Abstract

HALAC, S et al. Hailey-Hailey disease: report of two cases. Rev. argent. dermatol. [online]. 2015, vol.96, n.2, pp.92-102. ISSN 1851-300X.

Hailey-Hailey disease is an autosomal dominant disorder characterized by acantholysis. It is caused by mutations of ATP2C1 gene encoding the secretory pathway Ca++/Mn++ ATPase localized in Golgi apparatus. It usually presents as erythematous, erosive plaques with fissures at folds and sites of friction. The course is chronic, with relapses and remissions. Treatment options are neither specific nor totally effective for this disease. Topical corticosteroids in combination with antibiotics, systemic, surgical and physical therapy can be used. We report a female patient with self-limited disorder and another one with extensive disease, poor adherence and little response to treatment.

Keywords : Hailey-Hailey disease; Familial benign chronic pemphigus; Acantholytic disorder.