Gougerot-Hailey-Hailey benign familial chronic pemphigus as an immunopathology


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Abstract

Hailey-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.

About the authors

N. V Makhneva

Moscow Research and Practical Center of Dermato-Venereology and Cosmetology; M.F. Vladimirsky Moscow Regional Research and Clinical Institute

Email: makhneva@mail.ru
доктор мед. наук, профессор; Лаборатория фундаментальных методов исследования; кафедра дерматовенерологии и дерматоонкологии факультета усовершенствования врачей

E. B Davidenko

Moscow Research and Practical Center of Dermato-Venereology and Cosmetology

научный сотрудник; Лаборатория фундаментальных методов исследования

E. S Chernysh

Moscow Research and Practical Center of Dermato-Venereology and Cosmetology; M.F. Vladimirsky Moscow Regional Research and Clinical Institute

аспирант, лаборант-исследователь; Лаборатория фундаментальных методов исследования; кафедра дерматовенерологии и дерматоонкологии факультета усовершенствования врачей

L. V Beletskaya

Moscow Research and Practical Center of Dermato-Venereology and Cosmetology

доктор мед. наук, профессор; Лаборатория фундаментальных методов исследования

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