scholarly journals The pemphigus group – diseases and their treatment possibilities

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

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).


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 ◽  
...  

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

2004 ◽  
Vol 11 (4) ◽  
pp. 762-765 ◽  
Author(s):  
G. D'Agosto ◽  
A. Latini ◽  
M. Carducci ◽  
A. Mastroianni ◽  
A. Vento ◽  
...  

ABSTRACT The diagnosis of autoimmune bullous diseases is based on clinical observation and on the presence of autoantibodies directed to molecules involved in the adhesion systems of the skin. Immunofluorescence assays are the currently accepted method for detection of autoantibodies; such assays depend greatly on the skill of operators and are difficult to standardize. Recombinant desmoglein-1 (Dsg1), Dsg3, and BP180 peptides, the main autoantigens in pemphigus or bullous pemphigoid, have been used to develop new quantitative enzyme immunoassays (EIA) for the detection of specific antibodies. The present study was undertaken to evaluate the sensitivity and specificity of these immunoassays and to determine the correlation between the results and the clinical aspects of diseases. Serum samples from patients with pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, or mucous membrane pemphigoid, from healthy individuals, and from patients with unrelated autoimmune conditions were tested. Anti-desmoglein reactivity was detected in all the patients with pemphigus and in none of the controls. Patients with the more benign form of cutaneous disease had anti-Dsg1 antibodies, while patients with deeper cutaneous lesions or with mucosal involvement had anti-Dsg3 reactivity also, or exclusively. The BP180-based assay was positive for 66.6% of patients with bullous pemphigoid and for none of the patients with mucous membrane pemphigoid, and no reactivity was detected in the control sera. In conclusion, the anti-Dsg1 and anti-Dsg3 assays are useful in the diagnosis of pemphigus and provide information on the clinical phenotype of the disease. However, the sensitivity of EIA for detection of autoantibodies in bullous pemphigoid should be improved by the use of additional antigens or epitopes.


2001 ◽  
Vol 144 (2) ◽  
pp. 421-422 ◽  
Author(s):  
K. Ogata ◽  
H. Nakajima ◽  
M. Ikeda ◽  
Y. Yamamoto ◽  
M. Amagai ◽  
...  

2009 ◽  
Vol 53 (3) ◽  
pp. 228-231 ◽  
Author(s):  
Masataka Arakawa ◽  
Teruki Dainichi ◽  
Shinichiro Yasumoto ◽  
Takashi Hashimoto

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