Warycha M, Patel R, Meehan S, Merola JF
Familial benign chronic pemphigus (Hailey-Hailey disease). [Journal Article]
Dermatol Online J 2009; 15(8):15.
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.
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