Author: Dr. Abdurrahman Almurayshid, faculty member at Prince Sattam Bin Abdulaziz University, College of Medicine, Dermatology, Saudi Arabia. June 2015.
Hailey–Hailey disease, also called familial benignchronic pemphigus, is a blistering disease caused by mutations in the ATP2C1 gene, with autosomal-dominant inheritance. The ATP2C1 gene is vital for keratinocyte adhesion and differentiation. Usually the clinical presentation starts during the second or the third decade. The main affected areas are the skin folds, with moist fissured malodorous plaques and blisters. The course of disease is variable, and treatment is mainly symptomatic.
Histology of Hailey Hailey disease
In Hailey Hailey disease, acantholysis affects the whole epidermis, giving the classic description of the dilapidated brick wall (figures 1–4). The hair follicles are usually spared, typically, with acanthosis and dyskeratosis.