title: Familial benign chronic pemphigus (Hailey-Hailey disease).
abstract: A 57-year-old woman presented with a 27-year history of vesicles and crusted erosions of the intertriginous folds. Prior treatments had included topical glucocorticoids, mupirocin, and minocycline, all of which provided minimal relief. A skin biopsy specimen was consistent with Hailey-Hailey disease. Hailey-Hailey disease is the result of mutations in the ATP2C1 gene and is characterized by recurrent vesicles and erosions in intertriginous areas. Whereas topical and/or systemic antibiotic/antifungal agents in combination with topical glucocorticoids is the mainstay of treatment, case reports have documented dramatic improvement with oral retinoids, calcitriol, and tacrolimus.
authors.affiliation: Department of Dermatology, New York University, USA.
authors.forename: Melanie
authors.initials: M
authors.lastname: Warycha
authors.forename: Rishi
authors.initials: R
authors.lastname: Patel
authors.forename: Shane
authors.initials: S
authors.lastname: Meehan
authors.forename: Joseph F
authors.initials: JF
authors.lastname: Merola
mesh_terms: D005260:Female; D006801:Humans; D008875:Middle Aged; D016506:Pemphigus, Benign Familial / Q000473:pathology