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Pemphigus Foliaceus

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121. Data on HLA class I/II profile in Brazilian pemphigus patients Full Text available with Trip Pro

Data on HLA class I/II profile in Brazilian pemphigus patients Pemphigus are blistering autoimmune diseases related with genetic and environmental factors. Here we describe HLA genotyping in pemphigus patients. First, we review the HLA class I/II data on pemphigus reported in Brazilian samples and then present the HLA class I (-A, -B, -C) and class II (-DRB1, -DQA1, -DQB1) alleles related to susceptibility/resistance to pemphigus by comparing 86 patients with pemphigus foliaceus, 83 patients (...) with pemphigus vulgaris, and 1592 controls from the northeastern region of the state of São Paulo, Southeastern Brazil. The data presented here are related to the manuscript "Differential HLA class I and class II associations in Pemphigus Foliaceus and Pemphigus Vulgaris patients from a prevalent Southeastern Brazilian region" Brochado et al. (2016) [1].

2016 Data in brief

122. Validation of chemiluminescent enzyme immunoassay in detection of autoantibodies in pemphigus and pemphigoid. (Abstract)

define cut-off values for Dsg1, Dsg3, and BP180, we evaluated 47 serum samples from patients with pemphigus foliaceus (PF), 59 from those with pemphigus vulgaris (PV), 52 from those with bullous pemphigoid (BP), and 995 from healthy individuals. We also evaluated any fluctuations in CLEIA titers according to disease activity during the clinical course of 10 cases each of PF, PV, and BP. We used clinical symptom scores, the pemphigus disease area index (PDAI) and the bullous pemphigoid disease area (...) Validation of chemiluminescent enzyme immunoassay in detection of autoantibodies in pemphigus and pemphigoid. A novel chemiluminescent enzyme immunoassay (CLEIA) was recently developed to quantify autoantibodies specific for desmogleins (Dsgs) and BP180, the target antigens of pemphigus and pemphigoid. This assay is automated and highly accurate and efficient.To validate the use of the CLEIA for detection of autoantibodies during the clinical courses of patients with pemphigus and pemphigoid.To

2016 Journal of dermatological science

123. Pemphigus Foliaceus

Pemphigus Foliaceus Pemphigus Foliaceus - Dermatologic Disorders - MSD Manual Professional Edition Brought to you by The trusted provider of medical information since 1899 SEARCH SEARCH MEDICAL TOPICS Common Health Topics Resources QUIZZES & CASES Quizzes Cases The trusted provider of medical information since 1899 SEARCH SEARCH MEDICAL TOPICS Common Health Topics Resources QUIZZES & CASES Quizzes Cases / / / / IN THIS TOPIC OTHER TOPICS IN THIS CHAPTER Test your knowledge Diagnostic Tests (...) University Click here for Patient Education NOTE: This is the Professional Version. CONSUMERS: Pemphigus foliaceus is an autoimmune blistering disorder in which splits in the superficial epidermis result in cutaneous erosions. Bullae are elevated, fluid-filled blisters ≥ 10 mm in diameter. Pemphigus foliaceus usually occurs in middle-aged patients, affecting men and women in equal numbers. An endemic form of pemphigus foliaceus, fogo selvagem, occurs in younger adults and children, particularly in South

2013 Merck Manual (19th Edition)

124. E-cadherin autoantibody profile in Pemphigus Vulgaris (E-cadherin autoantibody profile in Pemphigus Vulgaris patients). (Abstract)

E-cadherin autoantibody profile in Pemphigus Vulgaris (E-cadherin autoantibody profile in Pemphigus Vulgaris patients). Pemphigus vulgaris (PV) is an autoimmune skin blistering disease. The main targets of autoantibodies are the desmosomal proteins desmoglein (Dsg)3 and Dsg1. Anti-E-cadherin antibody is the second most frequent antibody found in pemphigus foliaceus (fogo selvagem), but the frequency in PV is unknown.To determine the anti-E-cadherin antibody profile in the two major subtypes

2013 British Journal of Dermatology

125. Immune response in pemphigus and beyond: progresses and emerging concepts. (Abstract)

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

2015 Seminars in immunopathology

126. A Randomized, Double-Blind Study to Evaluate the Efficacy and Safety of Rituximab Versus MMF in Patients With Pemphigus Vulgaris

study participation Able to comply with the study protocol, in the investigator's judgment Exclusion Criteria: Diagnosis of pemphigus foliaceus or evidence of paraneoplastic pemphigus or other non-PV autoimmune blistering disease History of a severe allergic or anaphylactic reaction to humanized or murine monoclonal antibodies, or known hypersensitivity to any component of rituximab Known hypersensitivity or contraindication to MMF, mycophenolic acid, polysorbate, or oral corticosteroids Lack (...) A Randomized, Double-Blind Study to Evaluate the Efficacy and Safety of Rituximab Versus MMF in Patients With Pemphigus Vulgaris A Study to Evaluate the Efficacy and Safety of Rituximab Versus Mycophenolate Mofetil (MMF) in Participants With Pemphigus Vulgaris (PV) - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You

2015 Clinical Trials

127. Wound Dressings for Pemphigus and Pemphigoid

Inclusion Criteria: Diagnosis of pemphigus vulgaris, pemphigus foliaceus , pemphigus vegetans, paraneoplastic pemphigus or bullous pemphigoid At least four areas of active disease in the trunk or limbs of 8 x 8 cm characterized by denudation, ulceration, scabs or blisters. Capable of being evaluated weekly either as outpatient or hospitalized Actively treated with immunosuppressive or immunomodulatory drugs for pemphigus or pemphigoid control Willing to sign an informed consent Exclusion Criteria (...) Wound Dressings for Pemphigus and Pemphigoid Wound Dressings for Pemphigus and Pemphigoid - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Wound Dressings for Pemphigus and Pemphigoid The safety

2015 Clinical Trials

128. Usefulness of specific anti-desmoglein 1 and 3 enzyme-linked immunoassay and indirect immunofluorescence in the evaluation of pemphigus activity. (Abstract)

vulgaris (PV) and nine with pemphigus foliaceus (PF) during the course of the disease. In each sample, we measured anti-Dsg1 and anti-Dsg3 antibodies by ELISA. A receiver operating characteristics (ROC) curve was calculated to determine a cutoff value for anti-Dsg1 and anti-Dsg3 antibodies with optimal sensitivity and specificity. In 263 samples, we compared the ROC curves of anti-Dsg1 and anti-Dsg3 antibodies with the ROC curves of the IIF results.Activity of pemphigus was associated with a wide range (...) Usefulness of specific anti-desmoglein 1 and 3 enzyme-linked immunoassay and indirect immunofluorescence in the evaluation of pemphigus activity. The objective was to assess the relationship between enzyme-linked immunoassay (ELISA) values of desmoglein (Dsg) 1 and Dsg3 antibodies and indirect immunofluorescence (IIF) values of anti-epithelial antibodies with disease activity in patients with pemphigus.In a retrospective study, we analyzed 353 serum samples taken from 35 patients with pemphigus

2015 International Journal of Dermatology

129. Analysis of the reactivity of indirect immunofluorescence in patients with pemphigus foliaceus and pemphigus vulgaris using rat bladder epithelium as a substrate Full Text available with Trip Pro

Analysis of the reactivity of indirect immunofluorescence in patients with pemphigus foliaceus and pemphigus vulgaris using rat bladder epithelium as a substrate To evaluate the reactivity of indirect immunofluorescence using rat bladder epithelium as a substrate in patients with pemphigus foliaceus and pemphigus vulgaris from the Department of Dermatology, University of São Paulo Medical School, Brazil.Thirty-two patients (8 male and 24 female) from the Department of Dermatology, University (...) of São Paulo Medical School, were selected. Three had mucosal pemphigus vulgaris, 20 had mucocutaneous pemphigus vulgaris, and 9 had pemphigus foliaceus. Patients' sera were tested by indirect immunofluorescence performed on human foreskin and rat bladder epithelium and by ELISA assays utilizing baculovirus-expressed recombinant desmoglein 3 and desmoglein 1.No patients with mucosal pemphigus vulgaris, 5 of 20 patients with mucocutaneous pemphigus vulgaris (25%) and 4 of 9 patients with pemphigus

2011 Clinics

130. Vulvo-cervico-vaginal manifestations and evaluation of Papanicolaou smears in pemphigus vulgaris and pemphigus foliaceus. (Abstract)

Vulvo-cervico-vaginal manifestations and evaluation of Papanicolaou smears in pemphigus vulgaris and pemphigus foliaceus. Vulvo-cervico-vaginal involvement has rarely been reported in pemphigus vulgaris (PV) and has not been reported in pemphigus foliaceus (PF).We sought to evaluate genital lesions and Papanicolaou (Pap) smears in female patients with PV and PF.This prospective study includes all consecutive cases of female patients with PV and PF seen from May 2009 to February 2010 (...) . Gynecologic examination was performed and Pap smears were collected for cytologic analysis from each patient.A total of 56 patients were given a diagnosis of pemphigus (41 PV and 15 PF). Genital involvement was observed in 9 patients with PV (22%) and the vulva was the most common genital site of involvement. Of these 9 patients, 8 presented with active skin/mucous lesions. Four of 15 patients with PF had genital lesions and vulva was the exclusive site of involvement. Three of 4 patients with PF

2011 Journal of American Academy of Dermatology

131. Durable remission of pemphigus with a fixed-dose rituximab protocol. Full Text available with Trip Pro

intravenously if clinically warranted at 6-month intervals or repeated full dosing).A retrospective cohort study was conducted using records from a tertiary referral center for autoimmune bullous disorders. Participants included 92 patients (pemphigus vulgaris, 84 [91%], and pemphigus foliaceus, 8 [9%]) who received rituximab treatment between May 1, 2006, and August 30, 2012.The primary outcomes were time to relapse and achievement of a complete response with or without treatment at the end (...) Durable remission of pemphigus with a fixed-dose rituximab protocol. Rituximab induces B-lymphocyte apoptosis by targeting CD20 antigen and has shown efficacy in antibody-mediated autoimmune disease. Rituximab is increasingly being acknowledged as an effective and safe treatment option for pemphigus.To assess the clinical response of patients with pemphigus to rituximab using a modified fixed-dose rheumatoid arthritis protocol (1 g intravenously on days 1 and 15, followed by 500 mg

2014 JAMA dermatology (Chicago, Ill.)

132. Correlation of Conventional and Conformational Anti-desmoglein Antibodies with Phenotypes and Disease Activities in Patients with Pemphigus Vulgaris. Full Text available with Trip Pro

Correlation of Conventional and Conformational Anti-desmoglein Antibodies with Phenotypes and Disease Activities in Patients with Pemphigus Vulgaris. Pemphigus is an autoimmune disease characterised by anti-desmoglein (Dsg) antibodies in the serum of patients. The disease can be divided into pemphigus foliaceus and pemphigus vulgaris. Anti-Dsg1 antibody is generally related to pemphigus with cutaneous lesion, and the anti-Dsg3 antibody is related to pemphigus with mucosa lesion. Twenty-nine (...) patients with pemphigus vulgaris were selected in the clinical study. The severity of the cutaneous and mucosa lesions of these patients was evaluated using Pemphigus disease area index (PDAI). Conventional and conformational anti-Dsg index values were determined using enzyme-linked immunosorbent assay (ELISA) and ethylenediaminetetraacetic acid-treated ELISA. The relationship between clinical phenotypes and immunological profiles was analysed. In the correlation analysis, both the conventional

2014 Acta Dermato-Venereologica

133. Specific immunoadsorption of pathogenic autoantibodies in pemphigus requires the entire ectodomains of desmogleins. (Abstract)

Specific immunoadsorption of pathogenic autoantibodies in pemphigus requires the entire ectodomains of desmogleins. Pemphigus foliaceus (PF) and pemphigus vulgaris (PV) are life-threatening autoimmune blistering skin diseases. They are characterized by circulating autoantibodies which bind to the ectodomains of desmoglein (Dsg) 1 and Dsg3. These antibodies induce acantholysis in skin and mucous membranes. In severe cases of pemphigus, immunoadsorption is applied to remove total IgG from patient (...) were mainly conformation-dependent. However, adsorption of pemphigus foliaceus IgG using this protein coupled to a Sepharose matrix did not completely remove pathogenicity from the sera, as proven by a keratinocyte dissociation assay. In contrast, full-length Dsg1 and Dsg3 ectodomains were able to specifically adsorb anti-Dsg antibodies and to efficiently eliminate pathogenicity. Therefore, the complete and correctly folded ectodomains of both desmogleins are required for therapeutic

2014 Experimental Dermatology

134. Prevalence of myasthenia gravis and associated autoantibodies in paraneoplastic pemphigus and their correlations with symptoms and prognosis. (Abstract)

. Serum autoantibodies against acetylcholine receptor (AChR), acetylcholinesterase (AChE), titin, ryanodine receptor (RyR) and muscle-specific kinase (MuSK) were measured by enzyme-linked immunosorbent assay. Patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), connective tissue disease (CTD) and non-PNP MG (NP-MG), and healthy donors, served as controls. These autoantibodies in PNP were also compared in the presence or absence of dyspnoea or muscle weakness. Cox regression and log-rank (...) Prevalence of myasthenia gravis and associated autoantibodies in paraneoplastic pemphigus and their correlations with symptoms and prognosis. Paraneoplastic pemphigus (PNP) involves multiple organs, but little is known about its neurological involvement.To investigate the symptoms, prognosis and profiles of associated autoantibodies in myasthenia gravis (MG), and their correlations in patients with PNP.Fifty-eight patients with PNP were assessed for myasthenic symptoms and laboratory evidence

2014 British Journal of Dermatology

135. Cutaneous pemphigus vulgaris with absence of desmoglein 1 autoantibodies. An example of the extended desmoglein compensation theory. (Abstract)

Cutaneous pemphigus vulgaris with absence of desmoglein 1 autoantibodies. An example of the extended desmoglein compensation theory. A 79 year-old male patient presented clinically as a typical case of pemphigus foliaceus. He displayed a seborrhoeic pattern of crusting and erosions with an absence of oral involvement. Surprisingly, a biopsy showed suprabasilar acantholysis and direct immunofluorescence consistent with pemphigus vulgaris. Indirect pemphigus antibodies were positive (...) for desmoglein 3 (DSG-3) but negative for DSG-1 antibodies. Most cases of cutaneous pemphigus are reported to have both DSG-1 and DSG-3 antibodies. A rare cutaneous subtype of pemphigus vulgaris is reported in the literature that may present clinically as seen in our patient. Our patient is clinically similar to these previous reported cases but with a negative DSG-1 antibody titre. The extended DSG compensation theory explains the differences of clinical expression in pemphigus based on the variable

2014 Australasian Journal of Dermatology

136. Autoantibody Levels and Clinical Disease Severity in Patients with Pemphigus: Comparison of Aggregated Anti-desmoglein ELISA Values and Indirect Immunofluorescence Titres. Full Text available with Trip Pro

values and IIF titres with clinical activity over a longer treatment period are scarce. In our retrospective study, we compared aggregated repetitive intra-patient ELISA values and IIF titres with grades of clinical disease (1 = remission, 2 = moderate activity, 3 = exacerbation) in 47 patients suffering from pemphigus vulgaris (PV, n = 36) and pemphigus foliaceus (PF, n=11). We found that anti-Dsg1 ELISA values in PF and mucocutaneous PV as well as anti-Dsg3 ELISA values in PV best reflect disease (...) Autoantibody Levels and Clinical Disease Severity in Patients with Pemphigus: Comparison of Aggregated Anti-desmoglein ELISA Values and Indirect Immunofluorescence Titres. Detecting serum-autoantibodies by anti-Desmoglein-1 (anti-Dsg1) and anti-Dsg3 ELISAs as well as indirect immunofluorescence (IIF) are established complementary methods to diagnose pemphigus. Whether autoantibody levels also reflect clinical disease activity is still a matter of debate, as head-to-head comparisons of ELISA

2014 Acta Dermato-Venereologica

137. Diagnosis and classification of pemphigus and bullous pemphigoid. (Abstract)

Diagnosis and classification of pemphigus and bullous pemphigoid. Pemphigus and bullous pemphigoid represent the two major groups of autoimmune blistering diseases. Pemphigus has three major variants: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus and is characterized by autoantibodies directed against the cell surface of keratinocytes, producing acantholysis that in turn leads to intraepithelial blisters in the skin and/or mucous membranes. In bullous pemphigoid (...) , the autoantibodies are present at the dermo-epidermal junction and attack the hemidesmosomes, causing subepidermal blister formation. The classification of the major variants of both the pemphigus group and bullous pemphigoid can be based on the combination of clinical, histopathological and immunopathological criteria. Many tools are available for the diagnosis of these entities including biopsy, direct and indirect immunofluorescence, immunoprecipitation, immunoblotting and ELISA. However, currently

2014 Autoimmunity reviews

138. Clinical and immunological profiles in 17 Japanese patients with drug-induced pemphigus studied at Kurume University. (Abstract)

and 2012. Complicated diseases, clinical and histopathological manifestations, responsible drugs and findings in immunofluorescence, enzyme-linked immunosorbent assays (ELISAs), immunoblotting (IB) and prognosis were analysed.Eight of the 17 patients with DIP showed pemphigus foliaceus-like appearance, three showed pemphigus herpetiformis-like appearance, and six showed atypical bullous lesions. Responsible drugs were thiol-containing drugs in 16 patients (bucillamine in nine cases, d-penicillamine (...) -kDa periplakin. Four cases showed anti-Dsg3 antibodies without mucosal lesions. While 11 cases recovered after discontinuation of the causative drugs, six patients had a very protracted or intractable disease course, and might develop true pemphigus.The present study indicated that the majority of the patients with DIP studied showed a pemphigus foliaceus-type phenotype with anti-Dsg1 autoantibodies, caused by thiol-containing drugs.© 2014 British Association of Dermatologists.

2014 British Journal of Dermatology

139. Pemphigus foliaceus in a juvenile Cashmere goat, and outcome after prednisolone and methylprednisolone therapy Full Text available with Trip Pro

Pemphigus foliaceus in a juvenile Cashmere goat, and outcome after prednisolone and methylprednisolone therapy A juvenile Cashmere goat was evaluated and treated for generalized pustular and crusting dermatitis. A diagnosis of pemphigus foliaceus was made based on histopathology and immunohistochemistry. Treatment with prednisolone resulted in resolution of the lesions. Despite tapering doses and combination with methylprednisolone for monthly maintenance, the kid was euthanized for poor growth

2011 The Canadian Veterinary Journal

140. Pemphigus, IgA (Diagnosis)

with having IgA-pemphigus foliaceus (IgA-PF) or IgA-pemphigus vulgaris (IgA-PV), respectively. All other cases not meeting these criteria are diagnosed as unclassified IAD. IgA pemphigus has not been described in animals. Although the term IgA pemphigus has not been established in the veterinary literature, IgA deposition around epidermal cells has been detected by direct immunofluorescence in animals affected with pemphigus foliaceus. Medscape articles on other variants of pemphigus include (...) , Hashimoto T, et al. Autoantibodies against desmocollins in European patients with pemphigus. Clin Exp Dermatol . 2009 Dec. 34(8):898-903. . Chan LS, Vanderlugt CJ, Hashimoto T, et al. Epitope spreading: lessons from autoimmune skin diseases. J Invest Dermatol . 1998 Feb. 110(2):103-9. . Chorzelski TP, Beutner EH, Kowalewski C, et al. IgA pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis. J Am Acad Dermatol . 1991 May. 24(5 Pt 2):839-44. . Erdag G, Qureshi HS, Greer KE

2014 eMedicine.com

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