Clinic, diagnostics and treatment of epidermolysis bullosa

Drozhdina M.B., Koshkin S.V., Zakhur I.I., Bobro V.A.

Federal State Budgetary Educational Institution of Higher Education "Kirov State Medical University" of the Ministry of
Healthcare of the Russian Federation
Kirov regional state budgetary institution of health care “Kirov regional clinical dermatovenerologic hospital”

The article presents updated information on the study of the most severe bullous dermatosis - epidermolysis bullosa, and mutations underlying this disease. The most sensitive and specific methods of diagnosis, pathways of disease transmission, and the main studied mutation genes are described. The article describes the main types of treatment for epidermolysis bullosa that are used in the world: protein, gene and cell therapy, as well as experimental models for the treatment of this disease. The article describes a clinical case of continuously recurring acquired epidermolysis bullosa, combined with severe diseases: thyroid cancer and chronic lymphocytic leukemia. The patient was examined for HLA antigens. Histocompatibility antigens of class I were identified: A3, 9(24) and B21(49), 35 of class II: DRB1* 11,13.
A hypothesis was put forward about the mutual influence of genes that cause these diseases on the severity and severity of epidermolysis bullosa and other pathological processes in the described patient.