Familial benign chronic pemphigus was initially described in 19391 in two brothers with a recurrent pruritic blistering eruption of the neck. Distinctive histopathologic features combining aspects of both pemphigus and Darier's disease were found. As cases were identified, it was noted to affect other sites, including the perineum, groin, and intertriginous areas, and began to be called Hailey-Hailey disease (HH). Isolated areas of involvement with this dominantly inherited disorder may pose a diagnostic dilemma, particularly when presenting with vulvar lesions, as clinical findings may simulate other disorders. We describe three patients with HH who presented with erosive vulvar lesions and review this uncommon entity.
Report of Cases
A 36-year-old nurse was referred with a 15-month history of vaginal and vulvar pain, ascribed to candidiasis. The biopsy specimen disclosed persistent erosive lesions demonstrative of acantholysis. Indirect immunofluorescence was negative. Treatments included topical steroids, vitamin A (Aquasol A), and
Wieselthier JS, Pincus SH. Hailey-Hailey Disease of the Vulva. Arch Dermatol. 1993;129(10):1344-1345. doi:10.1001/archderm.1993.01680310116027