scholarly journals Revisiting the question of pemphigus classification

2021 ◽  
Vol 97 (2) ◽  
pp. 9-15
Author(s):  
Alexey V. Samtsov ◽  
Evgeny V. Sokolovskiy ◽  
Natalia P. Teplyuk ◽  
Irena E. Belousova ◽  
Muza M. Kokhan ◽  
...  

Analysis of various classifications of pemphigus shows that there are no fundamental differences between them. The main distinctions consist in use of diverse terms in naming of some forms of pemphigus and in inclusion or exclusion of certain subtypes from the classifications. Authors propose to use the following classification in the dermatological clinical practice, for educational and scientific purposes and for clinical guidelines: 1) pemphigus vulgaris (1.1. Pemphigu s vegetans); 2) pemphigus foliaceus (2.1. Pemphigus endemic (Fogo selvagem), 2.2. Pemphigus erythematosus (Senear Usher)); 3) herpetiform pemphigus; 4) paraneoplastic pemphigus; 5) IgA pemphigus (5.1. Subcorneal pustular dermatosis, 5.2. Intraepidermal neutrophilic dermatosis).

2021 ◽  
Vol 97 (4) ◽  
pp. 1741-182
Author(s):  
Lilla Soltész ◽  
◽  
Andrea Szegedi ◽  
Krisztián Gáspár

The diseases of the pemphigus group are chronic conditions belonging to the autoimmune blistering disorders. They appear with characteristic skin and mucous membrane symptoms. Their major forms are pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. The aim of this review is to present the diseases of the pemphigus group and to summarize the latest therapeutic options with the exception of paraneoplastic and IgA pemphigus that are discussed separately


2007 ◽  
Vol 156 (4) ◽  
pp. 635-641 ◽  
Author(s):  
L.F. Mentink ◽  
M.C.J.M. de Jong ◽  
G.J. Kloosterhuis ◽  
J. Zuiderveen ◽  
M.F. Jonkman ◽  
...  

Author(s):  
М.Е. Zapolsky ◽  
М.М. Lebediuk ◽  
N.B. Prokofyeva ◽  
V.V. Goncharenko ◽  
S.V. Nerubashchenko

The modern classification of autoimmune bullous dermatoses is represented by three groups of diseases that have not only clinical, but also histomorphological, histochemical and immunogenetic similarities. Autoimmune bullous dermatoses are a group of heterogeneous diseases accompanied by the formation of blisters and erosions on the skin and/or mucous membranes under the influence of specific autoimmune complexes. The main clinical manifestations and diagnostic markers of autoimmune bullous dermatoses (ABD) belonging to the pemphigus group are considered.Objective — to analyze the prevalence of clinical forms of pemphigus in the southern region of Ukraine for the period from 2010 to 2020.Materials and methods. The observation group consisted of 88 patients aged 21 to 75 years. The features of clinical manifestations of typical and atypical forms of pemphigus have been studied, a diagnostic algorithm and criteria for the effectiveness of treatment in the early stages of the disease have been developed.Results and discussion. Pemphigus vulgaris was found in 61 (69.3 %), pemphigus vegetans — in 7 (7.9 %), pemphigus erythematosus — in 6 (6.8 %), herpetiform pemphigus — in 4 (4.5 %), IgA pemphigus — in 3 (3.4 %), paraneoplastic pemphigus — in 3 (3.4 %) patients.Conclusions. Modern clinical diagnostic features of ABD (pemphigus group) require the inclusion of immunofluorescence microscopy, direct and indirect enzyme immunoassay in laboratory screening. Not only positive clinical dynamics, but also the rate of disappearance of autoantibodies that induce the diseases (desmoglein, desmoplakin, periplakin, etc.) should be considered the criteria of the effectiveness of treatment of patients with ABD. At the same time, knowledge of the clinical features of ABD and the diseases that mimic them remains an important aspect.


2016 ◽  
Vol 43 (3) ◽  
pp. 138-140
Author(s):  
Lubna Khondker ◽  
Md Sirajul Islam Khan ◽  
Abida Sultana ◽  
Md Saiful Islam Bhuiyan

This cross-sectional study was done to evaluate the pattern of patients suffering from pemphigus disease attending in the department of Dermatology and Venereology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh from 2006 to 2010. Thirty patients of pemphigus diagnosed clinically and confirmed by histopathology and immunology, were included in the study. Among the thirty patients, 18(60%) had pemphigus vulgaris. Among the patients of pemphigus foliaceus, 13.33% patients were in the age group of 30-45 years, among the patients of pemphigus erythematosus, 6.67% patients were in the age group of 30-45 years and among the patients of paraneoplastic pemphigus, 3.33% patients were in the age group of 46-60 years. Among the different varieties of pemphigus, highest 66.67% had the disease duration of 1 to 6 months. Male were more than female. Highest 76.50 % showed both mucosal and skin involvement as initial clinical presentation. In future, this study should be carried out on a large scale with prolong period, large sample size and in different hospitals in Bangladesh.Bangladesh Med J. 2014 Sep; 43 (3): 138-140


Medicina ◽  
2021 ◽  
Vol 57 (10) ◽  
pp. 1080
Author(s):  
Ali M. Malik ◽  
Sarah Tupchong ◽  
Simo Huang ◽  
Abhirup Are ◽  
Sylvia Hsu ◽  
...  

Clinicians may encounter a variety of skin conditions that present with vesiculobullous lesions in their everyday practice. Pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus represent the spectrum of autoimmune bullous dermatoses of the pemphigus family. The pemphigus family of diseases is characterized by significant morbidity and mortality. Considering the risks associated with a delayed diagnosis or misdiagnosis and the potential for overlap in clinical features and treatment, evaluation for suspected pemphigus disease often requires thorough clinical assessment and laboratory testing. Diagnosis is focused on individual biopsies for histopathology and direct immunofluorescence. Additional laboratory methods used for diagnosis include indirect immunofluorescence and enzyme-linked immunosorbent assay. Recent advancements, including anti-CD20 therapy, have improved the efficacy and reduced the morbidity of pemphigus treatment. This contribution presents updates on the pathophysiology, clinical features, diagnostic work-up, and medical management of pemphigus. Improved strategies for diagnosis and clinical assessment are reviewed, and newer treatment options are discussed.


1993 ◽  
Vol 11 (1) ◽  
pp. 113-117 ◽  
Author(s):  
Diya F. Mutasim ◽  
Nancy J. Pelc ◽  
Grant J. Anhalt

Cureus ◽  
2021 ◽  
Author(s):  
Natalie Stumpf ◽  
Simo Huang ◽  
Lawrence D Hall ◽  
Sylvia Hsu

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