MODERN CLINICAL FEATURES OF AUTOIMMUNE BULLOUS DERMATOSES

Abstract

Autoimmune bullous dermatoses are a group of acquired and inherited diseases caused by the production of autoantibodies directed against protein structures of the epidermis and dermo-epidermal junction. The most severe and potentially dangerous bullous dermatoses are acantholytic pemphigus and bullous pemphigoid. Mortality from acantholytic pemphigus is 10.0 - 30.0 %. Aim. To demonstrate the diversity of clinical manifestations and the need for multidisciplinary interaction in the management of patients with autoimmune bullous dermatoses. Materials and methods. The literature review of materials of domestic and foreign researchers describe the clinical course of autoimmune bullous dermatoses using the search engines Pubmed, Medline, Cochrane library, Elibrary (total 73). The clinical course of bladder dermatosis varies from localized forms with a relatively mild degree of severity to generalized lethal forms that are characterized by the formation of bullas that open with the formation of long-term non-healing erosions that can occur both on the skin and on the mucous membranes of the eyes, nose, mouth, esophagus, genitalia. The article describes the most significant complaints from patients with lesions on mucous membranes, the description of the endoscopic picture of lesions in the gastrointestinal tract in patients with autoimmune bullous dermatoses, the description of the classical clinical picture of pemphigus acantholyticus, represented by blisters with serous contents, with listless, flabby cover and erosions prone to peripheral growth, a description of the clinical symptoms of Nikolsky, Asbo-Khansen and Sheklov, most significant for the differential diagnosis of bullous dermatoses. Furthermore authors describe cases with non typical clinical findings autoimmune bullous dermatoses and unusual site of the pathologic process. That can cause diagnostic errors leading the process to spread, postponement of the start of treatment, which in turn requires the appointment of high doses of systemic glucocorticosteroids. Improving the prognosis and quality of life of patients is possible only with the interdisciplinary interaction of a dermatovenereologist with adjacent specialists