical scenarios (CS01?CS06) CS01 Bullous lesions in two children: solitary mastocytoma S. Tolga Yavuz, Ozan Koc, Ali Gungor, Faysal Gok CS02 Multi-System Allergy (MSA) of cystic fibrosis: our institutional experience Jessica Hawley, Christopher O?Brien, Matthew Thomas, Malcolm Brodlie, Louise Michaelis CS03 Cold urticaria in pediatric age: an invisible cause for severe reactions In?s Mota, ?ngela Gaspar, Susana Piedade, Gra?a Sampaio, Jos? Geraldo ...

Beau´s lines due to cytostatic drugs in a patient with breast cancer

Directory of Open Access Journals (Sweden)

Patricia Chang

2014-04-01

Full Text Available Female patient, 47 years old who was hospitalized due to urinary tract infection, during her hospitalization bullous lesions appeared on her left limb and interconsultation to the Dermatology Department was made. The patient has been treated by breast cancer with docetaxel, doxorubicin and cyclophosphamide; she had received 5 cycles of chemotherapy Clinical examination showed vesicles on an erythematous base of the left arm following a linear pattern and the diagnosis of herpes zoster was done.

Toxic epidermal necrolysis and Stevens-Johnson syndrome

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French Lars E

2010-12-01

Full Text Available Abstract Toxic epidermal necrolysis (TEN and Stevens Johnson Syndrome (SJS are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. Both are rare, with TEN and SJS affecting approximately 1or 2/1,000,000 annually, and are considered medical emergencies as they are potentially fatal. They are characterized by mucocutaneous tenderness and typically hemorrhagic erosions, erythema and more or less severe epidermal detachment presenting as blisters and areas of denuded skin. Currently, TEN and SJS are considered to be two ends of a spectrum of severe epidermolytic adverse cutaneous drug reactions, differing only by their extent of skin detachment. Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. Several drugs are at "high" risk of inducing TEN/SJS including: Allopurinol, Trimethoprim-sulfamethoxazole and other sulfonamide-antibiotics, aminopenicillins, cephalosporins, quinolones, carbamazepine, phenytoin, phenobarbital and NSAID's of the oxicam-type. Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. Diagnosis relies mainly on clinical signs together with the histological analysis of a skin biopsy showing typical full-thickness epidermal necrolysis due to extensive keratinocyte apoptosis. Differential diagnosis includes linear IgA dermatosis and paraneoplastic pemphigus, pemphigus vulgaris and bullous pemphigoid, acute generalized exanthematous pustulosis (AGEP, disseminated fixed bullous drug eruption and staphyloccocal scalded skin syndrome (SSSS. Due to the high risk of mortality, management of patients with SJS/TEN requires rapid diagnosis, evaluation of the prognosis

Some Important Diseases of Tree Fruits - Diseases of Vegetable Crops - Diseases of Grapes - Diseases of Tree Nuts.

Science.gov (United States)

Petersen, Donald H.; And Others

This agriculture extension service publication from Pennsylvania State University consists of four sections on plant disease recognition and control. The titles of these four sections are: (1) Some Important Diseases of Tree Fruits; (2) Diseases of Vegetable Crops; (3) Diseases of Crops; and (4) Diseases of Tree Nuts. The first section discusses…

Cutaneous and systemic hypersensitivity reactions to metallic implants

DEFF Research Database (Denmark)

Basko-Plluska, Juliana L; Thyssen, Jacob P; Schalock, Peter C

2011-01-01

Cutaneous reactions to metal implants, orthopedic or otherwise, are well documented in the literature. The first case of a dermatitis reaction over a stainless steel fracture plate was described in 1966. Most skin reactions are eczematous and allergic in nature, although urticarial, bullous....... However, other metal ions as well as bone cement components can cause such hypersensitivity reactions. To complicate things, patients may also develop delayed-type hypersensitivity reactions to metals (ie, in-stent restenosis, prosthesis loosening, inflammation, pain, or allergic contact dermatitis...

4th Pediatric Allergy and Asthma Meeting (PAAM)

OpenAIRE

Yavuz, S. Tolga; Koc, Ozan; Gungor, Ali; Gok, Faysal; Hawley, Jessica; O’Brien, Christopher; Thomas, Matthew; Brodlie, Malcolm; Michaelis, Louise; Mota, Inês; Gaspar, Ângela; Piedade, Susana; Sampaio, Graça; Dias, José Geraldo; Paiva, Miguel

2016-01-01

Table of contents WORKSHOP 4: Challenging clinical scenarios (CS01–CS06) CS01 Bullous lesions in two children: solitary mastocytoma S. Tolga Yavuz, Ozan Koc, Ali Gungor, Faysal Gok CS02 Multi-System Allergy (MSA) of cystic fibrosis: our institutional experience Jessica Hawley, Christopher O’Brien, Matthew Thomas, Malcolm Brodlie, Louise Michaelis CS03 Cold urticaria in pediatric age: an invisible cause for severe reactions Inês Mota, Ângela Gaspar, Susana Piedade, Graça Sampaio, José Geraldo ...

Renal disease in patients with celiac disease.

Science.gov (United States)

Boonpheng, Boonphiphop; Cheungpasitporn, Wisit; Wijarnpreecha, Karn

2018-04-01

Celiac disease, an inflammatory disease of small bowel caused by sensitivity to dietary gluten and related protein, affects approximately 0.5-1% of the population in the Western world. Extra-intestinal symptoms and associated diseases are increasingly recognized including diabetes mellitus type 1, thyroid disease, dermatitis herpetiformis and ataxia. There have also been a number of reports of various types of renal involvement in patients with celiac disease including diabetes nephropathy, IgA nephropathy, membranous nephropathy, membranoproliferative glomerulonephritis, nephrotic syndrome related to malabsorption, oxalate nephropathy, and associations of celiac disease with chronic kidney disease and end-stage kidney disease. This review aims to present the current literature on possible pathologic mechanisms underlying renal disease in patients with celiac disease.

Immune response in pemphigus and beyond: progresses and emerging concepts.

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Di Zenzo, Giovanni; Amber, Kyle T; Sayar, Beyza S; Müller, Eliane J; Borradori, Luca

2016-01-01

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are two severe autoimmune bullous diseases of the mucosae and/or skin associated with autoantibodies directed against desmoglein (Dsg) 3 and/or Dsg1. These two desmosomal cadherins, typifying stratified epithelia, are components of cell adhesion complexes called desmosomes and represent extra-desmosomal adhesion receptors. We herein review the advances in our understanding of the immune response underlying pemphigus, including human leucocyte antigen (HLA) class II-associated genetic susceptibility, characteristics of pathogenic anti-Dsg antibodies, antigenic mapping studies as well as findings about Dsg-specific B and T cells. The pathogenicity of anti-Dsg autoantibodies has been convincingly demonstrated. Disease activity and clinical phenotype correlate with anti-Dsg antibody titers and profile while passive transfer of anti-Dsg IgG from pemphigus patients' results in pemphigus-like lesions in neonatal and adult mice. Finally, adoptive transfer of splenocytes from Dsg3-knockout mice immunized with murine Dsg3 into immunodeficient mice phenotypically recapitulates PV. Although the exact pathogenic mechanisms leading to blister formation have not been fully elucidated, intracellular signaling following antibody binding has been found to be necessary for inducing cell-cell dissociation, at least for PV. These new insights not only highlight the key role of Dsgs in maintenance of tissue homeostasis but are expected to progressively change pemphigus management, paving the way for novel targeted immunologic and pharmacologic therapies.

Resultados e indicações de ceratoplastias penetrantes realizadas por médicos em treinamento, num país em desenvolvimento Indications and results of penetrating keratoplasty performed by training physicians in a developing country

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Marta Ferrari Teixeira

2001-11-01

- "Hospital de Base", in terms of indication for penetrating keratoplasty and final visual acuity. The minimum follow-up to assess the final visual acuity was 6 months. The cases of penetrating keratoplasty were divided into 6 groups of preoperative diagnosis for better comparison and statistical analysis of the final visual acuity. Such groups were: keratoconus, pseudophakic bullous keratopathy, aphakic bullous keratopathy, Fuchs' endothelial distrophy, band keratopathy and leukomas. All surgeries were performed by residents under supervision. Results: Keratoconus was the group that reached the highest average increase of postoperative lines of visual acuity (7.55 ± 2.83 and band keratopathy the smallest one (0.33 ± 3.20. A significant difference was observed among the indications for penetrating keratoplasty when compared to developed countries, where keratoconus is the most predominant indication. Our analysis revealed that the most important indications for penetrating keratoplasty in decreasing order were: leukoma, pseudophakic bullous keratopathy, keratoconus, Fuchs' endothelial distrophy, aphakic bullous keratopathy and band keratopathy. Conclusion: Keratoconus showed significant difference of final visual acuity levels (p value < 0,05 when compared to the other 5 groups of penetrating keratoplasty indications.

[Periodontal disease in pediatric rheumatic diseases].

Science.gov (United States)

Fabri, Gisele M C; Savioli, Cynthia; Siqueira, José T; Campos, Lucia M; Bonfá, Eloisa; Silva, Clovis A

2014-01-01

Gingivitis and periodontitis are immunoinflammatory periodontal diseases characterized by chronic localized infections usually associated with insidious inflammation This narrative review discusses periodontal diseases and mechanisms influencing the immune response and autoimmunity in pediatric rheumatic diseases (PRD), particularly juvenile idiopathic arthritis (JIA), childhood-onset systemic lupus erythematosus (C-SLE) and juvenile dermatomyositis (JDM). Gingivitis was more frequently observed in these diseases compared to health controls, whereas periodontitis was a rare finding. In JIA patients, gingivitis and periodontitis were related to mechanical factors, chronic arthritis with functional disability, dysregulation of the immunoinflammatory response, diet and drugs, mainly corticosteroids and cyclosporine. In C-SLE, gingivitis was associated with longer disease period, high doses of corticosteroids, B-cell hyperactivation and immunoglobulin G elevation. There are scarce data on periodontal diseases in JDM population, and a unique gingival pattern, characterized by gingival erythema, capillary dilation and bush-loop formation, was observed in active patients. In conclusion, gingivitis was the most common periodontal disease in PRD. The observed association with disease activity reinforces the need for future studies to determine if resolution of this complication will influence disease course or severity. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

Drug delivery from the oral cavity: a focus on mucoadhesive buccal drug delivery systems.

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Shinkar, Dattatraya Manohar; Dhake, Avinash Sridhar; Setty, Chitral Mallikarjuna

2012-01-01

Since the early 1980s the concept of mucoadhesion has gained considerable interest in pharmaceutical technology. The various advantages associated with these systems made buccal drug delivery as a novel route of drug administration. It prolongs the residence time of the dosage form at the site of application. These systems remain in close contact with the absorption tissue, the mucous membrane, and thus contribute to improved and/or better therapeutic performance of the drug and of both local and systemic effects. This review highlights the anatomy and structure of oral mucosa, mechanism and theories of mucoadhesion, factors affecting mucoadhesion, characteristics and properties of desired mucoadhesive polymers, various types of dosage forms, and general considerations in design of mucoadhesive buccal dosage forms, permeation enhancers, and evaluation methods. Over the past few decades the mucoadhesive buccal drug delivery system has received a great deal of attention to develop mucoadhesive dosage forms to enable the prolonged retention at the site of action, providing a controlled release of drug for improved therapeutic outcome. Mucoadhesive drug delivery gives facility to include a permeation enhancer/enzyme inhibitor or pHmodifier in the formulation and versatility in designing as multidirectional or unidirectional release systems for local and systemic action. Local delivery to tissues of the oral cavity has a number of applications, including treatment of local conditions such as periodontal disease, bacterial and fungal infections, and aphthous stomatitis and vesiculo bullous diseases. For the treatment of chronic diseases, the mucoadhesive buccal drug delivery system allows easily accessibility and is generally well-accepted for administeringdrugs by systemic action.

Immunoglobulin E-Mediated Autoimmunity

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Marcus Maurer

2018-04-01

Full Text Available The study of autoimmunity mediated by immunoglobulin E (IgE autoantibodies, which may be termed autoallergy, is in its infancy. It is now recognized that systemic lupus erythematosus, bullous pemphigoid (BP, and chronic urticaria, both spontaneous and inducible, are most likely to be mediated, at least in part, by IgE autoantibodies. The situation in other conditions, such as autoimmune uveitis, rheumatoid arthritis, hyperthyroid Graves’ disease, autoimmune pancreatitis, and even asthma, is far less clear but evidence for autoallergy is accumulating. To be certain of an autoallergic mechanism, it is necessary to identify both IgE autoantibodies and their targets as has been done with the transmembrane protein BP180 and the intracellular protein BP230 in BP and IL-24 in chronic spontaneous urticaria. Also, IgE-targeted therapies, such as anti-IgE, must have been shown to be of benefit to patients as has been done with both of these conditions. This comprehensive review of the literature on IgE-mediated autoallergy focuses on three related questions. What do we know about the prevalence of IgE autoantibodies and their targets in different diseases? What do we know about the relevance of IgE autoantibodies in different diseases? What do we know about the cellular and molecular effects of IgE autoantibodies? In addition to providing answers to these questions, based on a broad review of the literature, we outline the current gaps of knowledge in our understanding of IgE autoantibodies and describe approaches to address them.

Screen-detected gallstone disease and cardiovascular disease

DEFF Research Database (Denmark)

Shabanzadeh, Daniel Mønsted; Skaaby, Tea; Sørensen, Lars Tue

2017-01-01

Knowledge about temporal associations for screen-detected gallstone disease and cardiovascular disease is limited. The objective of this study was to determine if screen-detected gallstones or cholecystectomy was associated with development of cardiovascular disease. A cohort study of three...... of cardiovascular disease through nationwide registers until December 2014. Multivariable Cox regression analyses were performed including traditional cardiovascular disease risk factors and apolipoprotein E genotype. Gallstone disease was identified in 10% (591/5928) of participants at baseline of whom 6.8% had...... gallstones and 3.2% had cholecystectomy. The study population was followed for a period of 32 years with only 1% lost to follow-up. Gallstone disease was associated with all cardiovascular disease (hazard ratio (HR) 1.36, 95% confidence interval (CI) [1.17;1.59]) and to the subgroups coronary artery (HR 1...

Complex parasitism and symbiosis of crinoid, subepidermal parasite, and tabulate coral. Lower Devonian (Pragian, Barrandian, Czech Republic

Czech Academy of Sciences Publication Activity Database

Galle, Arnošt; Prokop, J. R.

2000-01-01

Roč. 75, č. 4 (2000), s. 441-444 ISSN 1210-3527 R&D Projects: GA AV ČR IAA3013807 Institutional research plan: CEZ:AV0Z3013912 Subject RIV: DB - Geology ; Mineralogy http://www.geology.cz/bulletin/contents/2000/vol75no4/bullgeosci200004441a.pdf

Bullous pemphigoid antigen localization suggests an intracellular association with hemidesmosomes

DEFF Research Database (Denmark)

Westgate, G E; Weaver, A C; Couchman, J R

1985-01-01

immunofluorescent staining for BPA is linear at the basement membrane zone (BMZ) of skin and many other epithelial tissues. At higher magnification however, we observed a punctate staining pattern for BPA which was regular in appearance and suggested localization of BPA to discrete structures at the BMZ. Subsequent...... intracellularly both in vivo and in vitro. We suggest that BPA is not normally a lamina lucida component, but that it may form part of a linkage between the cytoskeleton and the basement membrane....

[Lung volume reduction surgery for emphysema and bullous pulmonary emphysema].

Science.gov (United States)

Le Pimpec-Barthes, F; Das Neves-Pereira, J-C; Cazes, A; Arame, A; Grima, R; Hubsch, J-P; Zukerman, C; Hernigou, A; Badia, A; Bagan, P; Delclaux, C; Dusser, D; Riquet, M

2012-04-01

The improvement of respiratory symptoms for emphysematous patients by surgery is a concept that has evolved over time. Initially used for giant bullae, this surgery was then applied to patients with diffuse microbullous emphysema. The physiological and pathological concepts underlying these surgical procedures are the same in both cases: improve respiratory performance by reducing the high intrapleural pressure. The functional benefit of lung volume reduction surgery (LVRS) in the severe diffuse emphysema has been validated by the National Emphysema Treatment Trial (NETT) and the later studies which allowed to identify prognostic factors. The quality of the clinical, morphological and functional data made it possible to develop recommendations now widely used in current practice. Surgery for giant bullae occurring on little or moderately emphysematous lung is often a simpler approach but also requires specialised support to optimize its results. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

Bullous eosinophilic cellulitis (Wells' syndrome) associated with Churg-Strauss syndrome

NARCIS (Netherlands)

Schuttelaar, M L A; Jonkman, M F

We report a patient with Churg-Strauss syndrome (CSS) with asthma, eosinophilia, nasal polyposis and ANCA-associated multisystem vasculitis, who's skin eruption started with erythematous urticarial-plaques followed by haemorrhagic bullae. Histology of the plaques revealed 'flame figures' in the

Disseminated strongyloidiasis in an immunocompromised host: A case report

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Nurul Suhaiza Hassanudin

2017-06-01

Full Text Available Infections caused by Strongyloides stercoralis (S. stercoralis in human are generally asymptomatic, however in immunocompromised individual, hyperinfection may develop with dissemination of larvae to extra-intestinal organs. The diagnosis could be easily missed due to asymptomatic presentation and insufficient exposure towards the infection itself, which may lead to low index of suspicion as a consequence. In this report, a case of a Malaysian male with underlying diabetes mellitus, hypertension, cerebrovascular accident, bullous pemphigus and syndrome of inappropriate antidiuretic hormone secretion who initially complained of generalized body weakness and poor appetite without any history suggestive of sepsis is presented. However, he developed septicemic shock later, and S. stercoralis larvae was incidentally found in the tracheal aspirate that was sent to look for acid fast bacilli. Regardless of aggressive resuscitation, the patient succumbed due to pulmonary hemorrhage and acute respiratory distress syndrome. It was revealed that the current case has alarmed us via incidental finding of S. stercoralis larvae in the tracheal aspirate, indicating that the importance of the disease should be emphasized in certain parts of the world and population respectively.

Quantitative & qualitative analysis of endothelial cells of donor cornea before & after penetrating keratoplasty in different pathological conditions

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Aruna K.R. Gupta

2016-01-01

Full Text Available Background & objectives: Endothelial cells of the donor cornea are known to be affected quantitatively and qualitatively in different pathological conditions after penetrating keratoplasty (PK and this has direct effect on the clarity of vision obtained after PK. This study was undertaken to analyze the qualitative and quantitative changes in donor endothelial cells before and after PK in different pathological conditions. Methods: A prospective investigational analysis of 100 consecutive donor corneas used for penetrating keratoplasty between June 2006 and June 2008, was conducted. The patients were evaluated on the first day, at the end of first week, first month, third and six months and one year. Results: A decrease was observed in endothelial cell count in all pathological conditions. After one year of follow up the loss was 33.1 per cent in corneal opacity, 45.9 per cent in acute infective keratitis (AIK, 58.5 per cent in regrafts, 28.5 per cent in pseudophakic bullous keratopathy (PBK, 37 per cent in descemetocele, 27 per cent in keratoconus and 35.5 per cent in aphakic bullous keratopathy (ABK cases. Interpretation & conclusions: The endothelial cell loss was highest in regraft cases which was significant (P<0.05, while the least endothelial cell loss was seen in keratoconus cases. The cell loss was associated with increase in coefficient of variation (CV, i.e. polymegathism and pleomorphism. Inspite of this polymegathism and pleomorphism, the clarity of the graft was maintained.

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

Farber's Disease

Science.gov (United States)

... management, and therapy of rare diseases, including the lipid storage diseases. Research on lipid storage diseases within the Network includes ... management, and therapy of rare diseases, including the lipid storage diseases. Research on lipid storage diseases within the Network includes ...

Association between periodontal diseases and systemic diseases

Directory of Open Access Journals (Sweden)

Patrícia Weidlich

2008-08-01

Full Text Available 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 regarding pneumonia. Although the majority of them observed an association between periodontitis and systemic conditions, a causal relationship still needs to be demonstrated. Further studies, particularly interventional well-designed investigations, with larger sample sizes, need to be conducted in Brazilian populations.

Link Between Celiac Disease and Inflammatory Bowel Disease.

Science.gov (United States)

Shah, Ayesha; Walker, Marjorie; Burger, Daniel; Martin, Neal; von Wulffen, Moritz; Koloski, Natasha; Jones, Mike; Talley, Nicholas J; Holtmann, Gerald J

2018-05-14

The aim of this analysis was to assess in patients with inflammatory bowel disease (IBD) the risk of celiac disease and in celiac disease patients the risk of IBD. Previous studies report a possible association between IBD and celiac disease; however, this link is controversial. Using the search terms "inflammatory bowel disease" and "celiac disease," we identified initially 1525 publications. In total 27 studies met inclusion criteria. Proportions and 95% confidence intervals (CIs) for the prevalence of IBD in celiac disease and vice versa were compared with published prevalence rates for the respective geographic regions. We included 41,482 adult IBD patients (20,357 with Crohn's disease; 19,791 with ulcerative colitis; and 459 patients with celiac disease). Overall, in IBD patients the prevalence of celiac disease was 1110/100,000 (95% CI, 1010-1210/100,000) as compared with a prevalence of 620/100,000 (95% CI, 610-630/100,000) in the respective populations (odds ratio, 2.23; 95% CI, 1.99-2.50). In contrast, in patients with celiac disease, 2130/100,000 had IBD (95% CI, 1590-2670/100,000) as compared with 260/100,000 (95% CI, 250/100,000-270/100,000) in the respective populations (odds ratio, 11.10; 95% CI, 8.55-14.40). This effect was not different for ulcerative colitis and Crohn's disease. Although there was no evidence for publication bias for celiac disease in IBD, the funnel plot suggested that the association between IBD in celiac disease might be influenced by publication bias. The data are consistent with the notion that celiac disease is a risk factor for IBD and to lesser degree patients with IBD have an increased risk of celiac disease.

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

Science.gov (United States)

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

2011-01-01

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

Allergic contact dermatitis mimicking angioedema due to paraphenylendiamine hypersensitivity: a case report.

Science.gov (United States)

Tukenmez Demirci, Gulsen; Kivanc Altunay, Ilknur; Atis, Guldehan; Kucukunal, Asli

2012-09-01

Active sensitization to paraphenylendiamine (PPD) and related compounds from temporary black henna tattoos has become an epidemic in the recent years. Hair dyes also include PPD like black henna tatoos which cause allergic contact dermatitis. Skin lesions of allergic contact dermatitis from PPD are mostly seen as an exudative erythema, an erythema multiforme-like eruption or a bullous contact dermatitis. We, herein, report a 27 year-old woman with an angioedema-like reaction occurring after the first exposure to hair dye who was unaware of being previously sensitized to PPD from black henna tattoo.

The evolution of plant secretory structures and emergence of terpenoid chemical diversity.

Science.gov (United States)

Lange, Bernd Markus

2015-01-01

Secretory structures in terrestrial plants appear to have first emerged as intracellular oil bodies in liverworts. In vascular plants, internal secretory structures, such as resin ducts and laticifers, are usually found in conjunction with vascular bundles, whereas subepidermal secretory cavities and epidermal glandular trichomes generally have more complex tissue distribution patterns. The primary function of plant secretory structures is related to defense responses, both constitutive and induced, against herbivores and pathogens. The ability to sequester secondary (or specialized) metabolites and defense proteins in secretory structures was a critical adaptation that shaped plant-herbivore and plant-pathogen interactions. Although this review places particular emphasis on describing the evolution of pathways leading to terpenoids, it also assesses the emergence of other metabolite classes to outline the metabolic capabilities of different plant lineages.

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

African Journals Online (AJOL)

Huntington's disease is an inherited intricate brain illness. It is a neurodegenerative, insidious disorder; the onset of the disease is very late to diagnose. It is caused by an expanded CAG repeat in the Huntingtin gene, which encodes an abnormally long polyglutamine repeat in the Huntingtin protein. Huntington's disease ...

Endocrine Diseases

Science.gov (United States)

... Syndrome (PCOS) Pregnancy and Thyroid Disease Primary Hyperparathyroidism Prolactinoma Thyroid Tests Turner Syndrome Contact Us The National ... Management Liver Disease Urologic Diseases Endocrine Diseases Diet & Nutrition Blood Diseases Diagnostic Tests La información de la ...

Ribbing disease

International Nuclear Information System (INIS)

Mukkada, Philson J; Franklin, Teenu; Rajeswaran, Rangasami; Joseph, Santhosh

2010-01-01

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

Liposomes for Targeted Delivery of Active Agents against Neurodegenerative Diseases (Alzheimer's Disease and Parkinson's Disease

Directory of Open Access Journals (Sweden)

Carlos Spuch

2011-01-01

Full Text Available Neurodegenerative diseases, such as Alzheimer's disease and Parkinson's disease represent a huge unmet medical need. The prevalence of both diseases is increasing, but the efficacy of treatment is still very limited due to various factors including the blood brain barrier (BBB. Drug delivery to the brain remains the major challenge for the treatment of all neurodegenerative diseases because of the numerous protective barriers surrounding the central nervous system. New therapeutic drugs that cross the BBB are critically needed for treatment of many brain diseases. One of the significant factors on neurotherapeutics is the constraint of the blood brain barrier and the drug release kinetics that cause peripheral serious side effects. Contrary to common belief, neurodegenerative and neurological diseases may be multisystemic in nature, and this presents numerous difficulties for their potential treatment. Overall, the aim of this paper is to summarize the last findings and news related to liposome technology in the treatment of neurodegenerative diseases and demonstrate the potential of this technology for the development of novel therapeutics and the possible applications of liposomes in the two most widespread neurodegenerative diseases, Alzheimer's disease and Parkinson's disease.

Women's Heart Disease: Heart Disease Risk Factors

Science.gov (United States)

... this page please turn JavaScript on. Feature: Women's Heart Disease Heart Disease Risk Factors Past Issues / Winter 2014 Table ... or habits may raise your risk for coronary heart disease (CHD). These conditions are known as risk ...

Autoantibodies other than anti-desmogleins in pemphigus vulgaris patients

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Marwah Adly Saleh

2017-01-01

Full Text Available Background: Pemphigus vulgaris (PV is an immunoglobulin G-mediated autoimmune bullous skin disease. Nonorgan-specific antibodies were detected in Tunisian and Brazilian pemphigus patients with different prevalence. Materials and Methods: Fifty PV patients and fifty controls were screened for antinuclear antibodies (ANAs, anti-smooth muscle antibodies (ASMAs, anti-parietal antibodies (APAs, anti-mitochondrial antibodies, and Anti-nuclear cytoplasmic antibodies (ANCA by indirect immunofluorescence. Results: Thirty-nine patients were female and 11 were male. Fifteen patients did not receive treatment before while 35 patients were on systemic steroid treatment ± azathioprine. Twenty (40% of the PV patients and 1 (2% control had positive ANA. ANA was significantly higher in PV patients than controls, P< 0.0001. ASMAs were detected in 20 (40% PV patients and none of the controls. ASMA was significantly higher in PV patients than controls, P< 0.0001. No significant difference was detected between treated and untreated regarding ANA, P - 0.11. However, there was a significant difference between treated and untreated regarding ASMA, P- 0.03. Six patients (12% and none of the controls had positive APA. There was a significant difference between the patients and the controls in APA. P- 0.027. Conclusion: Egyptian PV patients showed more prevalent ANA, ASMA, and APA than normal controls. Follow-up of those patients is essential to detect the early development of concomitant autoimmune disease. Environmental factors might account for the variability of the nonorgan-specific antibodies among different populations.

A Case of Mature Natural Killer-Cell Neoplasm Manifesting Multiple Choroidal Lesions: Primary Intraocular Natural Killer-Cell Lymphoma

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Yoshiaki Tagawa

2015-11-01

Full Text Available Purpose: Natural killer (NK cell neoplasm is a rare disease that follows an acute course and has a poor prognosis. It usually emerges from the nose and appears in the ocular tissue as a metastasis. Herein, we describe a case of NK-cell neoplasm in which the eye was considered to be the primary organ. Case: A 50-year-old female displayed bilateral anterior chamber cells, vitreous opacity, bullous retinal detachment, and multiple white choroidal mass lesions. Although malignant lymphoma or metastatic tumor was suspected, various systemic examinations failed to detect any positive results. A vitrectomy was performed OS; however, histocytological analyses from the vitreous sample showed no definite evidence of malignancy, and IL-10 concentration was low. Enlarged choroidal masses were fused together. Three weeks after the first visit, the patient suddenly developed an attack of fever, night sweat, and hepatic dysfunction, and 5 days later, she passed away due to multiple organ failure. Immunohistochemisty and in situ hybridization revealed the presence of atypical cells positive for CD3, CD56, and Epstein-Barr virus-encoded RNAs, resulting in the diagnosis of NK-cell neoplasm. With the characteristic clinical course, we concluded that this neoplasm was a primary intraocular NK-cell lymphoma. Conclusions: This is the first report to describe primary intraocular NK-cell neoplasm. When we encounter atypical choroidal lesions, we should consider the possibility of NK-cell lymphoma, even though it is a rare disease.

Contribution of interventional radiology to diagnosis and staging of bronchogenic carcinoma

International Nuclear Information System (INIS)

Wittich, G.R.; Jantsch, H.; Sonnenberg, E. van; Karnel, F.; Kumpan, W.; Greene, R.

1986-01-01

The value of percutaneous radiological fine needle biopsy of the thorax will be discussed in relation to sputum cytology, bronchoscopy, mediastinoscopy and open biopsy. Commun indications for fine needle biopsies are the solitary pulmonary nodule, unless it shows definite radiological criteria of a benign lesion, chest wall lesions including Pancoast tumors as well as pulmonary lesions, which were negative on bronchoscopy. Contraindications - in part relative - are coagulopathy, pulmonary arterial and venous hypertension, bullous emphysema, chronic obstructive pulmonary disease, diseases of the lung with an oxygen tension of less than 60 mm Hg and positive pressure mechanical ventilation. Fluoroscopy is the preferred method for localization. CT guidance is used for mediastinal and hilar lesions as well as for pulmonary lesions close to large vessels and for small lesions which are not clearly identified by fluoroscopy in two planes. The sensitivity of fine needle biopsy in the diagnosis of primary lung cancer was 87% in a total of 963 patients. The most common complication was pneumothorax which occurred in 27% of the biopsies guided by fluoroscopy and in 36% of those guided by CT. Catheter drainage of pneumothorax was performed in one third of these patients. Hemoptysis and local parenchymal hemorrhage were found in less than 5% and were without clinical consequence. In addition to technique, results and complications of percutaneous thoracic biopsies, methods of adrenal and liver biopsy in patients with carcinoma of the lung will be discussed. (Author)

Effects of marijuana smoking on the lung.

Science.gov (United States)

Tashkin, Donald P

2013-06-01

Regular smoking of marijuana by itself causes visible and microscopic injury to the large airways that is consistently associated with an increased likelihood of symptoms of chronic bronchitis that subside after cessation of use. On the other hand, habitual use of marijuana alone does not appear to lead to significant abnormalities in lung function when assessed either cross-sectionally or longitudinally, except for possible increases in lung volumes and modest increases in airway resistance of unclear clinical significance. Therefore, no clear link to chronic obstructive pulmonary disease has been established. Although marijuana smoke contains a number of carcinogens and cocarcinogens, findings from a limited number of well-designed epidemiological studies do not suggest an increased risk for the development of either lung or upper airway cancer from light or moderate use, although evidence is mixed concerning possible carcinogenic risks of heavy, long-term use. Although regular marijuana smoking leads to bronchial epithelial ciliary loss and impairs the microbicidal function of alveolar macrophages, evidence is inconclusive regarding possible associated risks for lower respiratory tract infection. Several case reports have implicated marijuana smoking as an etiologic factor in pneumothorax/pneumomediastinum and bullous lung disease, although evidence of a possible causal link from epidemiologic studies is lacking. In summary, the accumulated weight of evidence implies far lower risks for pulmonary complications of even regular heavy use of marijuana compared with the grave pulmonary consequences of tobacco.

Cocaine-induced pulmonary changes: HRCT findings

Directory of Open Access Journals (Sweden)

Renata Rocha de Almeida

2015-08-01

Full Text Available AbstractObjective: To evaluate HRCT scans of the chest in 22 patients with cocaine-induced pulmonary disease.Methods: We included patients between 19 and 52 years of age. The HRCT scans were evaluated by two radiologists independently, discordant results being resolved by consensus. The inclusion criterion was an HRCT scan showing abnormalities that were temporally related to cocaine use, with no other apparent causal factors.Results:In 8 patients (36.4%, the clinical and tomographic findings were consistent with "crack lung", those cases being studied separately. The major HRCT findings in that subgroup of patients included ground-glass opacities, in 100% of the cases; consolidations, in 50%; and the halo sign, in 25%. In 12.5% of the cases, smooth septal thickening, paraseptal emphysema, centrilobular nodules, and the tree-in-bud pattern were identified. Among the remaining 14 patients (63.6%, barotrauma was identified in 3 cases, presenting as pneumomediastinum, pneumothorax, and hemopneumothorax, respectively. Talcosis, characterized as perihilar conglomerate masses, architectural distortion, and emphysema, was diagnosed in 3 patients. Other patterns were found less frequently: organizing pneumonia and bullous emphysema, in 2 patients each; and pulmonary infarction, septic embolism, eosinophilic pneumonia, and cardiogenic pulmonary edema, in 1 patient each.Conclusions: Pulmonary changes induced by cocaine use are varied and nonspecific. The diagnostic suspicion of cocaine-induced pulmonary disease depends, in most of the cases, on a careful drawing of correlations between clinical and radiological findings.

Cocaine-induced pulmonary changes: HRCT findings

International Nuclear Information System (INIS)

Almeida, Renata Rocha de; Zanetti, Glaucia; Marchiori, Edson; Souza, Luciana Soares de; Silva, Jorge Luiz Pereira e; Mancano, Alexandre Dias; Nobre, Luiz Felipe; Hochhegger, Bruno; Marchiori, Edson

2015-01-01

Objective: To evaluate HRCT scans of the chest in 22 patients with cocaine-induced pulmonary disease. Methods: We included patients between 19 and 52 years of age. The HRCT scans were evaluated by two radiologists independently, discordant results being resolved by consensus. The inclusion criterion was an HRCT scan showing abnormalities that were temporally related to cocaine use, with no other apparent causal factors. Results: In 8 patients (36.4%), the clinical and tomographic findings were consistent with 'crack lung', those cases being studied separately. The major HRCT findings in that subgroup of patients included ground-glass opacities, in 100% of the cases; consolidations, in 50%; and the halo sign, in 25%. In 12.5% of the cases, smooth septal thickening, paraseptal emphysema, centrilobular nodules, and the tree-in-bud pattern were identified. Among the remaining 14 patients (63.6%), barotrauma was identified in 3 cases, presenting as pneumomediastinum, pneumothorax, and hemopneumothorax, respectively. Talcosis, characterized as perihilar conglomerate masses, architectural distortion, and emphysema, was diagnosed in 3 patients. Other patterns were found less frequently: organizing pneumonia and bullous emphysema, in 2 patients each; and pulmonary infarction, septic embolism, eosinophilic pneumonia, and cardiogenic pulmonary edema, in 1 patient each. Conclusions: Pulmonary changes induced by cocaine use are varied and nonspecific. The diagnostic suspicion of cocaine-induced pulmonary disease depends, in most of the cases, on a careful drawing of correlations between clinical and radiological findings. (author)

Cocaine-induced pulmonary changes: HRCT findings

Energy Technology Data Exchange (ETDEWEB)

Almeida, Renata Rocha de; Zanetti, Glaucia; Marchiori, Edson, E-mail: edmarchiori@gmail.com [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Programa de Pos-Graduacao em Radiologia; Souza Junior, Arthur Soares [Faculdade de Medicina de Petropolis, Petropolis, RJ (Brazil); Souza, Luciana Soares de [Ultra-X, Sao Jose do Rio Preto, SP (Brazil); Silva, Jorge Luiz Pereira e [Universidade Federal da Bahia (UFBA), Salvador (Brazil). Dep. de Medicina e Apoio Diagnostico; Escuissato, Dante Luiz [Universidade Federal do Parana (UFPR), Curitiba (Brazil). Dept. de Clinica Medica; Irion, Klaus Loureiro [Liverpool Heart and Chest Hospital NHS Foundation Trust, Liverpool (United Kingdom); Mancano, Alexandre Dias [Hospital Anchieta, Taguatinga, DF (Brazil); Nobre, Luiz Felipe [Universidade Federal de Santa Catarina (UFSC), Florianopolis, SC (Brazil); Hochhegger, Bruno [Universidade Federal de Ciencias da Saude de Porto Alegre, Porto Alegre, RS (Brazil); Marchiori, Edson [Universidade Federal Fluminense (UFF), Niteroi, RJ (Brazil)

2015-07-15

Objective: To evaluate HRCT scans of the chest in 22 patients with cocaine-induced pulmonary disease. Methods: We included patients between 19 and 52 years of age. The HRCT scans were evaluated by two radiologists independently, discordant results being resolved by consensus. The inclusion criterion was an HRCT scan showing abnormalities that were temporally related to cocaine use, with no other apparent causal factors. Results: In 8 patients (36.4%), the clinical and tomographic findings were consistent with 'crack lung', those cases being studied separately. The major HRCT findings in that subgroup of patients included ground-glass opacities, in 100% of the cases; consolidations, in 50%; and the halo sign, in 25%. In 12.5% of the cases, smooth septal thickening, paraseptal emphysema, centrilobular nodules, and the tree-in-bud pattern were identified. Among the remaining 14 patients (63.6%), barotrauma was identified in 3 cases, presenting as pneumomediastinum, pneumothorax, and hemopneumothorax, respectively. Talcosis, characterized as perihilar conglomerate masses, architectural distortion, and emphysema, was diagnosed in 3 patients. Other patterns were found less frequently: organizing pneumonia and bullous emphysema, in 2 patients each; and pulmonary infarction, septic embolism, eosinophilic pneumonia, and cardiogenic pulmonary edema, in 1 patient each. Conclusions: Pulmonary changes induced by cocaine use are varied and nonspecific. The diagnostic suspicion of cocaine-induced pulmonary disease depends, in most of the cases, on a careful drawing of correlations between clinical and radiological findings. (author)

The Relationship Between Fatty Liver Disease and Periodontal Disease

Science.gov (United States)

2017-03-22

Periodontitis is a highly prevalent and destructive chronic disease. Numerous studies support an association between periodontal disease and other...destruction seen in periodontal disease. The association between the two diseases has never been investigated. A reasonable mechanism in which periodontal ...disease may play a role in the destruction seen in NAFLD is the remote site infection of periodontal disease. Chewing and oral hygiene measures lead to

Nonalcoholic fatty liver disease - A multisystem disease?

Science.gov (United States)

Mikolasevic, Ivana; Milic, Sandra; Turk Wensveen, Tamara; Grgic, Ivana; Jakopcic, Ivan; Stimac, Davor; Wensveen, Felix; Orlic, Lidija

2016-01-01

Non-alcoholic fatty liver disease (NAFLD) is one of the most common comorbidities associated with overweight and metabolic syndrome (MetS). Importantly, NAFLD is one of its most dangerous complications because it can lead to severe liver pathologies, including fibrosis, cirrhosis and hepatic cellular carcinoma. Given the increasing worldwide prevalence of obesity, NAFLD has become the most common cause of chronic liver disease and therefore is a major global health problem. Currently, NAFLD is predominantly regarded as a hepatic manifestation of MetS. However, accumulating evidence indicates that the effects of NAFLD extend beyond the liver and are negatively associated with a range of chronic diseases, most notably cardiovascular disease (CVD), diabetes mellitus type 2 (T2DM) and chronic kidney disease (CKD). It is becoming increasingly clear that these diseases are the result of the same underlying pathophysiological processes associated with MetS, such as insulin resistance, chronic systemic inflammation and dyslipidemia. As a result, they have been shown to be independent reciprocal risk factors. In addition, recent data have shown that NAFLD actively contributes to aggravation of the pathophysiology of CVD, T2DM, and CKD, as well as several other pathologies. Thus, NAFLD is a direct cause of many chronic diseases associated with MetS, and better detection and treatment of fatty liver disease is therefore urgently needed. As non-invasive screening methods for liver disease become increasingly available, detection and treatment of NAFLD in patients with MetS should therefore be considered by both (sub-) specialists and primary care physicians. PMID:27920470

Cardiovascular diseases

International Nuclear Information System (INIS)

Kodama, Kazunori

1992-01-01

This paper is aimed to discuss the involvement of delayed radiation effects of A-bomb exposure in cardiovascular diseases. First, the relationship between radiation and cardiovascular diseases is reviewed in the literature. Animal experiments have confirmed the relationship between ionizing radiation and vascular lesions. There are many reports which describe ischemic heart disease, cervical and cerebrovascular diseases, and peripheral disease occurring after radiation therapy. The previous A-bomb survivor cohort studies, i.e., the RERF Life Span Study and Adult Health Study, have dealt with the mortality rate from cardiovascular diseases, the prevalence or incidence of cardiovascular diseases, pathological findings, clinical observation of arteriosclerosis, ECG abnormality, blood pressure abnormality, and cardiac function. The following findings have been suggested: (1) A-bomb exposure is likely to be involved in the mortality rate and incidence of ischemic heart disease and cerebrovascular diseases; (2) similarly, the involvement of A-bomb exposure is considered in the prevalence of the arch of aorta; (3) ECG abnormality corresponding to ischemic heart disease may reflect the involvement of A-bomb exposure. To confirm the above findings, further studies are required on the basis of more accurate information and the appropriate number of cohort samples. Little evidence has been presented for the correlation between A-bomb exposure and both rheumatic heart disease and congenital heart disease. (N.K.) 88 refs

Perianal disease, small bowel disease, smoking, prior steroid or early azathioprine/biological therapy are predictors of disease behavior change in patients with Crohn's disease.

Science.gov (United States)

Lakatos, Peter Laszlo; Czegledi, Zsofia; Szamosi, Tamas; Banai, Janos; David, Gyula; Zsigmond, Ferenc; Pandur, Tunde; Erdelyi, Zsuzsanna; Gemela, Orsolya; Papp, Janos; Lakatos, Laszlo

2009-07-28

To assess the combined effect of disease phenotype, smoking and medical therapy [steroid, azathioprine (AZA), AZA/biological therapy] on the probability of disease behavior change in a Caucasian cohort of patients with Crohn's disease (CD). Three hundred and forty well-characterized, unrelated, consecutive CD patients were analyzed (M/F: 155/185, duration: 9.4 +/- 7.5 years) with a complete clinical follow-up. Medical records including disease phenotype according to the Montreal classification, extraintestinal manifestations, use of medications and surgical events were analyzed retrospectively. Patients were interviewed on their smoking habits at the time of diagnosis and during the regular follow-up visits. A change in disease behavior was observed in 30.8% of patients with an initially non-stricturing, non-penetrating disease behavior after a mean disease duration of 9.0 +/- 7.2 years. In a logistic regression analysis corrected for disease duration, perianal disease, smoking, steroid use, early AZA or AZA/biological therapy use were independent predictors of disease behavior change. In a subsequent Kaplan-Meier survival analysis and a proportional Cox regression analysis, disease location (P = 0.001), presence of perianal disease (P < 0.001), prior steroid use (P = 0.006), early AZA (P = 0.005) or AZA/biological therapy (P = 0.002), or smoking (P = 0.032) were independent predictors of disease behavior change. Our data suggest that perianal disease, small bowel disease, smoking, prior steroid use, early AZA or AZA/biological therapy are all predictors of disease behavior change in CD patients.

Celiac disease and new diseases related to gluten

Science.gov (United States)

Jiménez Ortega, Ana Isabel; Martínez García, Rosa María; Quiles Blanco, María José; Majid Abu Naji, Jamil Abdel; González Iglesias, María José

2016-07-12

Celiac disease is the most common chronic intestinal disease. Nowadays it´s known that this is a multisistemic pathology of immune mechanism, triggered by gluten, which occurs in genetically susceptible individuals. It affects approximately 1% of the world population, which is a very high prevalence, affects all age groups and has symptoms both digestive and extra-digestive. Since it is a disease that requires maintaining a gluten-free diet and medical monitoring for life, it is important to know it and establish its diagnosis properly. Along with celiac disease a number of new diseases related to gluten are diagnosed increasingly, including the non celiac gluten sensitivity or wheat allergy. The suffering of celiac disease, or other related diseases, by conditioning diet changes of the affected individual, it may be associated with nutritional imbalances that need to monitor and try to solve. Therefore patients with this problem need special nutritional advice.

EPSTEIN-BARR VIRUS AND CYTOMEGALOVIRUS: IS THEIR ROLE IN PEMPHIGUS REALLY INCIDENTAL? A PRELIMINARY REPORT

Directory of Open Access Journals (Sweden)

N. V. Makhneva

2016-01-01

Full Text Available Background: Epstein-Barr (EBV and cytomegaloviral (CMV infections are among most prevalent in the population worldwide that are associated with autoimmune processes. However, conflicting data of the studies on the role of herpes viral infections in the etiology of autoimmune pemphigus does not allow for reliable recognition of these viruses as triggers in the development and course of this bullous dermatosis. Aim: To assess specific IgM and IgG antibodies to herpes virus infections in patients with autoimmune pemphigus. Materials and methods: Serum samples from 15  patients with autoimmune pemphigus were analyzed by chemoluminescent immunoassay. Results: In the serum samples of 14/15 (93.3% patients with autoimmune pemphigus we found specific IgG antibodies to nuclear and capsid EBV proteins at the levels of 30.7 to 600 U/mL (median, 147.5 [102.62; 313.25] U/mL and from 33.5 to 567 U/mL (median, 186 [85.95; 492.5] U/mL, respectively. Specific IgG antibodies to the EBV early protein were found only in 6.7% of cases. In all patients, there were no specific IgM antibodies to EBV capsule antigens. All patients (100% had specific IgG anti-CMV antibodies in the range from 64.5 to 138 U/mL (median, 103.5 [94.83; 113.75] U/mL. In 30% of cases, there were specific IgM anti-CMV antibodies at titers of 11 to 12.3 U/mL (median, 5 [5; 9.5] U/mL. Conclusion: The results of the preliminary study showed that 93.3%  of autoimmune pemphigus cases have an underlying chronic infection caused by EBV and CMV. The finding of the high titers of IgG anti-EBV and anti-CMV antibodies allows to conclude that the association of these viruses with the bullous dermatosis is not just a chance. It makes further research undoubtedly necessary. Its results would draw more accurate conclusions on the role of EBV and CMV in the pathogenesis of autoimmune pemphigus and to find new perspectives in the treatment of patients with this life-threatening disease.

Eritema multiforme mayor desencadenado por antimicrobianos Big multiform erythema triggered by antimicrobials

Directory of Open Access Journals (Sweden)

Ronaldo de Carvalho Raimundo

2010-03-01

Full Text Available El eritema multiforme, aparece como una enfermedad sistémica con la participación de la piel y las membranas mucosas en relación con varios factores como las infecciones bacterianas o virales, y en particular la administración de drogas, analgésicos y antibióticos en general. Se presenta un paciente masculino de 29 años de edad con eritema multiforme mayor desencadenado por antimicrobianos con la aparición de lesiones vesiculares-bulloso-ulcerosas en las regiones de los labios, encías, la lengua y la mucosa genital en tratamiento de una infección del tracto urinario con norfloxacino 400 mg por una semana. Fue realizado un tratamiento de soporte con el uso de colutorios para la higienización bucal y pomada a base de corticoide para protección de las úlceras, antihistamínicos y orientación nutricional de dieta líquida hipercalórica e hiperproteica. Este síndrome está caracterizado como un proceso eruptivo buloso agudo que compromete la calidad de vida del paciente y no hay pruebas de laboratorio específicas por lo que su diagnóstico debe estar basado en la revisión minuciosa de la anamnesis y en los hallazgos clínicos.The multiform erythema appears as a systemic disease where skin and the mucous membranes have participation in relation to some factors such as bacterial or viral infections and in particular the drugs administration, analgesics and antibiotics in general. The aim of present paper was the presentation of case of big multiform erythema triggering by antimicrobials. Authors present the case of a male patient aged 29 with appearance of ulcerous bullous-vesicular lesions in lips, gums, tongue and genital mucosa under treatment with 400 mg norfloxacin due to urinary tract infection for a week. We made support treatment using mouthwashes for oral hygiene and corticoids ointment for ulcer protection, antihistaminics and nutritional guiding of a hypercaloric and hyperprotein liquid diet. This syndrome is characterized

Refractory disease in autoimmune diseases

NARCIS (Netherlands)

Vasconcelos, Carlos; Kallenberg, Cees; Shoenfeld, Yehuda

Refractory disease (RD) definition has different meanings but it is dynamic, according to knowledge and the availability of new drugs. It should be differentiated from severe disease and damage definitions and it must take into account duration of adequate therapy and compliance of the patient. It

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

A disease state fingerprint for evaluation of Alzheimer's disease

DEFF Research Database (Denmark)

Mattila, Jussi; Koikkalainen, Juha; Virkki, Arho

2011-01-01

Diagnostic processes of Alzheimer's disease (AD) are evolving. Knowledge about disease-specific biomarkers is constantly increasing and larger volumes of data are being measured from patients. To gain additional benefits from the collected data, a novel statistical modeling and data visualization...... interpretation of the information. To model the AD state from complex and heterogeneous patient data, a statistical Disease State Index (DSI) method underlying the DSF has been developed. Using baseline data from the Alzheimer's Disease Neuroimaging Initiative (ADNI), the ability of the DSI to model disease......'s degree of similarity to previously diagnosed disease population. A summary of patient data and results of the computation are displayed in a succinct Disease State Fingerprint (DSF) visualization. The visualization clearly discloses how patient data contributes to the AD state, facilitating rapid...

Dermatological diseases in patients with chronic kidney disease.

Science.gov (United States)

Gagnon1, Amy L; Desai, Tejas

2013-04-01

There are a variety of dermatological diseases that are more commonly seen in patients with chronic kidney disease (CKD) and renal transplants than the general population. Directory of Open Access Journals (DOAJ), Google Scholar, Pubmed (NLM), LISTA (EBSCO) and Web of Science has been searched. Some cutaneous diseases are clearly unique to this population. Of them, Lindsay's Nails, xerosis cutis, dryness of the skin, nephrogenic systemic fibrosis and acquired perforating dermatosis have been described in chronic kidney disease patients. The most common malignancy found in all transplant recipients is non-melanoma skin cancer. It is important for patients and physicians to recognize the manifestations of skin disease in patients suffering from chronic kidney disease to mitigate the morbidity associated with these conditions.

Addison's Disease

Science.gov (United States)

... of potassium and low levels of sodium. What causes Addison’s disease? Addison’s disease is caused by injury to your ... example, a problem with your pituitary gland can cause secondary Addison’s disease. Or, you may develop Addison’s disease if you ...

Heart Diseases

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... 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 ...

Estrogen-related and other disease diagnoses preceding Parkinson's disease

DEFF Research Database (Denmark)

Latourelle, Jeanne C; Dybdal, Merete; Destefano, Anita L

2010-01-01

Estrogen exposure has been associated with the occurrence of Parkinson's disease (PD), as well as many other disorders, and yet the mechanisms underlying these relations are often unknown. While it is likely that estrogen exposure modifies the risk of various diseases through many different...... mechanisms, some estrogen-related disease processes might work in similar manners and result in association between the diseases. Indeed, the association between diseases need not be due only to estrogen-related factors, but due to similar disease processes from a variety of mechanisms....

Polymorphism of the promoter region and exon 1 of the CTLA4 gene in endemic pemphigus foliaceus (fogo selvagem

Directory of Open Access Journals (Sweden)

D.P. Pavoni

2006-09-01

Full Text Available Endemic pemphigus foliaceus (EPF is an autoimmune bullous skin disease characterized by acantholysis and antibodies against a desmosomal protein, desmoglein 1. Genetic and environmental factors contribute to development of this multifactorial disease. HLA class II and some cytokine gene polymorphisms are the only genetic markers thus far known to be associated with susceptibility to or protection from EPF. The cytotoxic T-lymphocyte antigen-4 gene (CTLA4 encodes a key immunoreceptor molecule that regulates and inhibits T-cell proliferation. It participates in the regulatory process controlling autoreactivity and therefore has been considered a strong candidate gene in autoimmune diseases. In the search for genes that might influence EPF pathogenesis, we analyzed variants of the CTLA4 gene in a sample of 118 patients and 291 controls from a Brazilian population. This is the first study investigating the possible role of polymorphisms of the 2q33 chromosomal region in differential susceptibility to pemphigus foliaceus. Promoter region and exon 1 single nucleotide polymorphisms -318 (C,T and 49 (A,G were genotyped using sequence-specific oligonucleotide probes after amplification by the polymerase chain reaction. The allelic and genotypic frequencies did not differ significantly between the patient and the control groups (-318T: 9.8 and 10.9%, 49G: 33.0 and 35.2% were the allelic frequencies in patients and controls, respectively. In addition, no significant difference was found when the patient and control population samples were stratified by the presence of HLA-DRB1 alleles. We conclude that the CTLA4 -318 (C,T and 49 (A,G polymorphisms do not play a major role in EPF development.

Graves' Disease

Science.gov (United States)

... 2011 survey of clinical practice patterns in the management of Graves' disease. Journal of Clinical Endocrinology and Metabolism. 2012 Dec;97( ... 30 a.m. to 5 p.m. eastern time, M-F Follow Us NIH… Turning Discovery Into ... Disease Urologic Diseases Endocrine Diseases Diet & Nutrition ...

Gastroesophageal Reflux Disease in Children with Interstitial Lung Disease.

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Dziekiewicz, M A; Karolewska-Bochenek, K; Dembiński, Ł; Gawronska, A; Krenke, K; Lange, J; Banasiuk, M; Kuchar, E; Kulus, M; Albrecht, P; Banaszkiewicz, A

2016-01-01

Gastroesophageal reflux disease is common in adult patients with interstitial lung disease. However, no data currently exist regarding the prevalence and characteristics of the disease in pediatric patients with interstitial lung disease. The aim of the present study was to prospectively assess the incidence of gastroesophageal reflux disease and characterize its features in children with interstitial lung disease. Gastroesophageal reflux disease was established based on 24 h pH-impedance monitoring (MII-pH). Gastroesophageal reflux episodes (GERs) were classified according to widely recognized criteria as acid, weakly acid, weakly alkaline, or proximal. Eighteen consecutive patients (15 boys, aged 0.2-11.6 years) were enrolled in the study. Gastroesophageal reflux disease was diagnosed in a half (9/18) of children. A thousand GERs were detected by MII-pH (median 53.5; IQR 39.0-75.5). Of these, 585 (58.5 %) episodes were acidic, 407 (40.7 %) were weakly acidic, and eight (0.8 %) were weakly alkaline. There were 637 (63.7 %) proximal GERs. The patients in whom gastroesophageal reflux disease was diagnosed had a significantly higher number of proximal and total GERs. We conclude that the prevalence of gastroesophageal reflux disease in children with interstitial lung disease is high; thus, the disease should be considered regardless of presenting clinical symptoms. A high frequency of non-acid and proximal GERs makes the MII-pH method a preferable choice for the detection of reflux episodes in this patient population.

Disease-modifying drugs in Alzheimer's disease

Directory of Open Access Journals (Sweden)

Ghezzi L

2013-12-01

Full Text Available Laura Ghezzi, Elio Scarpini, Daniela Galimberti Neurology Unit, Department of Pathophysiology and Transplantation, University of Milan, Fondazione Cà Granda, IRCCS Ospedale Maggiore Policlinico, Milan, Italy Abstract: Alzheimer's disease (AD is an age-dependent neurodegenerative disorder and the most common cause of dementia. The early stages of AD are characterized by short-term memory loss. Once the disease progresses, patients experience difficulties in sense of direction, oral communication, calculation, ability to learn, and cognitive thinking. The median duration of the disease is 10 years. The pathology is characterized by deposition of amyloid beta peptide (so-called senile plaques and tau protein in the form of neurofibrillary tangles. Currently, two classes of drugs are licensed by the European Medicines Agency for the treatment of AD, ie, acetylcholinesterase inhibitors for mild to moderate AD, and memantine, an N-methyl-D-aspartate receptor antagonist, for moderate and severe AD. Treatment with acetylcholinesterase inhibitors or memantine aims at slowing progression and controlling symptoms, whereas drugs under development are intended to modify the pathologic steps leading to AD. Herein, we review the clinical features, pharmacologic properties, and cost-effectiveness of the available acetylcholinesterase inhibitors and memantine, and focus on disease-modifying drugs aiming to interfere with the amyloid beta peptide, including vaccination, passive immunization, and tau deposition. Keywords: Alzheimer's disease, acetylcholinesterase inhibitors, memantine, disease-modifying drugs, diagnosis, treatment

Co-localisation of advanced glycation end products and D-β-aspartic acid-containing proteins in gelatinous drop-like corneal dystrophy.

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Kaji, Yuichi; Oshika, Tetsuro; Takazawa, Yutaka; Fukayama, Masashi; Fujii, Noriko

2012-08-01

Gelatinous drop-like corneal dystrophy (GDLD), also known as familial subepithelial corneal amyloidosis, is an autosomal recessive disorder that causes progressive corneal opacity due to accumulation of amyloid fibrils in the corneal stroma. Genetic analyses have revealed that a mutation in membrane component chromosome 1 surface marker 1 gene is responsible for GDLD. However, the mechanism of amyloid formation in the corneal stroma remains unclear. The present study attempted to reveal the role of advanced glycation end products (AGE) and d-amino acids in amyloid formation in GDLD. Informed consent was obtained from five patients with GDLD, three patients with bullous keratopathy and three patients with interstitial keratitis and all the specimens were analysed. Localisation of amyloid fibrils was analysed using Congo-red and thioflavin T staining. In addition, the localisation of AGE (N(ε)-carboxy(methyl)-L-lysine, pyrraline and pentosidine) and D-β-aspartic acid-containing proteins, a major form of d-amino acid-containing proteins, was analysed immunohistochemically. In all GDLD specimens, strong immunoreactivity to AGE and D-β-aspartic acid-containing proteins was detected in the subepithelial amyloid-rich region. In contrast, amyloid fibrils, AGE, or D-amino acid-containing proteins were slightly detected in the corneal stroma of patients with bullous keratopathy and interstitial keratitis. Abnormally accumulated proteins rich in AGE and D-β-aspartic acid co-localise in the amyloid lesions in GDLD. These results indicate that non-enzymatic post-translational modifications of proteins, including AGE formation and isomerisation of aspartyl residues, will be the cause as well as the result of amyloid fibril formations in GDLD.

Perianal disease, small bowel disease, smoking, prior steroid or early azathioprine/biological therapy are predictors of disease behavior change in patients with Crohn’s disease

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Lakatos, Peter Laszlo; Czegledi, Zsofia; Szamosi, Tamas; Banai, Janos; David, Gyula; Zsigmond, Ferenc; Pandur, Tunde; Erdelyi, Zsuzsanna; Gemela, Orsolya; Papp, Janos; Lakatos, Laszlo

2009-01-01

AIM: To assess the combined effect of disease phenotype, smoking and medical therapy [steroid, azathioprine (AZA), AZA/biological therapy] on the probability of disease behavior change in a Caucasian cohort of patients with Crohn’s disease (CD). METHODS: Three hundred and forty well-characterized, unrelated, consecutive CD patients were analyzed (M/F: 155/185, duration: 9.4 ± 7.5 years) with a complete clinical follow-up. Medical records including disease phenotype according to the Montreal classification, extraintestinal manifestations, use of medications and surgical events were analyzed retrospectively. Patients were interviewed on their smoking habits at the time of diagnosis and during the regular follow-up visits. RESULTS: A change in disease behavior was observed in 30.8% of patients with an initially non-stricturing, non-penetrating disease behavior after a mean disease duration of 9.0 ± 7.2 years. In a logistic regression analysis corrected for disease duration, perianal disease, smoking, steroid use, early AZA or AZA/biological therapy use were independent predictors of disease behavior change. In a subsequent Kaplan-Meier survival analysis and a proportional Cox regression analysis, disease location (P = 0.001), presence of perianal disease (P < 0.001), prior steroid use (P = 0.006), early AZA (P = 0.005) or AZA/biological therapy (P = 0.002), or smoking (P = 0.032) were independent predictors of disease behavior change. CONCLUSION: Our data suggest that perianal disease, small bowel disease, smoking, prior steroid use, early AZA or AZA/biological therapy are all predictors of disease behavior change in CD patients. PMID:19630105

[Inpatients days in patients with respiratory diseases and periodontal disease].

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Fernández-Plata, Rosario; Olmedo-Torres, Daniel; Martínez-Briseño, David; González-Cruz, Herminia; Casa-Medina, Guillermo; García-Sancho, Cecilia

2017-01-01

Periodontal disease is a chronic inflammatory gingival process that has been associated with the severity of respiratory diseases. In Mexico a prevalence of 78% was found in population with social security and > 60 years old. The aim of this study is to establish the association between periodontal disease and respiratory diseases according to the inpatient days. A cross-sectional study was conducted from January to December 2011. We included hospitalized patients, ≥ 18 years of age, without sedation or intubated. A dentist classified patients into two groups according to the severity of the periodontal disease: mild-to-moderate and severe. We estimated medians of inpatient days by disease and severity. Negative binomial models were adjusted to estimate incidence rate ratios and predicted inpatient days. 3,059 patients were enrolled. The median of observed and predicted inpatient days was higher in the group of severe periodontal disease (p disease, tuberculosis, and influenza had the highest incidence rates ratios of periodontal disease (p periodontal disease is positively -associated with inpatient days of patients with respiratory diseases.

Vascular aspects of water uptake mechanisms in the toad skin: perfusion, diffusion, confusion

DEFF Research Database (Denmark)

Willumsen, Niels; Viborg, Arne L; Hillyard, Stanley D

2007-01-01

Blood cell flow (BCF) in the water absorbing "seat patch" region of toad skin was measured with laser Doppler flow cytometry. BCF of dehydrated toads increased by a factor of 6-8 when water contact was made and declined gradually as toads rehydrated. Water absorption was initially stimulated...... and declined in parallel with BCF. Water absorption measured during the initial rehydration period did not correlate with BCF and hydrated toads injected with AVT increased water absorption without an increase in BCF indicating the lack of an obligate relation between blood flow and water absorption....... Aquaporins 1-3 were characterized by RT-PCR analysis of seat patch skin. AQP 1 was localized in the endothelium of subepidermal capillaries and serves as a pathway for water absorption in series with the apical and basolateral membranes of the epithelium. Dehydrated toads rehydrated more rapidly from dilute...

Reinke Edema: Watch For Vocal Fold Cysts.

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Tüzüner, Arzu; Demirci, Sule; Yavanoglu, Ahmet; Kurkcuoglu, Melih; Arslan, Necmi

2015-06-01

Reinke edema is one of the common cause of dysphonia middle-aged population, and severe thickening of vocal folds require surgical treatment. Smoking plays a major role on etiology. Vocal fold cysts are also benign lesions and vocal trauma blamed for acquired cysts. We would like to present 3 cases with vocal fold cyst related with Reinke edema. First case had a subepidermal epidermoid cyst with Reinke edema, which could be easily observed before surgery during laryngostroboscopy. Second case had a mucous retention cyst into the edematous Reinke tissue, which was detected during surgical intervention, and third case had a epidermoid cyst that occurred 2 months after before microlaryngeal operation regarding Reinke edema reduction. These 3 cases revealed that surgical management of Reinke edema needs a careful dissection and close follow-up after surgery for presence of vocal fold cysts.

Disease phenotype at diagnosis in pediatric Crohn's disease

DEFF Research Database (Denmark)

de Bie, Charlotte I; Paerregaard, Anders; Kolacek, Sanja

2013-01-01

It has been speculated that pediatric Crohn's disease (CD) is a distinct disease entity, with probably different disease subtypes. We therefore aimed to accurately phenotype newly diagnosed pediatric CD by using the pediatric modification of the Montreal classification, the Paris classification....

Skin diseases: prevalence and predictors of itch and disease severity.

OpenAIRE

Verhoeven, E.W.M.

2009-01-01

Chronic skin diseases are known to be common among the general population. Nevertheless, little research attention has been paid to patients with skin diseases in the general population, and consequently, little is known about the impact of skin diseases on daily life within this population. General definitions of health encompass different dimensions of disease outcome divided in disease severity, accompanying physical symptoms, and psychosocial well-being. These dimensions of disease outcom...

[Parkinson's disease(s): recent insight into genetic factors

NARCIS (Netherlands)

Warrenburg, B.P.C. van de; Scheffer, H.; Heutink, P.; Bloem, B.R.

2007-01-01

In recent years, 5 genes have been identified that are unambiguously associated with genetic forms of Parkinson's disease. These genes probably explain less than 10% of all cases of Parkinson's disease. Clinically, these genetic forms can closely resemble idiopathic Parkinson's disease. Mutation

Coats' disease, Turner syndrome, and von Willebrand disease in a patient with Wildtype Norrie disease pseudoglioma.

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Desai, Rajen U; Saffra, Norman A; Krishna, Rati P; Rosenberg, Steven E

2011-01-01

The authors describe a girl diagnosed as having Coats' disease, Turner syndrome (45X karyotype), and type 1 von Willebrand disease. She tested negative for the Norrie disease pseudoglioma (NDP) gene located on the X-chromosome, which has been suspected of contributing to Coats' disease. Copyright 2010, SLACK Incorporated.

Enucleaton of the right eye due to large choroidal melanoma with simultaneous penetrating cornea transplantation from OD to OS (Case report).

OpenAIRE

E. A. Korchuganova; A. Yu. Slonimskiy,; O. Yu. Yatsenko; M. Yu. Lerner

2013-01-01

There is presentation case report of 75‑year old woman with choroidal melanoma (T3N0M0) on the right eye and failed graft on the left pseudophakic eye with far advanced glaucoma and ARMD. No treatment was given to the leading eye with VA 0,2. VA of OS = 1 / ∞ pr.l.certa; PKP OS in 2008 for pseudophakic bullous keratopathy on the eye with far advanced glaucoma. IOP was normal after previous filtering surgery. After PKP VA = 0,04; clear graft during 2 years; then gradually opacification and vas...

Wound healing in porcine skin following low-output carbon dioxide laser irradiation of the incision

Energy Technology Data Exchange (ETDEWEB)

Robinson, J.K.; Garden, J.M.; Taute, P.M.; Leibovich, S.J.; Lautenschlager, E.P.; Hartz, R.S.

1987-06-01

Wound healing of scalpel incisions to the depth of adipose tissue closed with conventional methods was compared with closure by low-output carbon dioxide laser irradiation. In 3 Pitman-Moore minipigs wound healing was evaluated at intervals from 1 to 90 days by the following methods: clinical variables of wound healing; formation of the basement membrane components bullous pemphigoid antigen, laminin, and fibronectin; and histological evaluation of the regeneration of the epidermis, neovascularization, and elastin and collagen formation. There was no significant difference in healing between wounds closed by the various conventional methods and by the low-output carbon dioxide laser.

Periodontal Disease and Systemic Diseases: An Update for the Clinician.

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John, Vanchit; Alqallaf, Hawra; De Bedout, Tatiana

2016-01-01

A link between periodontal disease and various systemic diseases has been investigated for several years. Interest in unearthing such a link has grown as the health care profession is looking for a better understanding of disease processes and their relationships to periodontal and other oral diseases. The article aims to provide recent information on the relationship between periodontal disease and systemic diseases such as; cardiovascular, respiratory, endocrine, musculoskeletal, and reproductive system related abnormalities.

Occupational skin diseases

DEFF Research Database (Denmark)

Mahler, V; Aalto-Korte, K; Alfonso, J H

2017-01-01

BACKGROUND: Work-related skin diseases (WSD) are caused or worsened by a professional activity. Occupational skin diseases (OSD) need to fulfil additional legal criteria which differ from country to country. OSD range amongst the five most frequently notified occupational diseases (musculoskeletal...... diseases, neurologic diseases, lung diseases, diseases of the sensory organs, skin diseases) in Europe. OBJECTIVE: To retrieve information and compare the current state of national frameworks and pathways to manage patients with occupational skin disease with regard to prevention, diagnosis, treatment...... in Science and Technology (COST) Action TD 1206 (StanDerm) (www.standerm.eu). RESULTS: Besides a national health service or a statutory health insurance, most European member states implemented a second insurance scheme specifically geared at occupational diseases [insurance against occupational risks...

Lysosomal storage diseases

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Ferreira, Carlos R.; Gahl, William A.

2016-01-01

Lysosomes are cytoplasmic organelles that contain a variety of different hydrolases. A genetic deficiency in the enzymatic activity of one of these hydrolases will lead to the accumulation of the material meant for lysosomal degradation. Examples include glycogen in the case of Pompe disease, glycosaminoglycans in the case of the mucopolysaccharidoses, glycoproteins in the cases of the oligosaccharidoses, and sphingolipids in the cases of Niemann-Pick disease types A and B, Gaucher disease, Tay-Sachs disease, Krabbe disease, and metachromatic leukodystrophy. Sometimes, the lysosomal storage can be caused not by the enzymatic deficiency of one of the hydrolases, but by the deficiency of an activator protein, as occurs in the AB variant of GM2 gangliosidosis. Still other times, the accumulated lysosomal material results from failed egress of a small molecule as a consequence of a deficient transporter, as in cystinosis or Salla disease. In the last couple of decades, enzyme replacement therapy has become available for a number of lysosomal storage diseases. Examples include imiglucerase, taliglucerase and velaglucerase for Gaucher disease, laronidase for Hurler disease, idursulfase for Hunter disease, elosulfase for Morquio disease, galsulfase for Maroteaux-Lamy disease, alglucosidase alfa for Pompe disease, and agalsidase alfa and beta for Fabry disease. In addition, substrate reduction therapy has been approved for certain disorders, such as eliglustat for Gaucher disease. The advent of treatment options for some of these disorders has led to newborn screening pilot studies, and ultimately to the addition of Pompe disease and Hurler disease to the Recommended Uniform Screening Panel (RUSP) in 2015 and 2016, respectively. PMID:29152458

Pick disease

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Semantic dementia; Dementia - semantic; Frontotemporal dementia; FTD; Arnold Pick disease; 3R tauopathy ... doctors tell Pick disease apart from Alzheimer disease. (Memory loss is often the main, and earliest, symptom ...

Prevalence of coeliac disease in Italian patients affected by Addison's disease.

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Biagi, Federico; Campanella, Jonia; Soriani, Alessandra; Vailati, Alberto; Corazza, Gino R

2006-03-01

It is well known that coeliac disease is associated with autoimmune endocrine diseases, such as autoimmune thyroid disease and insulin-dependent diabetes mellitus. Recently, coeliac disease has been shown in approximately 10% of patients with autoimmune Addison's disease. Addison's disease is the most common cause of primary adrenocortical insufficiency and it shares several clinical features with coeliac disease. Although hyperpigmentation and hypotension are the most specific signs, gastrointestinal symptoms are common and can be the first complaints of the patients. The aim of our study was to investigate the prevalence of coeliac disease in Italian patients with Addison's disease. Seventeen consecutive patients affected by Addison's disease (14 F, mean age 53.9 years, range 26-79 years) were enrolled in the study. Eleven of them were affected by Addison's disease associated with autoimmune thyroid disease and/or insulin-dependent diabetes mellitus; the other 6 patients were suffering from isolated Addison's disease. Diagnosis had been performed at the age of 40.5 years (range 23-55). Steroid treatment had already been started in 16 of the patients. Endomysial antibodies were tested in all of them and a duodenal biopsy was taken in those found to be positive for antiendomysial antibody (EMA). One out of 17 patients was found to be EMA positive. Duodenal biopsy confirmed the diagnosis of coeliac disease by showing subtotal villous atrophy. Although we studied only a small sample, our preliminary results confirmed that Addison's disease is associated with coeliac disease, being present in 5.9% of patients with Addison's disease. Since the symptoms can be similar and treatment of Addison's disease can mask coeliac disease, this association should always be actively investigated.

A disease state fingerprint for evaluation of Alzheimer's disease

DEFF Research Database (Denmark)

Mattila, Jussi; Koikkalainen, Juha; Virkki, Arho

2011-01-01

Diagnostic processes of Alzheimer's disease (AD) are evolving. Knowledge about disease-specific biomarkers is constantly increasing and larger volumes of data are being measured from patients. To gain additional benefits from the collected data, a novel statistical modeling and data visualization...... interpretation of the information. To model the AD state from complex and heterogeneous patient data, a statistical Disease State Index (DSI) method underlying the DSF has been developed. Using baseline data from the Alzheimer's Disease Neuroimaging Initiative (ADNI), the ability of the DSI to model disease...

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.

BULLOUS DEGENERATION OF THE LEFT LOWER LOBE IN A HEROIN-ADDICT

NARCIS (Netherlands)

SMEENK, FWJM; SERLIE, J; VANDERJAGT, EJ; POSTMUS, PE

1990-01-01

A 34 yr old heroin addict was referred because of chest pain caused by air-trapping in a bulla in the left lower lobe. There was a marked difference between the functional residual capacity measured by body-plethysmography and helium dilution. A slow wash-in and wash-out were demonstrated by

Heavy Chain Diseases

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... of heavy chain produced: Alpha Gamma Mu Alpha Heavy Chain Disease Alpha heavy chain disease (IgA heavy ... the disease or lead to a remission. Gamma Heavy Chain Disease Gamma heavy chain disease (IgG heavy ...

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.

Prion Diseases

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... with facebook share with twitter share with linkedin Prion Diseases Prion diseases are a related group of ... deer and elk. Why Is the Study of Prion Diseases a Priority for NIAID? Much about TSE ...

Diabetic Eye Disease

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... 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 ...

Infectious Diseases

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... But some of them can make you sick. Infectious diseases are diseases that are caused by germs. There ... many different ways that you can get an infectious disease: Through direct contact with a person who is ...

Heart Disease, Stroke, or Other Cardiovascular Disease and Adult Vaccination

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... Adult Diseases Resources Heart Disease, Stroke, or Other Cardiovascular Disease and Adult Vaccination Language: English (US) Español (Spanish) ... important step in staying healthy. If you have cardiovascular disease, talk with your doctor about getting your vaccinations ...

Association of Relationship between Periodontal Disease and Cardiovascular Disease.

Science.gov (United States)

Johar, N; Dhodapkar, S V; Kumar, R; Verma, T; Jajoo, A

2017-04-01

The present study was undertaken to determine the relationship between periodontal and cardiovascular disease. Previous studies have shown some co-relation between the two conditions. We included 186 patients divided into four groups. First two Groups (A1 & A2) were the patients with cardiac disease (100 in numbers) whilst Groups (B1 & B2) (86 in numbers) were treated as controls (without cardiac disease). Following markers of periodontal disease were assessed - plaque index, calculus index, gingival and periodontal index. Markers of cardiovascular disease included were LDL, HDL, total cholesterol and CRP. Ramfjords periodontal index was used to assess the extent of periodontal disease. In the present study there was a significant increase in CRP levels in Group A1 (CVD + PD) compared to controls and overall the two cardiac groups showed a significant increase in CRP compared to controls. There was a non-significant change in lipid profile markers (LDL, HDL and total cholesterol). Periodontal Disease Index (PDI) was also increased in Group A1 compared to other groups except Group B1 and overall in cardiac groups compared to non-cardiac (PD) groups. In this study no correlation between periodontal and cardiovascular disease was found. This may be due intake of statins by few patients in Group A with a confirmed diagnosis of cardiovascular disease.

Prostate Diseases

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... Home › Aging & Health A to Z › Prostate Diseases Font size A A A Print Share Glossary Basic ... body. Approximately 3 million American men have some type of prostate disease. The most common prostate diseases ...

Periodontal Diseases

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... diseases. The primary research focus was on oral bacteria. Periodontal diseases were thought to begin when chalky white ... tools to target their treatment specifically to the bacteria that trigger periodontal disease. At the same time, because biofilms form ...

Chronic Inflammatory Diseases and Atherosclerotic Cardiovascular Disease

DEFF Research Database (Denmark)

Hansen, Peter Riis

2018-01-01

Inflammation plays a significant role in atherosclerosis and cardiovascular disease (CVD). Patients with chronic inflammatory diseases are at increased risk of CVD, but it is debated whether this association is causal or dependent on shared risk factors, other exposures, genes, and/or inflammatory...... pathways. The current review summarizes epidemiological, clinical, and experimental data supporting the role of shared inflammatory mechanisms between atherosclerotic CVD and rheumatoid arthritis, psoriasis, inflammatory bowel disease, and periodontitis, respectively, and provides insights to future...... prospects in this area of research. Awareness of the role of inflammation in CVD in patients with chronic inflammatory diseases and the potential for anti-inflammatory therapy, e.g., with tumor necrosis factor-α inhibitors, to also reduce atherosclerotic CVD has evolved into guideline- based recommendations...

Experimental Human Cell and Tissue Models of Pemphigus

Science.gov (United States)

van der Wier, Gerda; Pas, Hendri H.; Jonkman, Marcel F.

2010-01-01

Pemphigus is a chronic mucocutaneous autoimmune bullous disease that is characterized by loss of cell-cell contact in skin and/or mucous membranes. Past research has successfully identified desmosomes as immunological targets and has demonstrated that acantholysis is initiated through direct binding of IgG. The exact mechanisms of acantholysis, however, are still missing. Experimental model systems have contributed considerably to today's knowledge and are still a favourite tool of research. In this paper we will describe to what extent human cell and tissue models represent the in vivo situation, for example, organ cultures of human skin, keratinocyte cultures, and human skin grafted on mice and, furthermore, how suitable they are to study the pathogenesis of pemphigus. Organ cultures closely mimic the architecture of the epidermis but are less suitable to answer posed biochemical questions. Cultured keratinocyte monolayers are convenient in this respect, but their desmosomal make-up in terms of adhesion molecules does not exactly reflect the in vivo situation. Reconstituted skin is a relatively new model that approaches organ culture. In models of human skin grafted on mice, acantholysis can be studied in actual human skin but now with all the advantages of an animal model. PMID:20585596

Ultrasound biomicroscopy as a tool for conjunctiva and eyelids evaluation

Directory of Open Access Journals (Sweden)

V. N. Trubilin

2015-01-01

Full Text Available Currently, ultrasound biomicroscopy (UBM is the only diagnostic method that uses a 50‑100 MHz transducer with a depth of penetration of 4 mm. This provides greater sensitivity and resolution.Aim. To visualize meibomian glands and to evaluate their morphological and functional status using UBM.Methods. UBM was performed in 14 patients aged 29‑81 with obvious meibomian gland dysfunction (MGD and healthy meibomian glands. 6 patients had no biomicroscopic signs of blepharitis while 8 patients had biomicroscopic signs of blepharitis, blepharoconjunctivitis, and MGD.Results. UBM provides detailed information on meibomian glands and eyelid margins and their pathological conditions, i.e., eyelid margin thickening due to inflammation, meibomian gland orifice obstruction, increase in distance between meibomian glands due to their atrophy, and cartilaginous tissue destruction. UBM findings may depend on patient age as well as on disease stage and severity. When re-positioning UBM transducer, bullous conjunctiva and subconjunctival cysts can be visualized. This provides differential diagnosis between opaque cysts and tumors.Conclusion. UBM combined with standard exams increases information value, reliability, and accuracy of the diagnostics of anterior segment disorders and facilitates the development of targeted therapeutic approaches. Further studies on diagnostic value of conjunctiva and eyelids UBM are required.

The lysosomal storage disease continuum with ageing-related neurodegenerative disease.

Science.gov (United States)

Lloyd-Evans, Emyr; Haslett, Luke J

2016-12-01

Lysosomal storage diseases and diseases of ageing share many features both at the physiological level and with respect to the mechanisms that underlie disease pathogenesis. Although the exact pathophysiology is not exactly the same, it is astounding how many similar pathways are altered in all of these diseases. The aim of this review is to provide a summary of the shared disease mechanisms, outlining the similarities and differences and how genetics, insight into rare diseases and functional research has changed our perspective on the causes underlying common diseases of ageing. The lysosome should no longer be considered as just the stomach of the cell or as a suicide bag, it has an emerging role in cellular signalling, nutrient sensing and recycling. The lysosome is of fundamental importance in the pathophysiology of diseases of ageing and by comparing against the LSDs we not only identify common pathways but also therapeutic targets so that ultimately more effective treatments can be developed for all neurodegenerative diseases. Copyright © 2016. Published by Elsevier B.V.

Periodontal disease and non-communicable diseases. Strength of bidirectional associations

OpenAIRE

Kassier, SM

2016-01-01

Periodontal disease (PD), along with cardiovascular and circulatory disease, diabetes mellitus, chronic respiratory disease and obesity, are globally regarded as some of the major non-communicable diseases (NCDs). The association between PD and these systemic illnesses is described as bidirectional. Gaining an understanding of the strength of the proposed associations between these diseases is important, as it will enable health professionals to identify common risk factors that will allow fo...

Dent disease

Directory of Open Access Journals (Sweden)

Rina R Rus

2017-04-01

Full Text Available Dent disease is an x-linked disorder of proximal renal tubular dysfunction that occurs almost exclusively in males. It is characterized by significant, mostly low molecular weight proteinuria, hypercalciuria, nephrocalcinosis, nephrolithiasis, and chronic kidney disease. Signs and symptoms of this condition appear in early childhood and worsen over time. There are two forms of Dent disease, which are distinguished by their genetic cause and pattern of signs and symptoms (type 1 and type 2. Dent disease 2 is characterized by the features described above and also associated with extrarenal abnormalities (they include mild intellectual disability, hypotonia, and cataract. Some researchers consider Dent disease 2 to be a mild variant of a similar disorder called Lowe syndrome.We represent a case of a 3-year old boy with significant proteinuria in the nephrotic range and hypercalciuria. We confirmed Dent disease type 1 by genetic analysis.

Prevalence of periodontal disease, its association with systemic diseases and prevention.

Science.gov (United States)

Nazir, Muhammad Ashraf

2017-01-01

Periodontal diseases are prevalent both in developed and developing countries and affect about 20-50% of global population. High prevalence of periodontal disease in adolescents, adults, and older individuals makes it a public health concern. Several risk factors such as smoking, poor oral hygiene, diabetes, medication, age, hereditary, and stress are related to periodontal diseases. Robust evidence shows the association of periodontal diseases with systemic diseases such as cardiovascular disease, diabetes, and adverse pregnancy outcomes. Periodontal disease is likely to cause 19% increase in the risk of cardiovascular disease, and this increase in relative risk reaches to 44% among individuals aged 65 years and over. Type 2 diabetic individuals with severe form of periodontal disease have 3.2 times greater mortality risk compared with individuals with no or mild periodontitis. Periodontal therapy has been shown to improve glycemic control in type 2 diabetic subjects. Periodontitis is related to maternal infection, preterm birth, low birth weight, and preeclampsia. Oral disease prevention strategies should be incorporated in chronic systemic disease preventive initiatives to curtail the burden of disease in populations. The reduction in the incidence and prevalence of periodontal disease can reduce its associated systemic diseases and can also minimize their financial impact on the health-care systems. It is hoped that medical, dental practitioners, and other health-care professionals will get familiar with perio-systemic link and risk factors, and need to refer to the specialized dental or periodontal care.

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...

TEMPORAL ORDER OF DISEASE PAIRS AFFECTS SUBSEQUENT DISEASE TRAJECTORIES

DEFF Research Database (Denmark)

Beck, Mette K; Westergaard, David; Jensen, Anders Boeck

2016-01-01

order of appearance. We discuss these different types of disease co-occurrences, and use the two diseases "sleep apnea" and "diabetes" to showcase the approach which otherwise can be applied to any disease pair. We benefit from seven million electronic medical records covering the entire population...... of Denmark for more than 20 years. Sleep apnea is the most common sleep-related breathing disorder and it has previously been shown to be bidirectionally linked to diabetes, meaning that each disease increases the risk of acquiring the other. We confirm that there is no significant temporal relationship......, as approximately half of patients with both diseases are diagnosed with diabetes first. However, we also show that patients diagnosed with diabetes before sleep apnea have a higher disease burden compared to patients diagnosed with sleep apnea before diabetes. The study clearly demonstrates that it is not only...

Coronary heart disease

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Heart disease, Coronary heart disease, Coronary artery disease; Arteriosclerotic heart disease; CHD; CAD ... buildup of plaque in the arteries to your heart. This may also be called hardening of the ...

Autoinflammatory Diseases

International Nuclear Information System (INIS)

Penaranda P, Edgar; Spinel B, Nestor; Restrepo, Jose F; Rondon H, Federico; Millan S, Alberto; Iglesias G Antonio

2010-01-01

We present a review article on the autoinflammatory diseases, narrating its historical origin and describing the protein and molecular structure of the Inflammasome, the current classification of the autoinflammatory diseases and a description of the immuno genetics and clinical characteristics more important of every disease.

Niemann-Pick disease

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NPD; Sphingomyelinase deficiency; Lipid storage disorder - Niemann-Pick disease; Lysosomal storage disease - Niemann-Pick ... lipofuscinoses or Batten disease (Wolman disease, cholesteryl ... metabolism of lipids. In: Kliegman RM, Stanton BF, St. Geme JW, ...

Chronic pulmonary disease - a multifacted disease complex in the horse

International Nuclear Information System (INIS)

Clarke, A.F.

1987-01-01

This paper reviews chronic pulmonary disease (CPD) as an insidiously developing disease capable of being manifest in many degrees. Horses may suffer mild, sub-clinical degrees of lower respiratory tract inflammation or small airway disease withouth showing symptoms at rest. This form of disease becomes manifest as poor performance when these horses take part in athletic competition. Factors relating to the aetiology, diagnosis, treatment and prevention of all degrees of small airway disease of horses are discussed. 30 refs

Gaucher disease

Science.gov (United States)

... please enable JavaScript. Gaucher disease is a rare genetic disorder in which a person lacks an enzyme called glucocerebrosidase (GBA). Causes Gaucher disease is rare in the general population. People of Eastern and Central European (Ashkenazi) Jewish heritage are more likely to have this disease. It ...

Disease Burden of 32 Infectious Diseases in the Netherlands, 2007-2011.

Directory of Open Access Journals (Sweden)

Alies van Lier

Full Text Available Infectious disease burden estimates provided by a composite health measure give a balanced view of the true impact of a disease on a population, allowing the relative impact of diseases that differ in severity and mortality to be monitored over time. This article presents the first national disease burden estimates for a comprehensive set of 32 infectious diseases in the Netherlands.The average annual disease burden was computed for the period 2007-2011 for selected infectious diseases in the Netherlands using the disability-adjusted life years (DALY measure. The pathogen- and incidence-based approach was adopted to quantify the burden due to both morbidity and premature mortality associated with all short and long-term consequences of infection. Natural history models, disease progression probabilities, disability weights, and other parameters were adapted from previous research. Annual incidence was obtained from statutory notification and other surveillance systems, which was corrected for under-ascertainment and under-reporting. The highest average annual disease burden was estimated for invasive pneumococcal disease (9444 DALYs/year; 95% uncertainty interval [UI]: 8911-9961 and influenza (8670 DALYs/year; 95% UI: 8468-8874, which represents 16% and 15% of the total burden of all 32 diseases, respectively. The remaining 30 diseases ranked by number of DALYs/year from high to low were: HIV infection, legionellosis, toxoplasmosis, chlamydia, campylobacteriosis, pertussis, tuberculosis, hepatitis C infection, Q fever, norovirus infection, salmonellosis, gonorrhoea, invasive meningococcal disease, hepatitis B infection, invasive Haemophilus influenzae infection, shigellosis, listeriosis, giardiasis, hepatitis A infection, infection with STEC O157, measles, cryptosporidiosis, syphilis, rabies, variant Creutzfeldt-Jakob disease, tetanus, mumps, rubella, diphtheria, and poliomyelitis. The very low burden for the latter five diseases can be

Disease Burden of 32 Infectious Diseases in the Netherlands, 2007-2011

Science.gov (United States)

Bouwknegt, Martijn; Kretzschmar, Mirjam E.; Mangen, Marie-Josée J.; Wallinga, Jacco; de Melker, Hester E.

2016-01-01

Background Infectious disease burden estimates provided by a composite health measure give a balanced view of the true impact of a disease on a population, allowing the relative impact of diseases that differ in severity and mortality to be monitored over time. This article presents the first national disease burden estimates for a comprehensive set of 32 infectious diseases in the Netherlands. Methods and Findings The average annual disease burden was computed for the period 2007–2011 for selected infectious diseases in the Netherlands using the disability-adjusted life years (DALY) measure. The pathogen- and incidence-based approach was adopted to quantify the burden due to both morbidity and premature mortality associated with all short and long-term consequences of infection. Natural history models, disease progression probabilities, disability weights, and other parameters were adapted from previous research. Annual incidence was obtained from statutory notification and other surveillance systems, which was corrected for under-ascertainment and under-reporting. The highest average annual disease burden was estimated for invasive pneumococcal disease (9444 DALYs/year; 95% uncertainty interval [UI]: 8911–9961) and influenza (8670 DALYs/year; 95% UI: 8468–8874), which represents 16% and 15% of the total burden of all 32 diseases, respectively. The remaining 30 diseases ranked by number of DALYs/year from high to low were: HIV infection, legionellosis, toxoplasmosis, chlamydia, campylobacteriosis, pertussis, tuberculosis, hepatitis C infection, Q fever, norovirus infection, salmonellosis, gonorrhoea, invasive meningococcal disease, hepatitis B infection, invasive Haemophilus influenzae infection, shigellosis, listeriosis, giardiasis, hepatitis A infection, infection with STEC O157, measles, cryptosporidiosis, syphilis, rabies, variant Creutzfeldt-Jakob disease, tetanus, mumps, rubella, diphtheria, and poliomyelitis. The very low burden for the latter five

Disease Burden of 32 Infectious Diseases in the Netherlands, 2007-2011.

Science.gov (United States)

van Lier, Alies; McDonald, Scott A; Bouwknegt, Martijn; Kretzschmar, Mirjam E; Havelaar, Arie H; Mangen, Marie-Josée J; Wallinga, Jacco; de Melker, Hester E

2016-01-01

Infectious disease burden estimates provided by a composite health measure give a balanced view of the true impact of a disease on a population, allowing the relative impact of diseases that differ in severity and mortality to be monitored over time. This article presents the first national disease burden estimates for a comprehensive set of 32 infectious diseases in the Netherlands. The average annual disease burden was computed for the period 2007-2011 for selected infectious diseases in the Netherlands using the disability-adjusted life years (DALY) measure. The pathogen- and incidence-based approach was adopted to quantify the burden due to both morbidity and premature mortality associated with all short and long-term consequences of infection. Natural history models, disease progression probabilities, disability weights, and other parameters were adapted from previous research. Annual incidence was obtained from statutory notification and other surveillance systems, which was corrected for under-ascertainment and under-reporting. The highest average annual disease burden was estimated for invasive pneumococcal disease (9444 DALYs/year; 95% uncertainty interval [UI]: 8911-9961) and influenza (8670 DALYs/year; 95% UI: 8468-8874), which represents 16% and 15% of the total burden of all 32 diseases, respectively. The remaining 30 diseases ranked by number of DALYs/year from high to low were: HIV infection, legionellosis, toxoplasmosis, chlamydia, campylobacteriosis, pertussis, tuberculosis, hepatitis C infection, Q fever, norovirus infection, salmonellosis, gonorrhoea, invasive meningococcal disease, hepatitis B infection, invasive Haemophilus influenzae infection, shigellosis, listeriosis, giardiasis, hepatitis A infection, infection with STEC O157, measles, cryptosporidiosis, syphilis, rabies, variant Creutzfeldt-Jakob disease, tetanus, mumps, rubella, diphtheria, and poliomyelitis. The very low burden for the latter five diseases can be attributed to the

Parkinson's Disease Dementia

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... 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 ...

[Emerging noninfectious diseases].

Science.gov (United States)

Consiglio, Ezequiel

2008-11-01

In recent years, emerging diseases were defined as being infectious, acquiring high incidence, often suddenly, or being a threat or an unexpected phenomenon. This study discusses the hallmarks of emerging diseases, describing the existence of noninfectious emerging diseases, and elaborating on the advantages of defining noninfectious diseases as emerging ones. From the discussion of various mental health disorders, nutritional deficiencies, external injuries and violence outcomes, work injuries and occupational health, and diseases due to environmental factors, the conclusion is drawn that a wide variety of noninfectious diseases can be defined as emergent. Noninfectious emerging diseases need to be identified in order to improve their control and management. A new definition of "emergent disease" is proposed, one that emphasizes the pathways of emergence and conceptual traits, rather than descriptive features.

Coronary heart disease after radiotherapy for peptic ulcer disease

International Nuclear Information System (INIS)

Carr, Zhanat A.; Land, Charles E.; Kleinerman, Ruth A.; Weinstock, Robert W.; Stovall, Marilyn; Griem, Melvin L.; Mabuchi, Kiyohiko

2005-01-01

Purpose: To evaluate the risk of coronary heart disease (CHD) and cerebrovascular disease after radiotherapy (RT) for peptic ulcer disease. Methods and materials: Peptic ulcer disease patients treated with RT (n = 1859) or by other means (n = 1860) at the University of Chicago Medical Center between 1936 and 1965, were followed through 1997. The observed numbers of cause-specific deaths were compared with the expected numbers from the general population rates. During RT, 5% of the heart was in the treatment field and the remainder of the heart mostly received scattered radiation. A volume-weighted cardiac dose was computed to describe the average tissue dose to the entire organ. We used Cox proportional hazards regression analysis to analyze the CHD and cerebrovascular disease risk associated with RT, adjusting for confounding factors. Results: Greater than expected CHD mortality was observed among the irradiated patients. The irradiated patients received volume-weighted cardiac doses ranging from 1.6 to 3.9 Gy and the portion of the heart directly in the field received doses of 7.6-18.4 Gy. The CHD risk increased with the cardiac dose (p trend = 0.01). The cerebrovascular disease risk was not associated with the surrogate carotid dose. Conclusion: The excess CHD risk in patients undergoing RT for peptic ulcer disease decades previously indicates the need for long-term follow-up for cardiovascular disease after chest RT

Rheumatic heart disease: infectious disease origin, chronic care approach.

Science.gov (United States)

Katzenellenbogen, Judith M; Ralph, Anna P; Wyber, Rosemary; Carapetis, Jonathan R

2017-11-29

Rheumatic heart disease (RHD) is a chronic cardiac condition with an infectious aetiology, causing high disease burden in low-income settings. Affected individuals are young and associated morbidity is high. However, RHD is relatively neglected due to the populations involved and its lower incidence relative to other heart diseases. In this narrative review, we describe how RHD care can be informed by and integrated with models of care developed for priority non-communicable diseases (coronary heart disease), and high-burden communicable diseases (tuberculosis). Examining the four-level prevention model (primordial through tertiary prevention) suggests primordial and primary prevention of RHD can leverage off existing tuberculosis control efforts, given shared risk factors. Successes in coronary heart disease control provide inspiration for similarly bold initiatives for RHD. Further, we illustrate how the Chronic Care Model (CCM), developed for use in non-communicable diseases, offers a relevant framework to approach RHD care. Systems strengthening through greater integration of services can improve RHD programs. Strengthening of systems through integration/linkages with other well-performing and resourced services in conjunction with policies to adopt the CCM framework for the secondary and tertiary prevention of RHD in settings with limited resources, has the potential to significantly reduce the burden of RHD globally. More research is required to provide evidence-based recommendations for policy and service design.

Associated Autoimmune Diseases

Science.gov (United States)

... gland in the neck, thick and coarse hair. Addison’s Disease Arare disease involving the adrenal gland. The prevalence of celiac disease in people with addison’s disease is significant. Symptoms of Addison’s may include weight ...

Mad Cow Disease

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... Safe Videos for Educators Search English Español Mad Cow Disease KidsHealth / For Teens / Mad Cow Disease What's ... are people to get it? What Is Mad Cow Disease? Mad cow disease is an incurable, fatal ...

Skin Diseases: Skin Health and Skin Diseases

Science.gov (United States)

Skip Navigation Bar Home Current Issue Past Issues Skin Diseases Skin Health and Skin Diseases Past Issues / Fall 2008 Table of Contents ... acne to wrinkles Did you know that your skin is the largest organ of your body? It ...

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…

Wilson’s Disease: An Inherited, Silent, Copper Intoxication Disease

Directory of Open Access Journals (Sweden)

Uta Merle

2016-07-01

Full Text Available Wilson’s disease is a rare, autosomal recessive, genetic, copper overload disease, which evokes multiple motor or neuropsychiatric symptoms and liver disease. It is the consequence of a variety of different mutations affecting the ATP7B gene. This gene encodes for a class IB, P-type, copper-transporting ATPase, which is located in the trans-Golgi network of the liver and brain, and mediates the excretion of excess copper into the bile. When functionally inactive, the excess copper is deposited in the liver, brain, and other tissues. Free copper induces oxidative stress, lipid peroxidation, and lowers the apoptotic threshold of the cell. The symptoms in affected persons can vary widely and usually appear between the ages of 6 years and 20 years, but there are also cases in which the disease manifests in advanced age. In this review, we discuss the considerations in diagnosis, clinical management, and treatment of Wilson’s disease. In addition, we highlight experimental efforts that address the pathogenesis of Wilson’s disease in ATP7B deficient mice, novel analytical techniques that will improve the diagnosis at an early stage of disease onset, and treatment results with copper-chelating agents.

Celiac Disease: Diagnosis.

Science.gov (United States)

Byrne, Greg; Feighery, Conleth F

2015-01-01

Historically the diagnosis of celiac disease has relied upon clinical, serological, and histological evidence. In recent years the use of sensitive serological methods has meant an increase in the diagnosis of celiac disease. The heterogeneous nature of the disorder presents a challenge in the study and diagnosis of the disease with patients varying from subclinical or latent disease to patients with overt symptoms. Furthermore the related gluten-sensitive disease dermatitis herpetiformis, while distinct in some respects, shares clinical and serological features with celiac disease. Here we summarize current best practice for the diagnosis of celiac disease and briefly discuss newer approaches. The advent of next-generation assays for diagnosis and newer clinical protocols may result in more sensitive screening and ultimately the possible replacement of the intestinal biopsy as the gold standard for celiac disease diagnosis.

Wireless Monitoring for Patients with Cardiovascular Diseases and Parkinson's Disease.

Science.gov (United States)

Kefaliakos, Antonios; Pliakos, Ioannis; Charalampidou, Martha; Diomidous, Marianna

2016-01-01

The use of applications for mobile devices and wireless sensors is common for the sector of telemedicine. Recently various studies and systems were developed in order to help patients suffering from severe diseases such as cardiovascular diseases and Parkinson's disease. They present a challenge for the sector because such systems demand the flow of accurate data in real time and the use of specialized sensors. In this review will be presented some very interesting applications developed for patients with cardiovascular diseases and Parkinson's disease.

Zmiany skórne w przebiegu ostrej białaczki szpikowej – opis przypadku

Directory of Open Access Journals (Sweden)

Waldemar Placek

2011-08-01

Full Text Available Introduction. Acute myeloid leukaemia is a malignant neoplastic diseaseof white blood cells. About 80% of acute leukaemias in adult arediagnosed as myeloid leukaemias. The presence of transformed cellclones in bone marrow and blood, which originate from very earlystages of myelopoiesis, is characteristic. Beside systemic symptoms(anaemia, thrombocytopenia there are infiltrations in other organs,including skin. Skin lesions are most frequently non-specific (pruritus,skin spots, erythema multiforme, bullous pemphigoid but can be alsospecific (skin tumours. Cases of diffuse xanthomatoses and myeloidsarcoma have also been found in patients with acute myeloidleukaemia. Skin lesions are present more often in lymphocytic thanmyeloid leukaemias. They may precede the disease or its relapse, occurconcomitantly or after systemic symptoms, and sometimes may be ofprognostic value.Objective. To present the case of a patient in whom skin lesions werethe cause of detailed examinations, which revealed acute myeloidleukaemia.Case report. We present a case of a 61-year old patient with purplebrownnodular lesions 1-3 cm in diameter on the face, scalp skin, trunk,limbs and the oral cavity’s mucous membranes with coexistent intensepruritus and generalized lymphadenopathy. The first skin lesionsoccurred three months earlier. During hospitalization in the Departmentof Dermatology the renewal of blasts in the circulatory blood wasobserved and skin biopsy revealed a large amount of mononuclearcells. The patient was directed to the haematological ward, where thediagnosis of acute myeloid leukaemia was established.Conclusions. Our case indicates that the presence of skin lesions andthorough diagnostic procedures may contribute to the diagnosis ofmalignant neoplastic diseases of white blood cells.

Wilson’s Disease

Directory of Open Access Journals (Sweden)

Figen Hanağası

2013-12-01

Full Text Available Wilson’s disease is a autosomal recessive disorder of copper metabolism. Clinical phenotypes include hepatic, haemolytic, neurologic and psychiatric diseases. Wilson’s disease is caused by mutations in the ATP7B gene. ATP7B encodes a hepatic copper-transporting protein, which is important for copper excretion into bile. Neurological symptoms in Wilson’s disease include variable combinations of dysathria, ataxia, parkinsonism, dystonia and tremor. Wilson’s disease is lethal if untreated. This review discusses the epidemiology, genetics, clinical features, etiopathophysiology, diagnostic tests, and treatment of Wilson’s disease

Influence of cerebrovascular disease on brain networks in prodromal and clinical Alzheimer's disease.

Science.gov (United States)

Chong, Joanna Su Xian; Liu, Siwei; Loke, Yng Miin; Hilal, Saima; Ikram, Mohammad Kamran; Xu, Xin; Tan, Boon Yeow; Venketasubramanian, Narayanaswamy; Chen, Christopher Li-Hsian; Zhou, Juan

2017-11-01

Network-sensitive neuroimaging methods have been used to characterize large-scale brain network degeneration in Alzheimer's disease and its prodrome. However, few studies have investigated the combined effect of Alzheimer's disease and cerebrovascular disease on brain network degeneration. Our study sought to examine the intrinsic functional connectivity and structural covariance network changes in 235 prodromal and clinical Alzheimer's disease patients with and without cerebrovascular disease. We focused particularly on two higher-order cognitive networks-the default mode network and the executive control network. We found divergent functional connectivity and structural covariance patterns in Alzheimer's disease patients with and without cerebrovascular disease. Alzheimer's disease patients without cerebrovascular disease, but not Alzheimer's disease patients with cerebrovascular disease, showed reductions in posterior default mode network functional connectivity. By comparison, while both groups exhibited parietal reductions in executive control network functional connectivity, only Alzheimer's disease patients with cerebrovascular disease showed increases in frontal executive control network connectivity. Importantly, these distinct executive control network changes were recapitulated in prodromal Alzheimer's disease patients with and without cerebrovascular disease. Across Alzheimer's disease patients with and without cerebrovascular disease, higher default mode network functional connectivity z-scores correlated with greater hippocampal volumes while higher executive control network functional connectivity z-scores correlated with greater white matter changes. In parallel, only Alzheimer's disease patients without cerebrovascular disease showed increased default mode network structural covariance, while only Alzheimer's disease patients with cerebrovascular disease showed increased executive control network structural covariance compared to controls. Our

Genotator: A disease-agnostic tool for genetic annotation of disease

Directory of Open Access Journals (Sweden)

Jung Jae-Yoon

2010-10-01

Full Text Available Abstract Background Disease-specific genetic information has been increasing at rapid rates as a consequence of recent improvements and massive cost reductions in sequencing technologies. Numerous systems designed to capture and organize this mounting sea of genetic data have emerged, but these resources differ dramatically in their disease coverage and genetic depth. With few exceptions, researchers must manually search a variety of sites to assemble a complete set of genetic evidence for a particular disease of interest, a process that is both time-consuming and error-prone. Methods We designed a real-time aggregation tool that provides both comprehensive coverage and reliable gene-to-disease rankings for any disease. Our tool, called Genotator, automatically integrates data from 11 externally accessible clinical genetics resources and uses these data in a straightforward formula to rank genes in order of disease relevance. We tested the accuracy of coverage of Genotator in three separate diseases for which there exist specialty curated databases, Autism Spectrum Disorder, Parkinson's Disease, and Alzheimer Disease. Genotator is freely available at http://genotator.hms.harvard.edu. Results Genotator demonstrated that most of the 11 selected databases contain unique information about the genetic composition of disease, with 2514 genes found in only one of the 11 databases. These findings confirm that the integration of these databases provides a more complete picture than would be possible from any one database alone. Genotator successfully identified at least 75% of the top ranked genes for all three of our use cases, including a 90% concordance with the top 40 ranked candidates for Alzheimer Disease. Conclusions As a meta-query engine, Genotator provides high coverage of both historical genetic research as well as recent advances in the genetic understanding of specific diseases. As such, Genotator provides a real-time aggregation of ranked

Genotator: a disease-agnostic tool for genetic annotation of disease.

Science.gov (United States)

Wall, Dennis P; Pivovarov, Rimma; Tong, Mark; Jung, Jae-Yoon; Fusaro, Vincent A; DeLuca, Todd F; Tonellato, Peter J

2010-10-29

Disease-specific genetic information has been increasing at rapid rates as a consequence of recent improvements and massive cost reductions in sequencing technologies. Numerous systems designed to capture and organize this mounting sea of genetic data have emerged, but these resources differ dramatically in their disease coverage and genetic depth. With few exceptions, researchers must manually search a variety of sites to assemble a complete set of genetic evidence for a particular disease of interest, a process that is both time-consuming and error-prone. We designed a real-time aggregation tool that provides both comprehensive coverage and reliable gene-to-disease rankings for any disease. Our tool, called Genotator, automatically integrates data from 11 externally accessible clinical genetics resources and uses these data in a straightforward formula to rank genes in order of disease relevance. We tested the accuracy of coverage of Genotator in three separate diseases for which there exist specialty curated databases, Autism Spectrum Disorder, Parkinson's Disease, and Alzheimer Disease. Genotator is freely available at http://genotator.hms.harvard.edu. Genotator demonstrated that most of the 11 selected databases contain unique information about the genetic composition of disease, with 2514 genes found in only one of the 11 databases. These findings confirm that the integration of these databases provides a more complete picture than would be possible from any one database alone. Genotator successfully identified at least 75% of the top ranked genes for all three of our use cases, including a 90% concordance with the top 40 ranked candidates for Alzheimer Disease. As a meta-query engine, Genotator provides high coverage of both historical genetic research as well as recent advances in the genetic understanding of specific diseases. As such, Genotator provides a real-time aggregation of ranked data that remains current with the pace of research in the disease

Neuroinflammation in Alzheimer's disease and prion disease

NARCIS (Netherlands)

Eikelenboom, P.; Bate, C.; van Gool, W. A.; Hoozemans, J. J. M.; Rozemuller, J. M.; Veerhuis, R.; Williams, A.

2002-01-01

Alzheimer's disease (AD) and prion disease are characterized neuropathologically by extracellular deposits of Abeta and PrP amyloid fibrils, respectively. In both disorders, these cerebral amyloid deposits are co-localized with a broad variety of inflammation-related proteins (complement factors,

Chronic obstructive pulmonary disease and chronic heart failure: two muscle diseases?

Science.gov (United States)

Troosters, Thierry; Gosselink, Rik; Decramer, Marc

2004-01-01

Chronic obstructive pulmonary disease and congestive heart failure are two increasingly prevalent chronic diseases. Although care for these patients often is provided by different clinical teams, both disease conditions have much in common. In recent decades, more knowledge about the systemic impact of both diseases has become available, highlighting remarkable similarities in terms of prognostic factors and disease management. Rehabilitation programs deal with the systemic consequences of both diseases. Although clinical research also is conducted by various researchers investigating chronic obstructive pulmonary disease and chronic heart failure, it is worthwhile to compare the progress in relation to these two diseases over recent decades. Such comparison, the purpose of the current review, may help clinicians and scientists to learn about progress made in different, yet related, fields. The current review focuses on the similarities observed in the clinical impact of muscle weakness, the mechanisms of muscle dysfunction, the strategies to improve muscle function, and the effects of exercise training on chronic obstructive pulmonary disease and chronic heart failure.

Two adolescent patients with coexistent Graves' disease and Moyamoya disease in Korea.

Science.gov (United States)

Cheon, Chong Kun; Kim, Su Yung; Yoo, Jae-Ho

2014-06-01

Moyamoya disease is a cerebrovascular condition that results in the narrowing of the vessels of the circle of Willis and collateral vessel formation at the base of the brain. Although relationships between Graves' disease and cerebrovascular accidents in Moyamoya disease are obscure, the coexistence of the two diseases is noteworthy. Moyamoya disease has been rarely reported in adolescent patients with thyrotoxicosis. Recently, we encountered two adolescent Korean patients with Moyamoya disease associated with Graves' disease who presented with episodic right-sided hemiparesis and syncope. These two girls who had Graves' disease had no history of other diseases or head trauma. A thyroid function test revealed a euthyroid state and a high thyroid-stimulating hormone (TSH) receptor antibody titer at that time. The patients were diagnosed with Moyamoya disease based on brain magnetic resonance angiography and cerebral four-vessel angiography. The patients underwent cranial revascularization by encephalo-duroarterio-synangiosis as soon as a diagnosis was made, which resulted in successful symptom resolution. They fared well and had no additional neurological symptoms as of their last follow-up visits. Here, we report these two cases of confirmed Moyamoya disease complicated by Graves' disease with a review of the literature, and discuss the possible association between the two diseases. To our knowledge, this is the first report in South Korea on Moyamoya disease associated with Graves' disease in adolescents with a euthyroid.

Phenotype and Clinical Course of Inflammatory Bowel Disease with Co-Existent Celiac Disease.

Science.gov (United States)

Tse, Chung Sang; Deepak, Parakkal; De La Fuente, Jaime; Bledsoe, Adam C; Larson, Joseph J; Murray, Joseph A; Papadakis, Konstantinos A

2018-05-07

Inflammatory bowel diseases, principally Crohn's disease and ulcerative colitis, and celiac disease are among the most common immune-mediated gastrointestinal diseases. We aim to elucidate the clinical course and outcomes of patients with concomitant inflammatory bowel disease and celiac disease, a unique population that remains scarcely studied to date. A retrospective matched case-control study of adults with coexistent inflammatory bowel disease and celiac disease was performed at a tertiary referral institution in North America. Logistic regression and Kaplan-Meier curves compared disease characteristics and clinical outcomes of the two groups. A total of 342 inflammatory bowel disease patients were included in this study, of which 114 had coexistent celiac disease and 228 did not. Patients with coexistent inflammatory bowel disease and celiac disease had higher rates of primary sclerosing cholangitis (19.3% vs 5.7%; odds ratio, 4.4; 95% confidence interval, 2.1-9.4; pceliac disease (10.5% vs 3.5%; odds ratio 3.2; 95% confidence interval 1.3-8.2; p=0.01), compared to patients without concomitant celiac disease. Patients with inflammatory bowel disease with concomitant celiac disease have unique phenotypic features compared to non-celiac inflammatory bowel disease, with higher risks for colitis-related hospitalizations, extensive colitis, and primary sclerosing cholangitis. Increased recognition of coexistent IBD and celiac disease can prompt clinicians to investigate for concomitant disease sooner, particularly in patients with seemingly refractory disease.

Periodontal disease and anemias associated with Crohn's disease. A case report.

Science.gov (United States)

Nagpal, Swati; Acharya, Anirudh B; Thakur, Srinath L

2012-03-01

Crohn's disease (CD) is an inflammatory bowel disease with oral findings, including periodontal manifestations. Anemias, such as iron deficiency and anemia of chronic disease (ACD), are the most common hematologic complications of CD. Periodontitis has systemic effects, and may tend toward anemia, which can be explained by depressed erythropoiesis. In the report presented here, the authors review a case of Crohn's disease diagnosed 10 years previous to the patient presenting with a changing anemic profile and periodontal disease. A discussion of patient and disease management is included.

Is the disease course predictable in inflammatory bowel diseases?

Science.gov (United States)

Lakatos, Peter Laszlo; Kiss, Lajos S

2010-01-01

During the course of the disease, most patients with Crohn’s disease (CD) may eventually develop a stricturing or a perforating complication, and a significant number of patients with both CD and ulcerative colitis will undergo surgery. In recent years, research has focused on the determination of factors important in the prediction of disease course in inflammatory bowel diseases to improve stratification of patients, identify individual patient profiles, including clinical, laboratory and molecular markers, which hopefully will allow physicians to choose the most appropriate management in terms of therapy and intensity of follow-up. This review summarizes the available evidence on clinical, endoscopic variables and biomarkers in the prediction of short and long-term outcome in patients with inflammatory bowel diseases. PMID:20518079

Celiac Disease in Patients with Cystic Fibrosis-Related Bone Disease

Directory of Open Access Journals (Sweden)

Melissa S. Putman

2017-01-01

Full Text Available Both cystic fibrosis (CF and celiac disease can cause low bone mineral density (BMD and fractures. Celiac disease may occur at a higher frequency in patients with CF than the general population, and symptoms of these conditions may overlap. We report on two patients presenting with CF-related bone disease in the past year who were subsequently found to have concurrent celiac disease. Because adherence to a gluten-free diet may improve BMD in patients with celiac disease, this could have important implications for treatment. Clinicians should consider screening for celiac disease in patients with CF who have low BMD, worsening BMD in the absence of other risk factors, and/or difficult to treat vitamin D deficiency.

Glycogen storage disease type II (Pompe disease in children

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A. N. Semyachkina

2014-01-01

Full Text Available The paper gives the data available in the literature, which reflect the manifestations, diagnosis, and current treatments of the rare (orphan inherited disease glycogen storage disease type II or Pomp disease in children, as well as its classification. The infant form is shown to be most severe, resulting in death from cardiovascular or pulmonary failure generally within the first year of a child’s life. Emphasis is laid on major difficulties in the differential and true diagnosis of this severe disease. Much attention is given to the new pathogenetic treatment — genetically engineered enzyme replacement drug Myozyme®. The authors describe their clinical case of a child with the juvenile form of glycogen storage disease type II (late-onset Pompe disease. Particular emphasis is laid on the clinical symptoms of the disease and its diagnostic methods, among which the morphological analysis of a muscle biopsy specimen by light and electron microscopies, and enzyme and DNA diagnoses are of most importance. The proband was found to have significant lysosomal glycogen accumulation in the muscle biopsy specimen, reduced lymphocyte acid α-1,4-glucosidase activity to 4,2 nM/mg/h (normal value, 13,0—53,6 nM/mg/h, described in the HGMD missense mutation database from 1000 G>A p.Gly334er of the GAA in homozygous state, which verified the diagnosis of Pompe disease. 

[Celiac disease - disease of children and adults: symptoms, disease complications, risk groups and comorbidities].

Science.gov (United States)

Majsiak, Emilia; Cichoż-Lach, Halina; Gubska, Olena; Cukrowska, Bożena

2018-01-23

About 1% of human population suffers from celiac disease (CD) and it is one of the most commonly diagnosed autoimmune disorders. Until recently it was believed that CD affects mainly children, but as the newest studies show, up to 60% recently diagnosed patients are adults, often over the age of 60. CD's medical signs are nonspecific. Atypical course of the disease with extraintestinal symptoms is being increasingly observed. The disease may also be asymptomatic over many years. The studies show that the average diagnosis of CD takes more than 10 years since the first symptoms appear. Nonspecific medical signs cause undiagnosed patients suffering from CD to visit gastroenterologists, endocrinologists, allergists, gynaecologists and other medical specialists. However, most frequently general practitioners have the first encounter with patients suffering from CD, therefore they are able to recognize symptoms of the disease at the earliest and refer the patient to a gastroenterologist. Early diagnosis and beginning of the treatment reduce complications of untreated CD. The aim of this paper is to show general practitioners symptoms, disease complications, risk groups and comorbidities of CD.

Pregnancy and Rheumatic Disease

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... with Rheumatic Disease Pregnancy & Rheumatic Disease Pregnancy and Rheumatic Disease Fast Facts Diseases with the potential to affect ... control. What are the effects of pregnancy on rheumatic disease? The effects of pregnancy on rheumatic diseases vary ...

Accurate heterogeneous dose calculation for lung cancer patients without high‐resolution CT densities

Science.gov (United States)

Li, Jonathan G.; Liu, Chihray; Olivier, Kenneth R.; Dempsey, James F.

2009-01-01

The aim of this study was to investigate the relative accuracy of megavoltage photon‐beam dose calculations employing either five bulk densities or independent voxel densities determined by calibration of the CT Houndsfield number. Full‐resolution CT and bulk density treatment plans were generated for 70 lung or esophageal cancer tumors (66 cases) using a commercial treatment planning system with an adaptive convolution dose calculation algorithm (Pinnacle3, Philips Medicals Systems). Bulk densities were applied to segmented regions. Individual and population average densities were compared to the full‐resolution plan for each case. Monitor units were kept constant and no normalizations were employed. Dose volume histograms (DVH) and dose difference distributions were examined for all cases. The average densities of the segmented air, lung, fat, soft tissue, and bone for the entire set were found to be 0.14, 0.26, 0.89, 1.02, and 1.12 g/cm3, respectively. In all cases, the normal tissue DVH agreed to better than 2% in dose. In 62 of 70 DVHs of the planning target volume (PTV), agreement to better than 3% in dose was observed. Six cases demonstrated emphysema, one with bullous formations and one with a hiatus hernia having a large volume of gas. These required the additional assignment of density to the emphysemic lung and inflammatory changes to the lung, the regions of collapsed lung, the bullous formations, and the hernia gas. Bulk tissue density dose calculation provides an accurate method of heterogeneous dose calculation. However, patients with advanced emphysema may require high‐resolution CT studies for accurate treatment planning. PACS number: 87.53.Tf

Impetigo: diagnosis and treatment.

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Hartman-Adams, Holly; Banvard, Christine; Juckett, Gregory

2014-08-15

Impetigo is the most common bacterial skin infection in children two to five years of age. There are two principal types: nonbullous (70% of cases) and bullous (30% of cases). Nonbullous impetigo, or impetigo contagiosa, is caused by Staphylococcus aureus or Streptococcus pyogenes, and is characterized by honey-colored crusts on the face and extremities. Impetigo primarily affects the skin or secondarily infects insect bites, eczema, or herpetic lesions. Bullous impetigo, which is caused exclusively by S. aureus, results in large, flaccid bullae and is more likely to affect intertriginous areas. Both types usually resolve within two to three weeks without scarring, and complications are rare, with the most serious being poststreptococcal glomerulonephritis. Treatment includes topical antibiotics such as mupirocin, retapamulin, and fusidic acid. Oral antibiotic therapy can be used for impetigo with large bullae or when topical therapy is impractical. Amoxicillin/clavulanate, dicloxacillin, cephalexin, clindamycin, doxycycline, minocycline, trimethoprim/sulfamethoxazole, and macrolides are options, but penicillin is not. Natural therapies such as tea tree oil; olive, garlic, and coconut oils; and Manuka honey have been anecdotally successful, but lack sufficient evidence to recommend or dismiss them as treatment options. Treatments under development include minocycline foam and Ozenoxacin, a topical quinolone. Topical disinfectants are inferior to antibiotics and should not be used. Empiric treatment considerations have changed with the increasing prevalence of antibiotic-resistant bacteria, with methicillin-resistant S. aureus, macrolide-resistant streptococcus, and mupirocin-resistant streptococcus all documented. Fusidic acid, mupirocin, and retapamulin cover methicillin-susceptible S. aureus and streptococcal infections. Clindamycin proves helpful in suspected methicillin-resistant S. aureus infections. Trimethoprim/sulfamethoxazole covers methicillin-resistant S

Topographic hot spot before descemet stripping automated endothelial keratoplasty is associated with postoperative hyperopic shift.

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Shimizu, Tsutomu; Yamaguchi, Takefumi; Satake, Yoshiyuki; Shimazaki, Jun

2015-03-01

The aim of this study was to investigate topographic "hot spots" on the anterior corneal surface before Descemet stripping automated endothelial keratoplasty (DSAEK) and their effects on postoperative visual acuity and hyperopic shift. Twenty-seven eyes of 27 patients with bullous keratopathy, who underwent DSAEK were studied. We defined a hot spot as a focal area with relatively high refractive power on the anterior corneal surface in eyes with bullous keratopathy. Refractive spherical equivalent, keratometric value, and corneal topography were retrospectively evaluated using anterior segment optical coherence tomography (AS-OCT). Hot spots were identified in 11 eyes (42.3%) before DSAEK and disappeared in 9 eyes of these eyes (81.8%) at 6 months after DSAEK. AS-OCT revealed focal epithelial thickening in the same areas as the hot spots. There was no significant difference in the postoperative visual acuity between eyes with and without hot spots (P > 0.05). The keratometric value of the anterior corneal surface significantly flattened from 45.7 ± 2.7 diopters (D) before DSAEK to 44.2 ± 2.7 D 1 month after DSAEK in eyes with hot spots (P = 0.01), whereas in eyes without hot spots, there were no significant differences in the keratometric values before and after DSAEK. At 6 months, the refractive change was +1.1 ± 1.3 D in eyes with hot spots and -0.2 ± 0.6 D in eyes without hot spots (P = 0.034). In eyes with focal epithelial thickening, topographic hot spots on the anterior corneal surface were observed using AS-OCT. The hot spots disappeared after DSAEK and had no influence on the postoperative visual acuity.

Consensus Conference: A reappraisal of Gaucher disease - diagnosis and disease management algorithms

Science.gov (United States)

Mistry, Pramod K.; Cappellini, Maria Domenica; Lukina, Elena; Özsan, Hayri; Pascual, Sara Mach; Rosenbaum, Hanna; Solano, Maria Helena; Spigelman, Zachary; Villarrubia, Jesús; Watman, Nora Patricia; Massenkeil, Gero

2010-01-01

Type 1 (non neuronopathic) Gaucher disease was the first lysosomal storage disorder for which an effective enzyme replacement therapy was developed and it has become a prototype for treatments for related orphan diseases. There are currently four treatment options available to patients with Gaucher disease, nevertheless, almost 25% of type 1 Gaucher patients do not gain timely access to therapy because of delays in diagnosis after the onset of symptoms. Diagnosis of Gaucher disease by enzyme testing is unequivocal, but the rarity of the disease and non-specific and heterogeneous nature of Gaucher disease symptoms may impede consideration of this disease in the differential diagnosis. To help promote timely diagnosis and optimal management of the protean presentations of Gaucher disease, a consensus meeting was convened to develop algorithms for diagnosis and disease management for Gaucher disease. PMID:21080341

Celiac disease

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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.

Graves disease with ophthalmopathy following radiotherapy for Hodgkin's disease

International Nuclear Information System (INIS)

Jacobson, D.R.; Fleming, B.J.

1984-01-01

The number of patients achieving long-term survival following neck irradiation for Hodgkin's disease and other malignancies is increasing. Paralleling this increase in survivors is the development of late complications of the therapy itself. Eleven patients have previously been reported who developed Graves ophthalmopathy 18 months to seven years after receiving neck radiotherapy for nonthyroidal malignancies. The seven patients who had HLA typing were all HLA-B8 negative, despite the reported association of the HLA-B8 antigen with Graves disease. A patient who is HLA-B8 positive who developed Graves ophthalmopathy and hyperthyroidism nine years after receiving mantle radiotherapy for Hodgkin's disease is reported. It is recommended that Graves disease be included among the thyroid diseases that receive consideration during follow-up of patients who have received mantle radiotherapy

Prevalence of periodontal disease, its association with systemic diseases and prevention

OpenAIRE

Nazir, Muhammad Ashraf

2017-01-01

Periodontal diseases are prevalent both in developed and developing countries and affect about 20-50% of global population. High prevalence of periodontal disease in adolescents, adults, and older individuals makes it a public health concern. Several risk factors such as smoking, poor oral hygiene, diabetes, medication, age, hereditary, and stress are related to periodontal diseases. Robust evidence shows the association of periodontal diseases with systemic diseases such as cardiovascular di...

Chronic Disease and Childhood Development: Kidney Disease and Transplantation.

Science.gov (United States)

Klein, Susan D.; Simmons, Roberta G.

As part of a larger study of transplantation and chronic disease and the family, 124 children (10-18 years old) who were chronically ill with kidney disease (n=72) or were a year or more post-transplant (n=52) were included in a study focusing on the effects of chronic kidney disease and transplantation on children's psychosocial development. Ss…

Case Report of a 21-Year-Old Man With Epidermolysis Bullosa Acquisita.

Science.gov (United States)

Cyr, Janelle; Liu, Annie; Ghazarian, Danny; Siddha, Sanjay

Epidermolysis bullosa acquisita (EBA) is a rare acquired type of mechanobullous disease affecting the dermal-epidermal junction (DEJ) of trauma prone acral surfaces. It manifests as tense vesicles, bullae, and milia and typically heals as atrophic hypo- or hyperpigmented scars. Classic noninflammatory mechanobullous EBA typically presents at a mean age of 48 years. A 21-year-old man presented with a 2-year history of nonpainful papular-vesicular lesions on his hands, knees, and toes after minor trauma to these areas. Physical exam revealed postinflammatory hypopigmented scarring and milia to the bilateral dorsal hands and bilateral extensor elbows and knees, with tense blisters on the dorsal hand and patella regions. Direct immunofluorescence revealed strong linear IgG and IgM with weak focal positivity for IgA and C3 at the DEJ. Blood work revealed an increased diffuse gamma region of 71 g/L (6-13 g/L) on serum protein electrophoresis. Pathology showed a fibrotic underlying dermis, with subepidermal bullae and separation and no significant inflammation. The patient was started on colchicine. This case showcases an unusual early age of presentation for mechanobullous EBA and illustrates the importance of interpreting pathology in the context of clinical findings and maintaining a high index of suspicion for EBA in younger patients who present with classic findings. This case is unique as it is the first report of an association between EBA and polyclonal gammopathy and could be suggestive of chronic inflammation, which would fit with our patient's chronic history of EBA.

Involvement of Gaucher Disease Mutations in Parkinson Disease.

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Vilageliu, Lluisa; Grinberg, Daniel

2017-01-01

Gaucher disease is an autosomal recessive lysosomal storage disorder, caused by mutations in the GBA gene. The frequency of Gaucher disease patients and heterozygote carriers that developed Parkinson disease has been found to be above that of the control population. This fact suggests that mutations in the GBA gene can be involved in Parkison's etiology. Analysis of large cohorts of patients with Parkinson disease has shown that there are significantly more cases bearing GBA mutations than those found among healthy individuals. Functional studies have proven an interaction between α-synuclein and GBA, the levels of which presented an inverse correlation. Mutant GBA proteins cause increases in α-synuclein levels, while an inhibition of GBA by α-synuclein has been also demonstrated. Saposin C, a coactivator of GBA, has been shown to protect GBA from this inhibition. Among the GBA variants associated with Parkinson disease, E326K seems to be one of the most prevalent. Interestingly, it is involved in Gaucher disease only when it forms part of a double-mutant allele, usually with the L444P mutation. Structural analyses have revealed that both residues (E326 and L444) interact with Saposin C and, probably, also with α-synuclein. This could explain the antagonistic role of these two proteins in relation to GBA. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

Development of Graves' disease following radiation therapy in Hodgkin's disease

International Nuclear Information System (INIS)

Loeffler, J.S.; Tarbell, N.J.; Garber, J.R.; Mauch, P.

1988-01-01

Radiation-related thyroid dysfunction is a common occurrence in patients with Hodgkin's disease treated with mantle field radiation. Although chemical and clinical hypothyroidism are most commonly seen, Graves' disease has also been described. We have examined the records of 437 surgically staged patients who received mantle field irradiation between April 1969 and December 1980 to ascertain the frequency of manifestations of Graves' disease. Within this group, seven patients developed hyperthyroidism accompanied by ophthalmic findings typical of those seen in Graves' disease. The actuarial risk of developing Graves' disease at 10 years following mantle irradiation for Hodgkin's disease was 3.3% in female patients and 1% in male patients in this study. The observed/expected ratios were 5.9 and 5.1 for female and male patients, respectively. This observed risk significantly exceeded that seen in the general population

Hematoma of the proximal nail fold due to oximeter in a child

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Patricia Chang

2014-01-01

Full Text Available Boy 4 years old, hospitalized due to hemorrhagic chickenpox and sepsis during his clinical examination besides hemorrhagic crust, vesicles and bullous he has also a cutaneous red lesion localized at the right proximal nail fold of the big toenail (Fig. 1, dermatoscopic view of the lesion (Fig. 2. The diagnosis of hematoma of the proximal nail fold due to oximeter was done. The proximal nail fold hematomas due to oximeter are uncommon dermatoses at this level that are caused for the pressure of the oximeter and it has been seeing in patient in Unit Intensive or Intermediate Unit Care since 2007.

The acknowledgement of the Schneeberg lung disease as occupational disease in the first decree of occupational diseases from 1925

International Nuclear Information System (INIS)

Schuettmann, W.

1987-01-01

The Schneeberg lung disease is the lung cancer, conditioned by radiation which is caused by the influence of radon and of its subsequent products. It has gained a great importance after World War II as a consequence of the intensified mining of uranium ore. From the history of the disease, lasting some centuries, the period of the twenties and thirties of this century is represented in which on one side the conception of the causal importance of radon has made its way little by little, and on the other side the disease was acknowledged as occupational disease within the first decree of occupational diseases in the former German Reich. Evaluating materials from Saxon archives it is described how the legislative preparations to the acknowledgement of the Schneeberg lung disease as occupational disease and the simultaneous research to the elucidation of nature and cause of the disease have penetrated and influenced each other. (author)

The elusive baseline of marine disease: are diseases in ocean ecosystems increasing?

Directory of Open Access Journals (Sweden)

Jessica R Ward

2004-04-01

Full Text Available Disease outbreaks alter the structure and function of marine ecosystems, directly affecting vertebrates (mammals, turtles, fish, invertebrates (corals, crustaceans, echinoderms, and plants (seagrasses. Previous studies suggest a recent increase in marine disease. However, lack of baseline data in most communities prevents a direct test of this hypothesis. We developed a proxy to evaluate a prediction of the increasing disease hypothesis: the proportion of scientific publications reporting disease increased in recent decades. This represents, to our knowledge, the first quantitative use of normalized trends in the literature to investigate an ecological hypothesis. We searched a literature database for reports of parasites and disease (hereafter "disease" in nine marine taxonomic groups from 1970 to 2001. Reports, normalized for research effort, increased in turtles, corals, mammals, urchins, and molluscs. No significant trends were detected for seagrasses, decapods, or sharks/rays (though disease occurred in these groups. Counter to the prediction, disease reports decreased in fishes. Formulating effective resource management policy requires understanding the basis and timing of marine disease events. Why disease outbreaks increased in some groups but not in others should be a priority for future investigation. The increase in several groups lends urgency to understanding disease dynamics, particularly since few viable options currently exist to mitigate disease in the oceans.

A cross-sectional survey to study the relationship of periodontal disease with cardiovascular disease, respiratory disease, and diabetes mellitus.

Science.gov (United States)

Oberoi, Sukhvinder Singh; Harish, Yashoda; Hiremath, Shivalingaswamy; Puranik, Manjunath

2016-01-01

Periodontal deterioration has been reported to be associated with systemic diseases such as cardiovascular disease (CVD), diabetes mellitus, respiratory disease, liver cirrhosis, bacterial pneumonia, nutritional deficiencies, and adverse pregnancy outcomes. The present study assessed the periodontal disease among patients with systemic conditions such as diabetes, CVD, and respiratory disease. The study population consisted of 220 patients each of CVD, respiratory disease, and diabetes mellitus, making a total of 660 patients in the systemic disease group. A control group of 340 subjects were also included in the study for comparison purpose. The periodontal status of the patients with these confirmed medical conditions was assessed using the community periodontal index of treatment needs (CPITNs) index. The prevalence of CPITN code 4 was found to be greater among the patients with respiratory disease whereas the mean number of sextants with score 4 was found to be greater among the patients with diabetes mellitus and CVD. The treatment need 0 was found to be more among the controls (1.18%) whereas the treatment need 1, 2, and 3 were more among the patients with respiratory disease (100%, 97.73%, and 54.8%), diabetes mellitus (100%, 100% and 46.4%), and CVD (100%, 97.73%, and 38.1%), in comparison to the controls (6.18%). From the findings of the present study, it can be concluded that diabetes mellitus, CVD, and respiratory disease are associated with a higher severity of periodontal disease.

A cross-sectional survey to study the relationship of periodontal disease with cardiovascular disease, respiratory disease, and diabetes mellitus

Directory of Open Access Journals (Sweden)

Sukhvinder Singh Oberoi

2016-01-01

Full Text Available Background: Periodontal deterioration has been reported to be associated with systemic diseases such as cardiovascular disease (CVD, diabetes mellitus, respiratory disease, liver cirrhosis, bacterial pneumonia, nutritional deficiencies, and adverse pregnancy outcomes. Aim: The present study assessed the periodontal disease among patients with systemic conditions such as diabetes, CVD, and respiratory disease. Materials and Methods: The study population consisted of 220 patients each of CVD, respiratory disease, and diabetes mellitus, making a total of 660 patients in the systemic disease group. A control group of 340 subjects were also included in the study for comparison purpose. The periodontal status of the patients with these confirmed medical conditions was assessed using the community periodontal index of treatment needs (CPITNs index. Results: The prevalence of CPITN code 4 was found to be greater among the patients with respiratory disease whereas the mean number of sextants with score 4 was found to be greater among the patients with diabetes mellitus and CVD. The treatment need 0 was found to be more among the controls (1.18% whereas the treatment need 1, 2, and 3 were more among the patients with respiratory disease (100%, 97.73%, and 54.8%, diabetes mellitus (100%, 100% and 46.4%, and CVD (100%, 97.73%, and 38.1%, in comparison to the controls (6.18%. Conclusion: From the findings of the present study, it can be concluded that diabetes mellitus, CVD, and respiratory disease are associated with a higher severity of periodontal disease.

The integrated disease network.

Science.gov (United States)

Sun, Kai; Buchan, Natalie; Larminie, Chris; Pržulj, Nataša

2014-11-01

The growing body of transcriptomic, proteomic, metabolomic and genomic data generated from disease states provides a great opportunity to improve our current understanding of the molecular mechanisms driving diseases and shared between diseases. The use of both clinical and molecular phenotypes will lead to better disease understanding and classification. In this study, we set out to gain novel insights into diseases and their relationships by utilising knowledge gained from system-level molecular data. We integrated different types of biological data including genome-wide association studies data, disease-chemical associations, biological pathways and Gene Ontology annotations into an Integrated Disease Network (IDN), a heterogeneous network where nodes are bio-entities and edges between nodes represent their associations. We also introduced a novel disease similarity measure to infer disease-disease associations from the IDN. Our predicted associations were systemically evaluated against the Medical Subject Heading classification and a statistical measure of disease co-occurrence in PubMed. The strong correlation between our predictions and co-occurrence associations indicated the ability of our approach to recover known disease associations. Furthermore, we presented a case study of Crohn's disease. We demonstrated that our approach not only identified well-established connections between Crohn's disease and other diseases, but also revealed new, interesting connections consistent with emerging literature. Our approach also enabled ready access to the knowledge supporting these new connections, making this a powerful approach for exploring connections between diseases.

Concomitant diseases and their effect on disease prognosis in Meniere's disease: diabetes mellitus identified as a negative prognostic factor.

Science.gov (United States)

Pieskä, Teemu; Kotimäki, Jouko; Männikkö, Minna; Sorri, Martti; Hietikko, Elina

2018-01-01

To study comorbidities and their effect on the disease progression in Meniere's disease (MD). Retrospective study on 350 definite MD patients diagnosed according to AAO-HNS 1995 criteria using hospital records and postal questionnaire. The prevalence of migraine, hypothyroidism, allergies, coronary heart disease and autoimmune diseases was more common in MD patients than reported in the general population of Finland. Diabetes mellitus was associated with both more severe hearing impairment (p = .033) and more frequent vertigo (p = .028) in MD patients. The number of concomitant diseases was associated with more frequent vertigo (p = .021). A patient's concomitant diseases, especially diabetes, should be treated effectively because they might affect the progression of MD. Further studies on the effects of concomitant diseases on MD prognosis are needed.

Esophageal involvement and interstitial lung disease in mixed connective tissue disease.

Science.gov (United States)

Fagundes, M N; Caleiro, M T C; Navarro-Rodriguez, T; Baldi, B G; Kavakama, J; Salge, J M; Kairalla, R; Carvalho, C R R

2009-06-01

Mixed connective tissue disease is a systemic inflammatory disorder that results in both pulmonary and esophageal manifestations. We sought to evaluate the relationship between esophageal dysfunction and interstitial lung disease in patients with mixed connective tissue disease. We correlated the pulmonary function data and the high-resolution computed tomography findings of interstitial lung disease with the results of esophageal evaluation in manometry, 24-hour intraesophageal pH measurements, and the presence of esophageal dilatation on computed tomography scan. Fifty consecutive patients with mixed connective tissue disease, according to Kasukawa's classification criteria, were included in this prospective study. High-resolution computed tomography parenchymal abnormalities were present in 39 of 50 patients. Esophageal dilatation, gastroesophageal reflux, and esophageal motor impairment were also very prevalent (28 of 50, 18 of 36, and 30 of 36, respectively). The presence of interstitial lung disease on computed tomography was significantly higher among patients with esophageal dilatation (92% vs. 45%; pmotor dysfunction (90% vs. 35%; pesophageal and pulmonary involvement, our series revealed a strong association between esophageal motor dysfunction and interstitial lung disease in patients with mixed connective tissue disease.

[Infectious diseases research].

Science.gov (United States)

Carratalà, Jordi; Alcamí, José; Cordero, Elisa; Miró, José M; Ramos, José Manuel

2008-12-01

There has been a significant increase in research activity into infectious diseases in Spain in the last few years. The Spanish Society of Infectious Diseases and Clinical Microbiology (SEIMC) currently has ten study groups, with the cooperation of infectious diseases specialists and microbiologists from different centres, with significant research activity. The program of Redes Temáticas de Investigación Cooperativa en Salud (Special Topics Cooperative Health Research Networks) is an appropriate framework for the strategic coordination of research groups from the Spanish autonomous communities. The Spanish Network for Research in Infectious Diseases (REIPI) and the Network for Research in AIDS (RIS) integrate investigators in Infectious Diseases from multiple groups, which continuously perform important research projects. Research using different experimental models in infectious diseases, in numerous institutions, is an important activity in our country. The analysis of the recent scientific production in Infectious Diseases shows that Spain has a good position in the context of the European Union. The research activity in Infectious Diseases carried out in our country is a great opportunity for the training of specialists in this area of knowledge.

Human Environmental Disease Network

DEFF Research Database (Denmark)

Taboureau, Olivier; Audouze, Karine

2017-01-01

During the past decades, many epidemiological, toxicological and biological studies have been performed to assess the role of environmental chemicals as potential toxicants for diverse human disorders. However, the relationships between diseases based on chemical exposure have been rarely studied...... by computational biology. We developed a human environmental disease network (EDN) to explore and suggest novel disease-disease and chemical-disease relationships. The presented scored EDN model is built upon the integration on systems biology and chemical toxicology using chemical contaminants information...... and their disease relationships from the reported TDDB database. The resulting human EDN takes into consideration the level of evidence of the toxicant-disease relationships allowing including some degrees of significance in the disease-disease associations. Such network can be used to identify uncharacterized...

Skin diseases: prevalence and predictors of itch and disease severity.

NARCIS (Netherlands)

Verhoeven, E.W.M.

2009-01-01

Chronic skin diseases are known to be common among the general population. Nevertheless, little research attention has been paid to patients with skin diseases in the general population, and consequently, little is known about the impact of skin diseases on daily life within this population. General

Responsiveness of Endoscopic Indices of Disease Activity for Crohn's Disease

NARCIS (Netherlands)

Khanna, Reena; Zou, Guangyong; Stitt, Larry; Feagan, Brian G.; Sandborn, William J.; Rutgeerts, Paul; McDonald, John W. D.; Dubcenco, Elena; Fogel, Ronald; Panaccione, Remo; Jairath, Vipul; Nelson, Sigrid; Shackelton, Lisa M.; Huang, Bidan; Zhou, Qian; Robinson, Anne M.; Levesque, Barrett G.; D'Haens, Geert

2017-01-01

The Crohn's Disease Endoscopic Index of Severity (CDEIS) and the Simple Endoscopic Score for Crohn's Disease (SES-CD) are commonly used to assess Crohn's disease (CD) activity; however neither instrument is fully validated. We evaluated the responsiveness to change of the SES-CD and CDEIS using data

AACE/ACE Disease State Clinical Review: Medical Management of Cushing Disease.

Science.gov (United States)

Hamrahian, Amir H; Yuen, Kevin C J; Hoffman, Andrew R

2014-07-01

To review available medical therapies for patients with Cushing disease and to provide a roadmap for their use in clinical practice. PubMed searches were performed to identify all of the available published data on medical management of Cushing disease. Medical therapy is usually not the first-line treatment for patients with Cushing disease but may be used to improve clinical manifestations of Cushing disease in patients who are not suitable candidates for surgery, following unsuccessful surgery or recurrence, or as a "bridge therapy" in those who have undergone radiotherapy. Medical therapy may also be used in preoperative preparation of patients with severe disease. Current available medical options for patients with Cushing disease include centrally acting agents, steroidogenesis inhibitors, and a glucocorticoid receptor antagonists. At present, there are no head-to-head studies comparing the efficacy, tolerability, and safety of different U.S. Food and Drug Administration (FDA)- and non-FDA-approved drugs in patients with Cushing disease. With the initiation of new studies and the completion of ongoing clinical trials, the number of FDA-approved drugs for medical treatment of Cushing disease is expected to increase. Medical therapy has an important adjunctive role in the management of patients with Cushing disease. The decision to initiate medical treatment depends on many factors, including patient characteristics and preference. Long-term studies are needed to better define the clinical efficacy, safety, and tolerability of medical treatment of Cushing disease, including the role of combination therapies.

DOSim: An R package for similarity between diseases based on Disease Ontology

Science.gov (United States)

2011-01-01

Background The construction of the Disease Ontology (DO) has helped promote the investigation of diseases and disease risk factors. DO enables researchers to analyse disease similarity by adopting semantic similarity measures, and has expanded our understanding of the relationships between different diseases and to classify them. Simultaneously, similarities between genes can also be analysed by their associations with similar diseases. As a result, disease heterogeneity is better understood and insights into the molecular pathogenesis of similar diseases have been gained. However, bioinformatics tools that provide easy and straight forward ways to use DO to study disease and gene similarity simultaneously are required. Results We have developed an R-based software package (DOSim) to compute the similarity between diseases and to measure the similarity between human genes in terms of diseases. DOSim incorporates a DO-based enrichment analysis function that can be used to explore the disease feature of an independent gene set. A multilayered enrichment analysis (GO and KEGG annotation) annotation function that helps users explore the biological meaning implied in a newly detected gene module is also part of the DOSim package. We used the disease similarity application to demonstrate the relationship between 128 different DO cancer terms. The hierarchical clustering of these 128 different cancers showed modular characteristics. In another case study, we used the gene similarity application on 361 obesity-related genes. The results revealed the complex pathogenesis of obesity. In addition, the gene module detection and gene module multilayered annotation functions in DOSim when applied on these 361 obesity-related genes helped extend our understanding of the complex pathogenesis of obesity risk phenotypes and the heterogeneity of obesity-related diseases. Conclusions DOSim can be used to detect disease-driven gene modules, and to annotate the modules for functions and

Lyme disease and post-treatment Lyme disease syndrome: the neglected disease in our own backyard.

Science.gov (United States)

Crowder, L A; Yedlin, V A; Weinstein, E R; Kortte, K B; Aucott, J N

2014-09-01

A survey was developed to assess experience and opinions about Lyme disease and post-treatment Lyme disease syndrome (PTLDS) among faculties in public health. No previous surveys of public health faculties have been found in the literature. This is a cross sectional study of public health school faculty members designed to measure knowledge and experience with Lyme disease and PTLDS using an internet survey instrument. Participants were recruited using all the publicly available e-mail addresses of faculty members in all the 50 accredited Schools of Public Health in the United States. A 15% response rate was seen for the survey. 50% of respondents were from Lyme endemic states. Less than 5% of faculty members consider themselves expert in Lyme or PTLDS. Many faculty members had known someone with Lyme disease or PTLDS, but few had been diagnosed themselves. Most believe that PTLDS can be severe and chronic, is not easy to treat, and does not resolve on its own, but were uncertain about its aetiology. Most respondents also felt that the incidence of Lyme disease will increase and that more education is needed. The need for further understanding and communication presents an opportunity for public health research and education in Lyme disease and the sequelae of PTLDS. Copyright © 2014 The Royal Society for Public Health. Published by Elsevier Ltd. All rights reserved.

Interaction of lifestyle, behaviour or systemic diseases with dental caries and periodontal diseases

DEFF Research Database (Denmark)

Chapple, Iain L C; Bouchard, Philippe; Cagetti, Maria Grazia

2017-01-01

Periodontal diseases and dental caries are the most common diseases of humans and the main cause of tooth loss. Both diseases can lead to nutritional compromise and negative impacts upon self-esteem and quality of life. As complex chronic diseases, they share common risk factors, such as a requir......Periodontal diseases and dental caries are the most common diseases of humans and the main cause of tooth loss. Both diseases can lead to nutritional compromise and negative impacts upon self-esteem and quality of life. As complex chronic diseases, they share common risk factors...... to periodontal diseases and caries susceptibility, with an attributable risk estimated to be up to 50%. The genetics literature for periodontal disease is more substantial than for caries and genes associated with chronic periodontitis are the vitamin D receptor (VDR), Fc gamma receptor IIA (Fc...... or composition, smoking, carbohydrate intake). Identification of these factors is crucial in the prevention of both diseases as well as in their management. AIM: To systematically appraise the scientific literature to identify potential risk factors for caries and periodontal diseases. METHODS: One systematic...

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 ...

Pediatric Celiac Disease

Science.gov (United States)

... a protein found in wheat, rye, and barley. Pediatric Celiac Disease If your child has celiac disease, ... physician. Established by the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) Celiac Disease Eosinophilic ...

Lyme Disease Data

Science.gov (United States)

... materials Why is CDC concerned about Lyme disease? 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 ...

Spreading disease: a controversy concerning the metaphysics of disease.

Science.gov (United States)

D'Amico, R

1998-01-01

This article concerns the metaphysics of disease. Is disease a fixed feature of the world or a social value or preference? I argue that disease is not a value-laden concept and thus debates concerning it differ fundamentally from debates concerning health, harm, or suffering where evaluative judgements are central. I show how the so-called social constructionist view of disease has been motivated both by ethical concerns with medical practices and general theoretical doubts about scientific naturalism. If I can show that ethical concerns about medical treatment can be answered without adopting social constructionism, that leaves only the broader theoretical question of naturalism. I cannot completely answer those theoretical doubts, but I show that the theoretical motivation is less convincing when it is separated from the moral challenge often accompanying it. I conclude that a convincing defense of the non-naturalistic conception of disease is rarely attempted and proves more difficult and counter-intuitive than its proponents assume.

CT findings of diffuse pleural diseases: differentiation of malignant disease from tuberculosis

International Nuclear Information System (INIS)

Roh, In Gye; Kook, Shin Ho; Lee, Young Rae; Chin, Seung Bum; Park, Yoon Ok; Park, Hae Won

1997-01-01

To evaluate whether or not previously known CT criteria for differentiating malignant and benign pleural diseases are useful in the differentiation of diffuse malignant pleural diseases and tuberculosis. We retrospectively analyzed CT scans of 42 patients comprising 20 cases of malignant pleural diseases and 22 cases of tuberculous pleural diseases, according to previously known CT criteria for differentiating malignant and benign pleural diseases. The most common shape of pleural effusion was crescentic in malignant pleural diseases and loculated in tuberculosis. The aggressive nature of pleural effusion, pleural rind, and pleura thickening was 1.5 times more frequently observed in malignant pleural diseases than in tuberculosis. Smooth thickening or smooth nodular pleural thickening and extrapleural deposition of fat were 1.5 times more frequently found in tuberculous than in malignant pleural diseases. Interruption of pleural thickening was found twice as frequently in malignant pleural diseases as in tuberculosis. Decreased lung volume was found twice as frequently in tuberculous as in malignant pleural diseases. Anatomical mediastinal pleural involvement was three times, and irregular nodular pleural thickening nine times more frequent in malignant pleural diseases than in tuberculosis. The sensitivity and specificity of CT findings above 70%, and thus suggesting malignant pleural diseases, were as follows : 1) aggressive nature of pleural fluid collection extending to the azygoesophageal recess or tongue of the lung (51.5%, 75%); 2) involvement of anatomical mediastinal pleura (69.2%, 73.7%); 3) irregular nodular pleural thickening (87.5%, 69%). Although there in overlap between previously known CT criteria for the differentiation of benign and malignant pleural diseases, the aggressive nature of pleural fluid collection extending to the azygoesophageal recess or tongue of the lung, the involvement of anatomical mediastinal pleura and irregular nodular

Hereditary neuromuscular diseases

Energy Technology Data Exchange (ETDEWEB)

Oezsarlak, O. E-mail: ozkan.ozsarlak@uza.be; Schepens, E.; Parizel, P.M.; Goethem, J.W. van; Vanhoenacker, F.; Schepper, A.M. de; Martin, J.J

2001-12-01

This article presents the actual classification of neuromuscular diseases based on present expansion of our knowledge and understanding due to genetic developments. It summarizes the genetic and clinical presentations of each disorder together with CT findings, which we studied in a large group of patients with neuromuscular diseases. The muscular dystrophies as the largest and most common group of hereditary muscle diseases will be highlighted by giving detailed information about the role of CT and MRI in the differential diagnosis. The radiological features of neuromuscular diseases are atrophy, hypertrophy, pseudohypertrophy and fatty infiltration of muscles on a selective basis. Although the patterns and distribution of involvement are characteristic in some of the diseases, the definition of the type of disease based on CT scan only is not always possible.

Disease burden of infectious diseases in Europe: a pilot study

NARCIS (Netherlands)

Lier EA van; Havelaar AH; LZO

2007-01-01

Consequences of different infectious diseases cannot be adequately compared with each other on the basis of the number of patients or mortality data only. It is better to combine all health effects and express the total impact as disease burden, which also takes duration and severity of diseases

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

Degenerative Nerve Diseases

Science.gov (United States)

Degenerative nerve diseases affect many of your body's activities, such as balance, movement, talking, breathing, and heart function. Many ... viruses. Sometimes the cause is not known. Degenerative nerve diseases include Alzheimer's disease Amyotrophic lateral sclerosis Friedreich's ...

Understanding cardiovascular disease

Science.gov (United States)

... page: //medlineplus.gov/ency/patientinstructions/000759.htm Understanding cardiovascular disease To use the sharing features on this page, ... lead to heart attack or stroke. Types of Cardiovascular Disease Coronary heart disease (CHD) is the most common ...

Lyme disease (image)

Science.gov (United States)

Lyme disease is an acute inflammatory disease characterized by skin changes, joint inflammation and symptoms similar to the ... that is caused by the bacterium Borrelia burgdorferi . Lyme disease is transmitted by the bite of a deer ...

Parkinson's Disease Videos

Medline Plus

Full Text Available ... Expert Briefings: Anxiety in Parkinson's Disease Expert Briefings: Nutrition and Parkinson's Disease NY Nightly News with Chuck ... Briefings: What's in the Parkinson's Pipeline? Expert Briefings: Nutrition and Parkinson's Disease 2010 Expert Briefings: Legal Issues: ...

Gene therapy for CNS diseases – Krabbe disease

Directory of Open Access Journals (Sweden)

Mohammad A. Rafi

2016-06-01

Full Text Available This is a brief report of the 19th Annual Meeting of the American Society of Gene and Cell Therapy that took place from May 4th through May 7th, 2016 in Washington, DC, USA. While the meeting provided many symposiums, lectures, and scientific sessions this report mainly focuses on one of the sessions on the "Gene Therapy for central nervous system (CNS Diseases" and specifically on the "Gene Therapy for the globoid cell leukodystrophy or Krabbe disease. Two presentations focused on this subject utilizing two animal models of this disease: mice and dog models. Different serotypes of adeno-associate viral vectors (AAV alone or in combination with bone marrow transplantations were used in these research projects. The Meeting of the ASGCT reflected continuous growth in the fields of gene and cell therapy and brighter forecast for efficient treatment options for variety of human diseases.

Parkinson's Disease Videos

Medline Plus

Full Text Available ... Progression of the Disease? OHSU - Parkinson's Disease: Pharmacological Management of Depression, Anxiety & Psychosis OHSU - Overview of Parkinson's ... Disease? What Are Some Strategies to Improve the Quality of Community Care for PD Patients? CareMAP: Dealing ...

Parkinson's Disease Videos

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Full Text Available ... Does Caregiving Change from Day to Day? Unconditional Love How Does Parkinson's Disease Affect the Urinary System? ... Mind Guide to Parkinson's Disease Guide to Deep Brain Stimulation Sleep: A Mind Guide to Parkinson’s Disease ...

Parkinson's Disease Videos

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Full Text Available ... Nonmotor Symptoms of Parkinson's Disease Expert Briefings: Gait, Balance and Falls in Parkinson's Disease Expert Briefings: Coping ... Conference: Lessons Learned How Does the DBS Device Work? OHSU - Parkinson's Disease: Managing Depression, Anxiety & Psychosis CareMAP: ...

Parkinson's Disease Videos

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Full Text Available ... Tips for Care Partners Nurse Webinars: Nursing Solutions: Innovations in PD Nurse Education CareMAP: Managing Advanced Parkinson's ... Progression of the Disease? OHSU - Parkinson's Disease: Pharmacological Management of Depression, Anxiety & Psychosis How Is Parkinson's Disease ...

Tay-Sachs Disease

Science.gov (United States)

Tay-Sachs disease is a rare, inherited disease. It is a type of lipid metabolism disorder. It causes too ... cells, causing mental and physical problems. . Infants with Tay-Sachs disease appear to develop normally for the first few ...

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 ...

Tick-borne disease.

Science.gov (United States)

Bratton, Robert L; Corey, Ralph

2005-06-15

Tick-borne diseases in the United States include Rocky Mountain spotted fever, Lyme disease, ehrlichiosis, tularemia, babesiosis, Colorado tick fever, and relapsing fever. It is important for family physicians to consider these illnesses when patients present with influenza-like symptoms. A petechial rash initially affecting the palms and soles of the feet is associated with Rocky Mountain spotted fever, whereas erythema migrans (annular macule with central clearing) is associated with Lyme disease. Various other rashes or skin lesions accompanied by fever and influenza-like illness also may signal the presence of a tick-borne disease. Early, accurate diagnosis allows treatment that may help prevent significant morbidity and possible mortality. Because 24 to 48 hours of attachment to the host are required for infection to occur, early removal can help prevent disease. Treatment with doxycycline or tetracycline is indicated for Rocky Mountain spotted fever, Lyme disease, ehrlichiosis, and relapsing fever. In patients with clinical findings suggestive of tick-borne disease, treatment should not be delayed for laboratory confirmation. If no symptoms follow exposure to tick bites, empiric treatment is not indicated. The same tick may harbor different infectious pathogens and transmit several with one bite. Advising patients about prevention of tick bites, especially in the summer months, may help prevent exposure to dangerous vector-borne diseases.

INTRACRANIAL HYDATID DISEASE: IMAGING FINDINGS OF A RARE DISEASE

Directory of Open Access Journals (Sweden)

idil Gunes Tatar

2014-06-01

Full Text Available Hydatid disease is caused by the larval stage of the parasite Echinococcus granulosus. It is mainly endemic in North African and Mediterranean countries. The disease usually manifests in liver and lungs although involvement of other organs are also seen. In this rare case intracranial hydatid disease in a 9-year-old female patient is presented with Magnetic Resonance Imaging findings. [J Contemp Med 2014; 4(2.000: 103-105

Stargardt Disease

Science.gov (United States)

... Stargardt disease, lipofuscin accumulates abnormally. The Foundation Fighting Blindness supports research studying lipofuscin build up and ways to prevent it. A decrease in color perception also occurs in Stargardt disease. This is ...

Prevalence and overlap of Disease Management Program diseases in older hospitalized patients

DEFF Research Database (Denmark)

Juul-Larsen, Helle Gybel; Petersen, Janne; Sivertsen, Ditte Maria

2017-01-01

Many countries, like Denmark, have tailored Disease Management Programs (DMPs) based on patients having single chronic diseases [defined institutionally as "program diseases" (PDs)], which can complicate treatment for those with multiple chronic diseases. The aims of this study were (a) to assess...... the prevalence and overlap among acutely hospitalized older medical patients of PDs defined by the DMPs, and (b) to examine transitions between different departments during hospitalization and mortality and readmission within two time intervals among patients with the different PDs. We conducted a registry study...... of 4649 acutely hospitalized medical patients ≥65 years admitted to Copenhagen University Hospital, Hvidovre, Denmark, in 2012, and divided patients into six PD groups (type 2 diabetes, chronic obstructive pulmonary disease, cardiovascular disease, musculoskeletal disease, dementia and cancer), each...

Using internet search queries for infectious disease surveillance: screening diseases for suitability.

Science.gov (United States)

Milinovich, Gabriel J; Avril, Simon M R; Clements, Archie C A; Brownstein, John S; Tong, Shilu; Hu, Wenbiao

2014-12-31

Internet-based surveillance systems provide a novel approach to monitoring infectious diseases. Surveillance systems built on internet data are economically, logistically and epidemiologically appealing and have shown significant promise. The potential for these systems has increased with increased internet availability and shifts in health-related information seeking behaviour. This approach to monitoring infectious diseases has, however, only been applied to single or small groups of select diseases. This study aims to systematically investigate the potential for developing surveillance and early warning systems using internet search data, for a wide range of infectious diseases. Official notifications for 64 infectious diseases in Australia were downloaded and correlated with frequencies for 164 internet search terms for the period 2009-13 using Spearman's rank correlations. Time series cross correlations were performed to assess the potential for search terms to be used in construction of early warning systems. Notifications for 17 infectious diseases (26.6%) were found to be significantly correlated with a selected search term. The use of internet metrics as a means of surveillance has not previously been described for 12 (70.6%) of these diseases. The majority of diseases identified were vaccine-preventable, vector-borne or sexually transmissible; cross correlations, however, indicated that vector-borne and vaccine preventable diseases are best suited for development of early warning systems. The findings of this study suggest that internet-based surveillance systems have broader applicability to monitoring infectious diseases than has previously been recognised. Furthermore, internet-based surveillance systems have a potential role in forecasting emerging infectious disease events, especially for vaccine-preventable and vector-borne diseases.

Chronic kidney disease

African Journals Online (AJOL)

disease, together with other related non -communicable diseases. (NCDs), poses not only a threat ... but because if we do not act against NCDs we will also be increasing individual and ... respiratory diseases and cancer. This is in recognition ...

Parkinson's Disease Videos

Medline Plus

Full Text Available ... We Walk at Moving Day CareMAP: Managing Caregiver Stress Aware in Care: Real Stories CareMAP: End-of- ... Progression of the Disease? OHSU - Parkinson's Disease: Pharmacological Management of Depression, Anxiety & Psychosis Overview of Parkinson's Disease ...

Parkinson's Disease Videos

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Full Text Available ... Nonmotor Symptoms of Parkinson's Disease Expert Briefings: Gait, Balance and Falls in Parkinson's Disease Expert Briefings: Coping ... and Parkinson's Disease? Hallucinations and Delusions CareMAP: Balancing Life and Caregiving CareMAP: Travel and Transportation: Part 1 ...

Parkinson's Disease Videos

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Full Text Available ... Nonmotor Symptoms of Parkinson's Disease Expert Briefings: Gait, Balance and Falls in Parkinson's Disease Expert Briefings: Coping ... Foundation How Is Parkinson's Disease Diagnosed? CareMAP: Balancing Life and Caregiving CareMAP: Travel and Transportation: Part 1 ...

Parkinson's Disease Videos

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Full Text Available ... Parkinson's Disease: One Voice, Many Listeners Expert Briefings: Medical Therapies: What's in the Parkinson's Pipeline? Expert Briefings: Under-recognized Nonmotor Symptoms of Parkinson's Disease Expert Briefings: Gait, Balance and Falls in Parkinson's Disease Expert Briefings: Coping ...

Addison′s disease

OpenAIRE

Soumya Brata Sarkar; Subrata Sarkar; Supratim Ghosh; Subhankar Bandyopadhyay

2012-01-01

Addison's disease is a rare endocrinal disorder, with several oral and systemic manifestations. A variety of pathological processes may cause Addison's disease. Classically, hyperpigmentation is associated with the disease, and intraoral pigmentation is perceived as the initial sign and develops earlier than the dermatological pigmentation. The symptoms of the disease usually progress slowly and an event of illness or accident can make the condition worse and may lead to a life-threatening cr...

[Tick-borne diseases].

Science.gov (United States)

Tissot Dupont, H; Raoult, D

1993-05-01

Due to their worldwide distribution, from hottest to coldest climates, and due to their behaviour, ticks are capable of transmitting numerous human and animal bacterial viral or parasitous diseases. Depending on the disease, they play the role of biological vector or intermediate host. In France, six tick borne diseases are of epidemiologic importance. Q fever (not often tick-borne), Mediterranean Spotted Fever, Lyme disease, Turalemia (human and animal), Babesiosis and Tick-borne Viral Encephalitis.

Staphylococcal disease in Africa: another neglected 'tropical' disease

NARCIS (Netherlands)

Herrmann, Mathias; Abdullah, Salim; Alabi, Abraham; Alonso, Pedro; Friedrich, Alexander W.; Fuhr, Günther; Germann, Anja; Kern, Winfried V.; Kremsner, Peter G.; Mandomando, Inacio; Mellmann, Alexander C.; Pluschke, Gerd; Rieg, Siegbert; Ruffing, Ulla; Schaumburg, Frieder; Tanner, Marcel; Peters, Georg; von Briesen, Hagen; von Eiff, Christof; von Müller, Lutz; Grobusch, Martin P.

2013-01-01

The term 'neglected tropical diseases' predominantly refers to single-entity, mostly parasitic diseases. However, a considerable morbidity and mortality burden is carried by patients infected with Gram-positive cocci and Gram-negative bacilli that are prevalent all over the world, yet have impact in

Staphylococcal disease in Africa : another neglected 'tropical' disease

NARCIS (Netherlands)

Herrmann, Mathias; Abdullah, Salim; Alabi, Abraham; Alonso, Pedro; Friedrich, Alexander W.; Fuhr, Guenther; Germann, Anja; Kern, Winfried V.; Kremsner, Peter G.; Mandomando, Inacio; Mellmann, Alexander C.; Pluschke, Gerd; Rieg, Siegbert; Ruffing, Ulla; Schaumburg, Frieder; Tanner, Marcel; von Briesen, Hagen; von Eiff, Christof; von Mueller, Lutz; Grobusch, Martin P.; Peters, Georg

The term 'neglected tropical diseases' predominantly refers to single-entity, mostly parasitic diseases. However, a considerable morbidity and mortality burden is carried by patients infected with Gram-positive cocci and Gram-negative bacilli that are prevalent all over the world, yet have impact in

Addison's disease secondary to connective tissue diseases: a report of six cases.

Science.gov (United States)

Zhang, Zhuo-li; Wang, Yu; Zhou, Wei; Hao, Yan-jie

2009-04-01

Addison's disease is an autoimmune process. However, Addison's disease associated with connective tissue diseases (CTD) is only occasionally reported. Here, we report six cases of Addison's disease secondary to a variety of CTD, which include systemic lupus erythematosus, Takayasu arteritis, systemic sclerosis, ankylosing spondylitis (AS) and antiphospholipid antibody syndrome. The association of Addison's disease with Takayasu arteritis and AS is reported for the first time. We also found high prevalence of hypothyroidism as concomitant autoimmune disorder. Our case series highlight the autoimmune features of Addison's disease. Therefore, we suggest considering adrenal dysfunction in patients with CTD.

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 ...

Heart disease and diet

Science.gov (United States)

Diet - heart disease; CAD - diet; Coronary artery disease - diet; Coronary heart disease - diet ... diet and lifestyle can reduce your risk of: Heart disease, heart attacks, and stroke Conditions that lead ...

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...

Hirschsprung disease.

Science.gov (United States)

Haricharan, Ramanath N; Georgeson, Keith E

2008-11-01

Hirschsprung disease is a relatively common condition managed by pediatric surgeons. Significant advances have been made in understanding its etiologies in the last decade, especially with the explosion of molecular genetic techniques and early diagnosis. The surgical management has progressed from a two- or three-stage procedure to a primary operation. More recently, definitive surgery for Hirschsprung disease through minimally invasive techniques has gained popularity. In neonates, the advancement of treatment strategies for Hirschsprung disease continues with reduced patient morbidity and improved outcomes.

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

DISEASES: text mining and data integration of disease-gene associations.

Science.gov (United States)

Pletscher-Frankild, Sune; Pallejà, Albert; Tsafou, Kalliopi; Binder, Janos X; Jensen, Lars Juhl

2015-03-01

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. The DISEASES resource is accessible through a web interface at http://diseases.jensenlab.org/, where the text-mining software and all associations are also freely available for download. Copyright © 2014 The Authors. Published by Elsevier Inc. All rights reserved.

Gallstone disease and mortality

DEFF Research Database (Denmark)

Shabanzadeh, Daniel Mønsted; Sørensen, Lars Tue; Jørgensen, Torben

2017-01-01

OBJECTIVES: The objective of this cohort study was to determine whether subjects with gallstone disease identified by screening of a general population had increased overall mortality when compared to gallstone-free participants and to explore causes of death. METHODS: The study population (N...... built. RESULTS: Gallstone disease was present in 10%. Mortality was 46% during median 24.7 years of follow-up with 1% lost. Overall mortality and death from cardiovascular diseases were significantly associated to gallstone disease. Death from unknown causes was significantly associated to gallstone...... disease and death from cancer and gastrointestinal disease was not associated. No differences in mortality for ultrasound-proven gallstones or cholecystectomy were identified. CONCLUSIONS: Gallstone disease is associated with increased overall mortality and to death from cardiovascular disease. Gallstones...

Gaucher Disease

Science.gov (United States)

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

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 ...

Sandhoff Disease

Science.gov (United States)

... which had been particularly high in people of Eastern European and Ashkenazi Jewish descent, but Sandhoff disease ... which had been particularly high in people of Eastern European and Ashkenazi Jewish descent, but Sandhoff disease ...

Coeliac disease

DEFF Research Database (Denmark)

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

2018-01-01

Coeliac disease is increasingly recognized as a global problem in both children and adults. Traditionally, the findings of characteristic changes of villous atrophy and increased intraepithelial lymphocytosis identified in duodenal biopsy samples taken during upper gastrointestinal endoscopy have...... been required for diagnosis. Although biopsies remain advised as necessary for the diagnosis of coeliac disease in adults, European guidelines for children provide a biopsy-sparing diagnostic pathway. This approach has been enabled by the high specificity and sensitivity of serological testing. However......, 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...

Quiz: Alzheimer's Disease Quiz | Alzheimer's disease | NIH MedlinePlus the Magazine

Science.gov (United States)

... of this page please turn Javascript on. Feature: Alzheimer's Disease Quiz: Alzheimer's Disease Quiz Past Issues / Fall 2010 Table of ... How many people in the United States have Alzheimer's disease? as many as 5.1 million as ...

Parkinson's Disease Videos

Medline Plus

Full Text Available ... Nonmotor Symptoms of Parkinson's Disease Expert Briefings: Gait, Balance and Falls in Parkinson's Disease Expert Briefings: Coping ... How Does Parkinson's Disease Affect Memory? CareMAP: Balancing Life and Caregiving CareMAP: La Alimentación y la Deglución, ...

Peptic Ulcer Disease

Science.gov (United States)

... Site ACG Patients Home / Digestive Health Topic / Peptic Ulcer Disease Peptic Ulcer Disease Basics Overview An “ulcer” is an open ... for pain in patients at risk for peptic ulcer disease. Peptic – caused by acid. PPIs – P roton P ump ...

Alzheimer disease

Science.gov (United States)

... likely need to plan for their loved one's future care. The final phase of the disease may ... disease and other dementias. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine . 25th ed. Philadelphia, PA: ...

Celiac disease and other autoimmune diseases in patients with collagenous colitis.

Science.gov (United States)

Vigren, Lina; Tysk, Curt; Ström, Magnus; Kilander, Anders F; Hjortswang, Henrik; Bohr, Johan; Benoni, Cecilia; Larson, Lasse; Sjöberg, Klas

2013-08-01

Collagenous colitis (CC) is associated with autoimmune disorders. The aim of the present study was to investigate the relationship between CC and autoimmune disorders in a Swedish multicenter study. Patients with CC answered questionnaires about demographic data and disease activity. The patient's files were scrutinized for information about autoimmune diseases. A total number of 116 CC patients were included; 92 women, 24 men, median age 62 years (IQR 55-73). In total, 30.2% had one or more autoimmune disorder. Most common were celiac disease (CeD; 12.9%) and autoimmune thyroid disease (ATD, 10.3%), but they also had Sjögren's syndrome (3.4%), diabetes mellitus (1.7%) and conditions in skin and joints (6.0%). Patients with associated autoimmune disease had more often nocturnal stools. The majority of the patients with associated CeD or ATD got these diagnoses before the colitis diagnosis. Autoimmune disorders occurred in one-third of these patients, especially CeD. In classic inflammatory bowel disease (IBD), liver disease is described in contrast to CC where no cases occurred. Instead, CeD was prevalent, a condition not reported in classic IBD. Patients with an associated autoimmune disease had more symptoms. Patients with CC and CeD had an earlier onset of their colitis. The majority of the patients with both CC and CeD were smokers. Associated autoimmune disease should be contemplated in the follow-up of these patients.

Coronary artery disease in patients with cerebrovascular disease: a prospective study

International Nuclear Information System (INIS)

Rokey, R.; Rolak, L.A.; Harati, Y.; Kutka, N.; Verani, M.S.

1984-01-01

Coronary artery disease is the cause of death in most patients who have transient ischemic attacks or stroke. Evaluation for this condition is not routinely performed in such patients, and no prospective studies have been reported. We prospectively examined 50 consecutive patients with transient ischemic attacks or mild stroke to determine the prevalence and importance of coronary artery disease. All patients were examined by a cardiologist and underwent both exercise thallium-201 scintigraphy and exercise radionuclide ventriculography. Sixteen patients were suspected to have coronary artery disease on the basis of clinical evaluation. In 15 of these the was confirmed by the nuclear scans. The remaining 34 patients had no clinical evidence of heart disease, yet 14 had abnormal cardiac scans. Twenty of 22 patients with abnormal scans who underwent cardiac catheterization had significant coronary artery disease or a cardiomyopathy. The discovery of heart disease altered clinical management in 13 patients. Overall, 29 of 50 patients had significant coronary artery disease, compared with a 7% prevalence of the condition in other patients of similar age at the same institution

Genetics and Rheumatic Disease

Science.gov (United States)

... Well with Rheumatic Disease Genetics and Rheumatic Disease Genetics and Rheumatic Disease Fast Facts Studying twins has ... 70%, and for non-identical pairs, even lower. Genetics and ankylosing spondylitis Each rheumatic disease has its ...

What Is Celiac Disease?

Science.gov (United States)

... Disease" Articles Celiac Disease Changes Everything / What is Celiac Disease? / Symptoms, Diagnosis and Treatment / Four Inches and Seven Pounds… / Learning to Live Well with Celiac Disease / Living Gluten-Free Spring 2015 Issue: Volume 10 ...

Celiac Disease Changes Everything

Science.gov (United States)

... Disease" Articles Celiac Disease Changes Everything / What is Celiac Disease? / Symptoms, Diagnosis and Treatment / Four Inches and Seven Pounds… / Learning to Live Well with Celiac Disease / Living Gluten-Free Spring 2015 Issue: Volume 10 ...

Heart disease - risk factors

Science.gov (United States)

Heart disease - prevention; CVD - risk factors; Cardiovascular disease - risk factors; Coronary artery disease - risk factors; CAD - risk ... a certain health condition. Some risk factors for heart disease you cannot change, but some you can. ...

Addison Disease

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

Lyme Disease

Science.gov (United States)

... spread to the nervous system, causing facial paralysis ( Bell's palsy ), or meningitis. The last stage of Lyme disease ... My Lyme Disease Risk? Bug Bites and Stings Bell's Palsy Rocky Mountain Spotted Fever Meningitis View more About ...

Refsum Disease

Science.gov (United States)

... night blindness due to degeneration of the retina (retinitis pigmentosa). If the disease progresses, other symptoms may include ... night blindness due to degeneration of the retina (retinitis pigmentosa). If the disease progresses, other symptoms may include ...

Epigenetics of kidney disease.

Science.gov (United States)

Wanner, Nicola; Bechtel-Walz, Wibke

2017-07-01

DNA methylation and histone modifications determine renal programming and the development and progression of renal disease. The identification of the way in which the renal cell epigenome is altered by environmental modifiers driving the onset and progression of renal diseases has extended our understanding of the pathophysiology of kidney disease progression. In this review, we focus on current knowledge concerning the implications of epigenetic modifications during renal disease from early development to chronic kidney disease progression including renal fibrosis, diabetic nephropathy and the translational potential of identifying new biomarkers and treatments for the prevention and therapy of chronic kidney disease and end-stage kidney disease.

Management of adynamic bone disease in chronic kidney disease: A brief review

Directory of Open Access Journals (Sweden)

Swathi K. Sista

2016-09-01

Full Text Available The Kidney Disease: Improving Global Outcomes (KDIGO work group released recommendations in 2006 to define the bone-related pathology associated with chronic kidney disease as renal osteodystrophy. In 2009, KDIGO released revised clinical practice guidelines which redefined systemic disorders of bone and mineral metabolism due to chronic kidney disease as chronic kidney disease-mineral and bone disorders. Conditions under this overarching term include osteitis fibrosa cystica, osteomalacia, and adynamic bone disease. We aim to provide a brief review of the histopathology, pathophysiology, epidemiology, and diagnostic features of adynamic bone disease, focusing on current trends in the management of this complex bone disorder.

Parkinson's Disease Videos

Science.gov (United States)

... Nonmotor Symptoms of Parkinson's Disease Expert Briefings: Gait, Balance and Falls in Parkinson's Disease Expert Briefings: Coping Skills for ... Caregivers: Caremap and Caring & Coping CareMAP: Movement and Falls: Part ... There Any Ways to Control the Rate of Progression of the Disease? CareMAP: ...

Immunoglobulin G4-related disease: a rare disease with an unusual presentation.

Science.gov (United States)

Khan, Muhammad Waqas; Hadley, Terrance; Kesler, Melissa; Gul, Zartash

2016-07-01

IgG4-RD can also present in the skeletal muscle, mimicking several other diseases. It is unusual for this relatively new classification of diseases to present in the muscles and can be mistakenly diagnosed as other autoimmune diseases rendering a delay in the appropriate management and progression of the disease.

Kidney Disease

Science.gov (United States)

... Staying Safe Videos for Educators Search English Español Kidney Disease KidsHealth / For Teens / Kidney Disease What's in ... Coping With Kidney Conditions Print What Do the Kidneys Do? You might never think much about some ...

Undifferentiated Connective Tissue Disease

Science.gov (United States)

... Home Conditions Undifferentiated Connective Tissue Disease (UCTD) Undifferentiated Connective Tissue Disease (UCTD) Make an Appointment Find a Doctor ... by Barbara Goldstein, MD (February 01, 2016) Undifferentiated connective tissue disease (UCTD) is a systemic autoimmune disease. This ...

American Lyme Disease Foundation

Science.gov (United States)

... Infectious Diseases, 35: 451-464, 2002) What is Lyme Disease? Lyme disease (LD) is an infection caused by ... mission with your own tax-deductible contribution. American Lyme Disease Foundation, Inc. PO Box 466 Lyme, CT 06371 ...

Inflammation and Heart Disease

Science.gov (United States)

... Disease Venous Thromboembolism Aortic Aneurysm More Inflammation and Heart Disease Updated:Jun 13,2017 Understand the risks of ... inflammation causes cardiovascular disease, inflammation is common for heart disease and stroke patients and is thought to be ...

Parkinson's Disease Videos

Medline Plus

Full Text Available ... Nonmotor Symptoms of Parkinson's Disease Expert Briefings: Gait, Balance and Falls in Parkinson's Disease Expert Briefings: Coping Skills for ... Caregivers: Caremap and Caring & Coping CareMAP: Movement and Falls: Part ... There Any Ways to Control the Rate of Progression of the Disease? CareMAP: ...

Sexual transmission of Lyme disease: challenging the tickborne disease paradigm.

Science.gov (United States)

Stricker, Raphael B; Middelveen, Marianne J

2015-01-01

Lyme disease caused by the spirochete Borrelia burgdorferi has become a major worldwide epidemic. In this article, we explore the clinical, epidemiological and experimental evidence for sexual transmission of Lyme disease in animal models and humans. Although the likelihood of sexual transmission of the Lyme spirochete remains speculative, the possibility of Lyme disease transmission via intimate human contact merits further study.

Hodgkin's disease part 1: pathology, staging, and management of early stage disease

International Nuclear Information System (INIS)

Mauch, Peter; Yahalom, Joachim

1995-01-01

Over the past 25 years there have been dramatic improvements in our understanding of the epidemiology, biology, natural history, and treatment of Hodgkins disease. Hodgkin's disease is one of the few cancers where both chemotherapy and radiation therapy have provided dramatic improvements in cure of this once uniformly fatal disease. Part 1 of the refresher course on Hodgkin's disease will include a review of: 1) New Findings in epidemiology, immunohistochemistry and molecular biology of the Reed-Sternberg cell including association with Epstein-Barr virus; 2) Review of pathology including discussions of NS 1 vs NS2, and nodular LP subclassifications; 3) Recommendations for staging including the role of staging laparotomy in Hodgkin's disease; 4) Standard techniques for commonly used radiation therapy fields for Hodgkin's disease and 5) Treatment of early stage Hodgkin's disease including an overview on recent and current clinical trials

Heart disease and women

Science.gov (United States)

... this page: //medlineplus.gov/ency/article/007188.htm Heart disease and women To use the sharing features on ... please enable JavaScript. People often DO NOT consider heart disease a woman's disease. Yet cardiovascular disease is the ...

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 ...

Digestive Diseases

Science.gov (United States)

... Lactose Intolerance Liver Disease Ménétrier’s Disease Microscopic Colitis Ostomy Surgery of the Bowel Pancreatitis Peptic Ulcers (Stomach ... and outreach materials. Clinical Trials Clinical trials offer hope for many people and opportunities to help researchers ...

Viral Disease Networks?

Science.gov (United States)

Gulbahce, Natali; Yan, Han; Vidal, Marc; Barabasi, Albert-Laszlo

2010-03-01

Viral infections induce multiple perturbations that spread along the links of the biological networks of the host cells. Understanding the impact of these cascading perturbations requires an exhaustive knowledge of the cellular machinery as well as a systems biology approach that reveals how individual components of the cellular system function together. Here we describe an integrative method that provides a new approach to studying virus-human interactions and its correlations with diseases. Our method involves the combined utilization of protein - protein interactions, protein -- DNA interactions, metabolomics and gene - disease associations to build a ``viraldiseasome''. By solely using high-throughput data, we map well-known viral associated diseases and predict new candidate viral diseases. We use microarray data of virus-infected tissues and patient medical history data to further test the implications of the viral diseasome. We apply this method to Epstein-Barr virus and Human Papillomavirus and shed light into molecular development of viral diseases and disease pathways.

Coeliac disease and epilepsy.

LENUS (Irish Health Repository)

Cronin, C C

2012-02-03

Whether there is an association between coeliac disease and epilepsy is uncertain. Recently, a syndrome of coeliac disease, occipital lobe epilepsy and cerebral calcification has been described, mostly in Italy. We measured the prevalence of coeliac disease in patients attending a seizure clinic, and investigated whether cerebral calcification occurred in patients with both coeliac disease and epilepsy. Screening for coeliac disease was by IgA endomysial antibody, measured by indirect immunofluorescence using sections of human umbilical cord. Of 177 patients screened, four patients were positive. All had small-bowel histology typical of coeliac disease. The overall frequency of coeliac disease in this mixed patient sample was 1 in 44. In a control group of 488 pregnant patients, two serum samples were positive (1 in 244). Sixteen patients with both coeliac disease and epilepsy, who had previously attended this hospital, were identified. No patient had cerebral calcification on CT scanning. Coeliac disease appears to occur with increased frequency in patients with epilepsy, and a high index of suspicion should be maintained. Cerebral calcification is not a feature of our patients with epilepsy and coeliac disease, and may be an ethnically-or geographically-restricted finding.

National disease management plans for key chronic non-communicable diseases in Singapore.

Science.gov (United States)

Tan, C C

2002-07-01

In Singapore, chronic, non-communicable diseases, namely coronary heart disease, stroke and cancer, account for more than 60% of all deaths and a high burden of disability and healthcare expenditure. The burden of these diseases is likely to rise with our rapidly ageing population and changing lifestyles, and will present profound challenges to our healthcare delivery and financing systems over the next 20 to 30 years. The containment and optimal management of these conditions require a strong emphasis on patient education and the development of integrated models of healthcare delivery in place of the present uncoordinated, compartmentalised way of delivering healthcare. To meet these challenges, the Ministry of Health's major thrusts are disease control measures which focus mainly on primary prevention; and disease management, which coordinates the national effort to reduce the incidence of these key diseases and their predisposing factors and to ameliorate their long-term impact by optimising control to reduce mortality, morbidity and complications, and improving functional status through rehabilitation. The key initiatives include restructuring of the public sector healthcare institutions into two clusters, each comprising a network of primary health care polyclinics, regional hospitals and tertiary institutions. The functional integration of these healthcare elements within each cluster under a common senior administrative and professional management, and the development of common clinical IT systems will greatly facilitate the implementation of disease management programmes. Secondly, the Ministry is establishing National Disease Registries in coronary heart disease, cancer, stroke, myopia and kidney failure, which will be valuable sources of clinical and outcomes data. Thirdly, in partnership with expert groups, national committees and professional agencies, the Ministry will produce clinical practice guidelines which will assist doctors and healthcare

Pediatric Inflammatory Bowel Diseases

DEFF Research Database (Denmark)

Lauritzen, Didde; Andreassen, Bente Utoft; Heegaard, Niels Henrik H

2018-01-01

Background: Kidney disease has been reported in adults with inflammatory bowel disease (IBD) and is regarded an extraintestinal manifestation or more rarely a side effect of the medical treatment. Methods: In this cross-sectional study we describe the extent of kidney pathology in a cohort of 56...... children with IBD. Blood and urine samples were analyzed for markers of kidney disease and ultrasonography was performed to evaluate pole-to-pole kidney length. Results: We found that 25% of the patients had either previously reported kidney disease or ultrasonographic signs of chronic kidney disease...... are at risk of chronic kidney disease, and the risk seems to be increased with the severity of the disease....

Norries disease

Directory of Open Access Journals (Sweden)

Saini J

1992-01-01

Full Text Available A 2-month-old male infant was found to have Norrie′s disease. The clinical presentation and detailed histological features diagnostic of the disease are discussed. This is the first authentic, histologically proven case of Norrie′s disease from India. The absence of hearing loss and mental retardation at the time of presentation at the early stage of infancy and the fact that the case was sporadic do not detract from the diagnosis. However the child at the age of one year developed hearing loss.

Menkes Disease

Science.gov (United States)

... SEARCH Definition Treatment Prognosis Clinical Trials Organizations Publications Definition Menkes disease is caused by a defective gene named ATPTA ... arteries. Weakened bones (osteoporosis) may result in fractures. × Definition Menkes disease is caused by a defective gene named ATPTA ...

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 ... severe symptoms similar to males with the disorder. × Definition Fabry disease is caused by the lack of or faulty ...

Fifth disease

Science.gov (United States)

Parvovirus B19; Erythema infectiosum; Slapped cheek rash ... Fifth disease is caused by human parvovirus B19. It often affects preschoolers or school-age children during the spring. The disease spreads through the fluids in the nose and mouth ...

Gastro-esophageal reflux disease and exacerbations in chronic obstructive pulmonary disease.

Science.gov (United States)

Ingebrigtsen, Truls S; Marott, Jacob L; Vestbo, Jørgen; Nordestgaard, Børge G; Hallas, Jesper; Lange, Peter

2015-01-01

We tested the hypothesis that gastro-esophageal reflux disease is a risk factor for exacerbations in individuals with chronic obstructive pulmonary disease (COPD). Among 9622 participants in the Copenhagen City Heart Study, we identified 1259 individuals with COPD and information on gastro-esophageal reflux disease and the regular use of acid inhibitory treatment. These individuals were followed for 5 years with regard to medically treated COPD exacerbations, which we defined as a short course treatment with oral corticosteroids alone or in combination with antibiotics. We applied a multivariable Cox regression analysis with adjustment for well-established risk factors associated with COPD exacerbations or gastro-esophageal reflux disease, including COPD severity, and symptoms. Individuals with COPD and gastro-esophageal reflux disease had more chronic bronchitis (31 vs 21%, P = 0.004), more breathlessness (39 vs 22%, P gastro-esophageal reflux disease. Among individuals with COPD and gastro-esophageal reflux disease, those who did not use acid inhibitory treatment regularly had an increased risk of COPD exacerbations during follow-up, hazards ratio (HR): HR = 2.7 (1.3-5.4, P = 0.006). Individuals with gastro-esophageal reflux disease, using acid inhibitory treatment regularly did not have an increased risk of exacerbations, HR = 1.2 (0.6-2.7, P = 0.63). Gastro-esophageal reflux disease was associated with an increased risk of medically treated exacerbations of COPD, but only in those individuals who did not use acid inhibitory treatment regularly. © 2014 Asian Pacific Society of Respirology.