Russian Journal of Skin and Venereal DiseasesRussian Journal of Skin and Venereal Diseases1560-95882412-9097Eco-Vector3686510.17816/dv36865Research ArticleGougerot-Hailey-Hailey benign familial chronic pemphigus as an immunopathologyMakhnevaN. Vmakhneva@mail.ruDavidenkoE. B-ChernyshE. S-BeletskayaL. V-Moscow Research and Practical Center of Dermato-Venereology and CosmetologyM.F. Vladimirsky Moscow Regional Research and Clinical Institute15042014172323621072020Copyright © 2014, Eco-Vector2014Hailey-Hailey’s disease is a rare autosomal dominant hereditary vesiculobullous dermatosis, caused by mutations of ATP2C1 gene. However, the absence of familial liability in some cases and correlation between the defective gene mutation type and clinical manifestations suggests the involvement of some other pathogenetic factors. The authors studied the involvement of the immune system in the development of Hailey-Hai-ley’s disease. Poorly affine IgG autoantibodies and soluble immune complexes in the epidermal intercellular binding substance with involvement of the complement system were demonstrated by the immunofluorescent methods. As numerous mechanisms may be involved in the development of Hailey-Hailey’s disease, further studies of the problem should be carried out.Hailey-Hailey’s diseaseantibodiesimmune complexesimmunofluorescent methodsболезнь Хейли-Хейлиантителаиммунные комплексыметоды иммунофлюоресценции[Dobson-Stone C., Fairclough R., Dunne E., Brown J., Dissanayake M., Munro C.S., et al. Hailey-Hailey disease: molecular and clinical characterization of novel mutations in the ATP2C1 gene. J. Invest. Dermatol. 2002; 118(2): 338-43.][Sudbrak R., Brown J., Dobson-Stone C., Carter S., Ramser J., White J., et al. Hailey-Hailey disease is caused by mutations in ATP2C1 encoding a novel Ca2+ pump. Hum. Mol. Genet. 2000; 9(7): 1131-40.][Ikeda S., Shigihara T., Mayuzumi N., Yu X., Ogawa H. 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