Abstract
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.
Highlights
Hailey-Hailey disease of the vulva is a rare autosomal dominant dermatosis characterized by malformation of desmosomes and bullae, in intertriginous areas.Cases: We reviewed the cases of 5 women followed at a single academic institution over a period of 17 years
Treatment of Hailey-Hailey disease must be tailored to the individual patient
Hailey-Hailey disease (HHD) is a rare autosomal dominant intraepidermal blistering disorder first described by the Hailey brothers in 1939.1 Onset is in the Please cite this paper as: Whitis AM, Diane Elas D, Karla Solheim K, Stockdale CK
Summary
The cases presented detail the varied treatments of Hailey-Hailey disease in 5 different individuals (See Table 1). Complications of Hailey-Hailey Disease experienced by our patients included episodes of superimposed fungal and bacterial infections as well as lichen simplex chronicus flares These were treated with antifungals, topical clindamycin and metronidazole, oral doxycycline or minocycline, and steroidantifungal bursts. Another complication is the rare occurrence of squamous cell carcinoma in Hailey-Hailey affected skin,[17,18,19] which was not experienced by any of our 5 patients. Multiple case reports have shown complete remission expected to last 612 months with multiple doses of botulinum toxin type A.20,21 This treatment option is attractive because it decreases the use of systemic and topical corticosteroids and minimizes their respective side effect profiles. It was not feasible for our patients due to high cost and lack of insurance coverage
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