CURRENT ISSUES IN ETIOPATHOGENESIS, CLINICAL MANIFESTATION AND DIAGNOSIS OF TRUE ACANTHOLYTIC PEMPHIGUS

The group of true acantholytic pemphigus is a large group of chronic autoimmune recurrent bullous dermatoses with common mechanisms of pathogenesis and various clinical manifestations. The etiological aspects of its occurrence remain controversial, but the autoimmune theory is generally accepted, explaining the formation of blisters on the mucous membranes and skin of patients due to the formation of autoimmune complexes in the interstitial substance of the stratum corneum of the epidermis and the subsequent development of acantholysis. Clinical signs are characterized by the development of dense, flaky blisters and multiple painful erosions, which without pathogenetic therapy cause discomfort, reduce the quality of life of patients and lead to death without pathogenetic therapy. The following clinical variants of pemphigus are distinguished: vulgaris, foliaceus, seborrheic, Brazilian and vegetative, as well as paraneoplastic and IgA pemphigus. The diagnosis is based on a combination of acantholysis and laboratory methods that reveal antibodies to desmogleins of types 1 and 3, as well as a number of clinical symptoms caused by the deposition of immune complexes in the left layer of the epidermis. Thus, true pemphigus is a group of similar clinical manifestations of bullous dermatoses of unknown etiology, characterized by the presence of blistering elements on the skin and mucous membranes, requiring a number of clinical and laboratory studies for diagnosis and differentiation with similar dermatoses. There are a number of diseases of an autoimmune and neoplastic nature associated with pemphigus, which represents a wide horizon for further research.