Revisiting the question of pemphigus classification

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).

Analysis of clinical and diagnostic features and criteria for the effectiveness of treatment of autoimmune bullous dermatoses pemphigus group

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.