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Background: Hailey-Hailey disease (familial benign chronic pemphigus) of the vulva is a rare autosomal dominant dermatosis characterized by malformation of desmosomes and bullae, particularly in intertriginous areas.

Cases: We reviewed the cases of 5 women followed at a single academic institution over a period of 17 years. Of the 5 patients, 4 carried a diagnosis of Hailey-Hailey at the time of presentation to our institution, and one was diagnosed on biopsy. 3 were members of a family group. Initial treatment of choice was a topical steroid (betamethasone in 3, fluocinonide and hydrocortisone in 1 each), vulvar skin guidelines, tepid soaks, a skin protectant (A&D ointment, zinc oxide) and additional treatment (crotamiton in 1, clotrimazole in 2, and Polysporin in 1). Final treatment regimens in 3 patients followed long term were zinc oxide/A&D and betamethasone-clotrimazole ointment in all 3, nystatin-triamcinolone ointment in 2, oral minocycline in 1, oral fluconazole prn in 1, and topical tacrolimus in 1. Treatment courses were complicated by superimposed fungal infections, superimposed bacterial infections, and lichen simplex chronicus flares. These were treated with antifungals, topical clindamycin and metronidazole, and steroid-antifungal bursts, respectively. Three of five patients followed long term achieved successful remission with occasional flares.

Discussion: Treatment of Hailey-Hailey disease must be tailored to the individual patient. Adherence to vulvar skin care guidelines is critical for the remission of Hailey-Hailey disease. Treatment is long-term and may be complicated by episodes of fungal and bacterial superinfection and lichen simplex chronicus.


Hailey-Hailey Disease, familial benign chronic pemphigus, vulva

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