Hailey-Hailey disease: A fold (intertriginous) dermatosis.

Abstract:

:Hailey-Hailey disease, also called benign familial pemphigus, is a late-onset blistering disorder that affects the flexures. There are typically painful erosions and cracks in affected areas. Lesions generally begin between 20 and 40 years of age. In two third of all cases, positive family history is detected. In pathogenesis, there is a defect in keratinocyte adhesion due to ATP2 C1 gene mutation. The result of the desmosomal decomposition is acantholysis. Menstruation, pregnancy, skin infections, physical trauma, excessive sweating and exposure to ultraviolet radiation are important triggering factors. Histopathologic changes are suprabasal acantholysis and formation of intraepidermal bullae. In the epidermis, a partial acantholysis that looks like broken bricks is observed.

journal_name

Clin Dermatol

journal_title

Clinics in dermatology

authors

Engin B,Kutlubay Z,Çelik U,Serdaroğlu S,Tüzün Y

doi

10.1016/j.clindermatol.2015.04.006

subject

Has Abstract

pub_date

2015-07-01 00:00:00

pages

452-5

issue

4

eissn

0738-081X

issn

1879-1131

pii

S0738-081X(15)00070-X

journal_volume

33

pub_type

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