Familial benign chronic pemphigus (Hailey-Hailey disease).
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.
Warycha
Melanie
M
Department of Dermatology, New York University, USA.
Patel
Rishi
R
Meehan
Shane
S
Merola
Joseph F
JF
D005260:Female; D006801:Humans; D008875:Middle Aged; D016506:Pemphigus, Benign Familial / Q000473:pathology