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Pemphigus Foliaceus

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101. Absence of Intercellular Antigens in the Deep Layers of the Epidermis in Pemphigus Foliaceus Full Text available with Trip Pro

Absence of Intercellular Antigens in the Deep Layers of the Epidermis in Pemphigus Foliaceus 12 patients with pemphigus foliaceus, a form of pemphigus with lesions that arise in the intercellular substance in the superficial layers of the epidermis, and 7 patients with pemphigus vulgaris, where lesions are in the deep layers, were studied by immunofluorescence. Circulating antibodies to intercellular antigens (IC antibodies) were found in 11 pemphigus foliaceus and 5 pemphigus vulgaris patients (...) . On direct immunofluorescence of skin lesions 75% (9 of 12), pemphigus foliaceus patients had intercellular deposits of IgG localized solely or predominantly in the superficial epidermal layers, whereas this was not the case in any of the patients with pemphigus vulgaris. Over 70% of the pemphigus foliaceus patients with predominantly superficial IgG deposits lacked in their lesions normal intercellular antigens usually expressed in the deep layers of the epidermis. This was shown by the inability of IC

1978 Journal of Clinical Investigation

102. Generalized Vaccinia in Patients with Pemphigus Foliaceus Full Text available with Trip Pro

Generalized Vaccinia in Patients with Pemphigus Foliaceus 13991124 1998 11 01 2018 12 01 0032-5473 39 1963 Feb Postgraduate medical journal Postgrad Med J Generalized vaccinia in patients with pemphigus foliaceus. 86-90 de SALLES-GOMES L L RABELLO S I SI MAGALDI-JORDAO F B FB PEDERNEIRAS C A CA AMOROSINO A A ANGULO J J JJ eng Journal Article England Postgrad Med J 0234135 0032-5473 OM Humans Pemphigus Vaccinia PEMPHIGUS VACCINIA 1963 2 1 1963 2 1 0 1 1963 2 1 0 0 ppublish 13991124 PMC2482011 Br

1963 Postgraduate medical journal

103. Pemphigus Foliaceus Complicated by Peripheral Vascular Disease Full Text available with Trip Pro

Pemphigus Foliaceus Complicated by Peripheral Vascular Disease 19994045 2010 06 25 2018 11 13 0035-9157 53 4 1960 Apr Proceedings of the Royal Society of Medicine Proc. R. Soc. Med. Pemphigus Foliaceus Complicated by Peripheral Vascular Disease. 298 Garretts M M eng Journal Article England Proc R Soc Med 7505890 0035-9157 2009 12 9 6 0 1960 4 1 0 0 1960 4 1 0 1 ppublish 19994045 PMC1871202 Br J Dermatol. 1959 Jun;71(6):223-5 13662523 Am J Med. 1950 Dec;9(6):752-6 14789795

1960 Proceedings of the Royal Society of Medicine

104. A Case of Pemphigus Foliaceus Full Text available with Trip Pro

A Case of Pemphigus Foliaceus 20754866 2011 03 29 2011 03 29 0007-1447 1 1745 1894 Jun 09 British medical journal Br Med J A Case of Pemphigus Foliaceus. 1237-8 Cuthbert C F CF eng Journal Article England Br Med J 0372673 0007-1447 2010 8 27 6 0 1894 6 9 0 0 1894 6 9 0 1 ppublish 20754866 PMC2405252

1894 British medical journal

105. Pemphigus Foliaceus of Sixteen Years' Duration Full Text available with Trip Pro

Pemphigus Foliaceus of Sixteen Years' Duration 19974977 2010 06 24 2010 06 24 0035-9157 4 Dermatol Sect 1911 Proceedings of the Royal Society of Medicine Proc. R. Soc. Med. Pemphigus Foliaceus of Sixteen Years' Duration. 15-7 Adamson H G HG eng Journal Article England Proc R Soc Med 7505890 0035-9157 2009 12 9 6 0 1911 1 1 0 0 1911 1 1 0 1 ppublish 19974977 PMC2004924

1911 Proceedings of the Royal Society of Medicine

106. Pemphigus

, Poland; 13 L'Istituto Dermopatico dell'Immacolata, Rome, Italy; 14 University of Lübeck, Germany; 15 University of Groningen, The Netherlands 2 CONTENTS 1 INTRODUCTION 4 1.1 METHODOLOGY OF GUIDELINE PREPARATION 5 2 INITIAL EVALUATION OF PEMPHIGUS 6 2.1 MAJOR OBJECTIVES 6 2.2 PROFESSIONS INVOLVED 6 2.3 CLINICAL EXAMINATION 7 2.3.1 Medical History 7 2.3.2 Physical Examination 7 2.3.2.1 General 7 2.3.2.2 Pemphigus vulgaris (PV) 8 2.3.2.3 Pemphigus foliaceus (PF) 8 2.3.2.4 Paraneoplastic pemphigus (PNP (...) and/or desmoglein 1, on epidermal keratinocytes 4 . Pemphigus is rare and its incidence has been estimated to about 2 new patients per 1 million inhabitants per year in Central Europe. Two main clinical variants are known, pemphigus vulgaris (PV) and pemphigus foliaceus (PF). The pathogenic role of anti-desmoglein 1/3 IgG has been clearly established since the injection of patients’ sera or affinity-purified IgG from pemphigus sera into neonatal mice reproduces immune pathologically and clinically the cardinal

2013 European Dermatology Forum

107. Calculation of cut-off values based on the ABSIS and PDAI pemphigus scoring systems for defining moderate, significant and extensive types of pemphigus. (Abstract)

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 (...) Calculation of cut-off values based on the ABSIS and PDAI pemphigus scoring systems for defining moderate, significant and extensive types of pemphigus. 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

2016 British Journal of Dermatology

108. A Study to Evaluate the Safety, PD, PK and Efficacy of ARGX-113 in Patients With Pemphigus

: November 7, 2017 Last Update Posted : March 5, 2019 See Sponsor: argenx BVBA Information provided by (Responsible Party): argenx BVBA Study Details Study Description Go to Brief Summary: The proposed study is an open-label, non-controlled, adaptive-design Phase II study to evaluate the safety, pharmacodynamics, pharmacokinetics, efficacy, and conditions of use (dosage, frequency of administration at maintenance) of ARGX-113 in patients with mild to moderate Pemphigus (Vulgaris or Foliaceus), either (...) newly diagnosed or relapsing. The total study duration for each patient is less than 6 months. It consists of a Screening period, an Induction, a maintenance treatment period followed by a treatment-free Follow-up (FU) period. Condition or disease Intervention/treatment Phase Pemphigus Vulgaris Pemphigus Foliaceus Drug: ARGX-113 Phase 2 Study Design Go to Layout table for study information Study Type : Interventional (Clinical Trial) Estimated Enrollment : 12 participants Intervention Model: Single

2017 Clinical Trials

109. Relationship between pemphigus and American tegumentary leishmaniasis: insights from serological and genetic profiles. (Abstract)

Relationship between pemphigus and American tegumentary leishmaniasis: insights from serological and genetic profiles. Antibodies against Leishmania peptides (Lbr-peps) and desmogleins (Dsgs) have been reported in pemphigus foliaceus (PF) and leishmaniasis patients, respectively. We aimed to compare serological and genetic features in a Brazilian region endemic for American tegumentary leishmaniasis (ATL) and pemphigus.Commercial anti-Dsg ELISA and in-house ELISA with Lbr-peps were used (...) to determine the serological profile, in addition to immunoblotting (IB) and indirect immunofluorescence (IIF) assays. HLA-DRB1 and -DQA1/DQB1 alleles were characterized by PCR combined with sequence-specific oligonucleotide probes (PCR-SSOP). The serological and genetic profiles were compared using 78 PF, 62 pemphigus vulgaris (PV) and 58 ATL patients against 163 and 1592 healthy controls, respectively.Some ATL patients showed positive results for anti-Dsg1 and/or anti-Dsg3 antibodies. They also revealed

2017 Transactions of the Royal Society of Tropical Medicine & Hygiene

110. Autoantibodies to full body vascular cell junctions colocalize with MYZAP, ARVCF, desmoplakins I and II and p0071 in endemic pemphigus in Colombia, South America. (Abstract)

Autoantibodies to full body vascular cell junctions colocalize with MYZAP, ARVCF, desmoplakins I and II and p0071 in endemic pemphigus in Colombia, South America. We previously described a new variant of endemic pemphigus foliaceus in El Bagre, Colombia (El Bagre-EPF).Here we aimed to investigate disease autoreactivity to vessels in all body organs/systems. We compared 57 patients and 57 controls from the endemic area, matched by demographics, age, sex, and work activity. We performed

2017 International Journal of Dermatology

111. Cardiac Rhythm and Pacemaking Abnormalities in Patients Affected by Endemic Pemphigus in Colombia May be a Result of Deposition of Autoantibodies, Complement, Fibrinogen and Other Molecules. (Abstract)

Cardiac Rhythm and Pacemaking Abnormalities in Patients Affected by Endemic Pemphigus in Colombia May be a Result of Deposition of Autoantibodies, Complement, Fibrinogen and Other Molecules. We previously showed that one-third of patients affected by endemic pemphigus foliaceus in El Bagre, Colombia (El Bagre-EPF), display autoreactivity to the heart.The purpose of this study was to investigate rhythm disturbances with the presence of autoantibodies and correlate them with ECG changes

2017 Heart Rhythm

112. A novel lateral flow immunoassay for the rapid detection of anti-Dsg3 IgG serum autoantibodies in pemphigus vulgaris. (Abstract)

detects anti-desmoglein 3 (Dsg3) IgG in human serum. In contrast to other diagnostic procedures, the assay is compact and simple to perform and delivers a fast "yes" or "no" answer within 10 minutes without additional hardware requirements for test evaluation. For validation, a blinded collection of 200 sera including 100 sera from 14 PV patients, 75 sera from 24 bullous pemphigoid patients and 25 sera from 6 patients with pemphigus foliaceus collected at different time points during disease was used (...) A novel lateral flow immunoassay for the rapid detection of anti-Dsg3 IgG serum autoantibodies in pemphigus vulgaris. Pemphigus vulgaris (PV) is a severe autoimmune blistering disease of the skin and mucous membranes. As autoantibodies play an essential role in the disease pathogenesis, the serological detection of anti-desmoglein 3 IgG represents a central tool in the diagnosis of the disease. In this study, we show the validation of a novel lateral flow immunoassay (LFIA) which rapidly

2017 Experimental Dermatology

113. Spatial Distribution of Pemphigus Occurrence over Five Decades in Southeastern Brazil. Full Text available with Trip Pro

Spatial Distribution of Pemphigus Occurrence over Five Decades in Southeastern Brazil. Well-defined locations of pemphigus cases support the hypothesis of environmental factors' involvement in its etiopathogenesis; however, these foci have never been described using specialized geographical tools. This is the first report to geo-reference pemphigus cases in a high-prevalence Brazilian region using geographic information systems. We aimed to report the spatio-temporal behavior of pemphigus (...) foliaceus (PF) and vulgaris (PV) in southeastern Brazil, over the last five decades to describe geographical clusters, as well as to characterize the land use in the city with the highest number of cases. Patients were identified from 1965 to 2014. Maps were developed using ArcGIS software and organized into decades from 1965 to 2014. Ribeirão Preto was identified as the city with the greatest number of cases. Land use was analyzed within a 2 km-buffer surrounding the residence of each patient. A total

2017 American Journal of Tropical Medicine & Hygiene

114. Routine detection of serum antidesmocollin autoantibodies is only useful in patients with atypical pemphigus. Full Text available with Trip Pro

against desmogleins 1 and 3 (n = 24) and sera from patients with autoimmune blistering diseases with positive direct IF microscopy taken at the time of diagnosis (n = 749). We found that detection of anti-Dsc serum reactivity is not helpful in the routine diagnosis of PV, pemphigus foliaceus and paraneoplastic pemphigus but may be valuable in pemphigus vegetans.© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. (...) Routine detection of serum antidesmocollin autoantibodies is only useful in patients with atypical pemphigus. Autoantibodies against the 3 desmocollin (Dsc; Dsc1-Dsc3) isoforms have been described in different pemphigus variants. Here, we developed state-of-the-art detection systems for serum anti-Dsc1, Dsc2 and Dsc1 IgG and IgA. These assays were applied in 5 different cohorts including pemphigus vulgaris (PV) patients with compatible direct immunofluorescence (IF) microscopy but no reactivity

2017 Experimental Dermatology

115. Scalp involvement in pemphigus: a prognostic marker Full Text available with Trip Pro

Scalp involvement in pemphigus: a prognostic marker Scalp involvement in the course of pemphigus is observed in 16-60% of patients.To determine the prognostic significance of scalp involvement in pemphigus vulgaris and pemphigus foliaceus.A total of 75 patients (46 with pemphigus vulgaris, 29 with pemphigus foliaceus) were included into this prospective study. The following clinical data were analyzed: Pemphigus Disease Area Index, time to complete clinical remission and duration of complete (...) clinical remission. Indirect immunofluorescence and enzyme-linked immunosorbent assay were performed to monitor serum pemphigus antibodies.Scalp involvement was observed in 30/46 (65.2%) patients with pemphigus vulgaris and 28/29 (96.6%) patients with pemphigus foliaceus. A positive correlation was found between scalp involvement and general disease severity as measured by the Pemphigus Disease Area Index (r = 0.7, p < 0.05). The time required to achieve a complete clinical remission in patients

2017 Advances in Dermatology and Allergology/Postȩpy Dermatologii i Alergologii

116. Pemphigus Full Text available with Trip Pro

Pemphigus Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion) causes blisters and erosions. Pemphigus has three major subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are characteristically raised against desmoglein 1 and desmoglein 3, which are cell-cell adhesion molecules found in desmosomes. The sites of blister formation (...) can be physiologically explained by the anti-desmoglein autoantibody profile and tissue-specific expression pattern of desmoglein isoforms. The pathophysiological roles of T cells and B cells have been characterized in mouse models of pemphigus and patients, revealing insights into the mechanisms of autoimmunity. Diagnosis is based on clinical manifestations and confirmed with histological and immunochemical testing. The current first-line treatment is systemic corticosteroids and adjuvant

2017 Nature reviews. Disease primers

117. Different signaling patterns contribute to loss of keratinocyte cohesion dependent on autoantibody profile in pemphigus Full Text available with Trip Pro

Different signaling patterns contribute to loss of keratinocyte cohesion dependent on autoantibody profile in pemphigus Pemphigus is an autoimmune blistering skin disease caused primarily by autoantibodies against desmoglein (Dsg)1 and 3. Here, we characterized the mechanisms engaged by pemphigus IgG from patients with different clinical phenotypes and autoantibody profiles. All pemphigus vulgaris (PV) and pemphigus foliaceus (PF) IgG and AK23, a monoclonal mouse antibody against Dsg3, caused (...) loss of cell cohesion, cytokeratin retraction and p38MAPK activation. Strong alterations in Dsg3 distribution were caused by mucosal (aDsg3 antibodies), mucocutaneous (aDsg1 + aDsg3) as well as atypical (aDsg3) PV-IgG. All PV-IgG fractions and AK23 compromised Dsg3 but not Dsg1 binding and enhanced Src activity. In contrast, rapid Ca2+ influx and Erk activation were induced by mucocutaneous PV-IgG and pemphigus foliaceus (PF) IgG (aDsg1) whereas cAMP was increased by mucosal and mucocutaneous PV

2017 Scientific reports

118. Serum IL-21 Levels in Patients With Pemphigus Vulgaris

by (Responsible Party): Marwa Kassim, Assiut University Study Details Study Description Go to Brief Summary: Pemphigus is severe antigen derived autoimmune bullous skin disorder, the word pemphigus is derived from the Greek word" pemphix " which means blister . Two main clinical variants are known pemphigus vulgaris (PV), and pemphigu foliaceus (PF). (Zenzo .et al., 2015). Condition or disease Pemphigus Vulgaris Detailed Description: Clinically, there are localized or generalized flaccid bullae that quickly (...) Serum IL-21 Levels in Patients With Pemphigus Vulgaris Serum IL-21 Levels in Patients With Pemphigus Vulgaris - 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. Serum IL-21 Levels in Patients With Pemphigus

2017 Clinical Trials

119. Polyclonal Regulatory T Cells (PolyTregs) for Pemphigus

in adult participants with active pemphigus.The purpose of this study is to test the safety and effect of Treg therapy in participants who have skin (cutaneous) involvement due to pemphigus. Condition or disease Intervention/treatment Phase Pemphigus Foliaceus Pemphigus Vulgaris Biological: Cohort 1: 2.5 x 10^8 PolyTregs Biological: Cohort 2: 10x10^8 PolyTregs Phase 1 Detailed Description: Up to 12 adults between the ages of 18 and 75 years of age who have been diagnosed with pemphigus and meet all (...) of Pemphigus Vulgaris (PV) or Pemphigus Foliaceus (PF), defined by H&E staining (e.g., Haemotoxylin and Eosin) and direct immunofluorescence staining of skin biopsy at any time prior to enrollment; Pemphigus treated with systemic corticosteroids within the 2 years prior to screening (historic or current); Presence of: anti-Dsg3 antibodies (>20.0 U/ml) at screening visit consistent with diagnosis of pemphigus vulgaris or, anti-Dsg1 antibodies (>20.0U/ml) at screening visit consistent with diagnosis

2017 Clinical Trials

120. Pemphigus, a pathomechanism of acantholysis. (Abstract)

Pemphigus, a pathomechanism of acantholysis. Autoantibodies to the desmosomal proteins desmoglein 1 and 3 cause pemphigus foliaceus and pemphigus vulgaris, which are characterised by keratinocyte dissociation (acantholysis) and intraepidermal blister formation. The passive transfer of pathogenic anti-desmoglein antibodies induces blisters in mice in vivo and the loss of keratinocyte adhesion in vitro. The pathogenetic mechanisms of acantholysis due to anti-desmoglein autoantibodies

2017 Australasian Journal of Dermatology

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