Hailey-Hailey's disease: Epidemiology and clinical pattern


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Abstract

Hailey-Hailey’s disease is a hereditary vesiculo-bullous dermatosis with autosomal dominant inheritance. The disease is prevalent everywhere, the patients are men and women aged mainly 20-40 years. However, it can also be seen in children and elderly individuals. This paper presents data on the epidemiology, etiology, clinical picture, and association of this genetic dermatosis with other diseases. The role of histocompatibility complex antigens, mutations in the ATP2C1 gene locus, and factors in triggering the disease development is discussed. Clinical manifestations of Hailey-Hailey’s disease vary greatly, which impedes its diagnosis. The data on relationship between this genetic dermatosis and visceral involvement and/or development as a paraneoplastic process suggest the existence of an extracutaneous pathological phenotype.

About the authors

Natalia V. Makhneva

M.F. Vladimirsky Moscow Regional Research and Clinical Institute

Email: makhneva@mail.ru
MD, PhD, DSc, prof. 129110, Moscow, Russia

E. S Chernysh

M.F. Vladimirsky Moscow Regional Research and Clinical Institute

аспирант 129110, Moscow, Russia

L. V Beletskaya

M.F. Vladimirsky Moscow Regional Research and Clinical Institute

доктор мед. наук, профессор 129110, Moscow, Russia

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