Abstract

ALEGRE-YATACO, PS; VELASCO-RODRIGUEZ, MF; SEGURA-PLASENCIA, NM  and  ALEGRE-GIRALDO, JY. Hailey-Hailey disease. Rev. argent. dermatol. [online]. 2016, vol.97, n.4, pp.90-98. ISSN 1851-300X.

Hailey-Hailey disease or familial benign chronic pemphigus, is a very rare genodermatosis, autosomal dominant inheritance, has no sex and race predilection. It is caused by mutations in the ATP2C1 gene, which causes dysfunction calcium channel, key in regulating epidermal differentiation and processing desmosomal proteins involved in cell adhesion, as a result there is a defect in adherence of keratinocytes and acantholysis occurs and generating alterations in the epidermis. It´s clinical manifestations are characterized by flaccid, pruritic and painful  ampules and vesicles located in intertriginous areas and rarely in mucosas; chronic and recurrent evolution. We report the unusual case of a male patient aged 86, presenting six months ago eroded and painful vesicles on the neck, armpits, groin and oral mucosa; previously treated with corticosteroids, unimproved, whereby lesions are biopsied, being histopathology compatible with Hailey-Hailey disease.

Keywords : Hailey-Hailey disease; familial benign chronic pemphigus; genodermatosis; ampules and vesicles; intertriginous.