View of the clinic, diagnosis and treatment of familial benign pemphigus (Hailey — Hailey disease). Literature review

Abstract

The article describes modern views on predisposing factors, histological and genetic changes, the role of ATP2C1 encoding a mutant gene, localized on chromosome 3 in the pathogenesis of Hailey — Hailey disease. Diagnostic criteria, differential diagnostics with other diseases and methods of modern treatment of this disease are presented.

Keywords

• hailey — hailey disease
• encoding the mutant gene atp2c1
• adhesive properties of keratinocytes
• secretory path of the enzyme ca2+/ mn2+ atpase (hspca1)