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September 1977

Isolated Familial Benign Chronic Pemphigus

Author Affiliations

Evansville, Ind

Arch Dermatol. 1977;113(9):1302. doi:10.1001/archderm.1977.01640090150051

To the Editor.—  Familial benign chronic pemphigus (FBCP), or HaileyHailey disease, is a rare hereditary disorder that is characterized by recurrent vesicles and bullae in the intertriginous areas, such as the axillae and groin. The eruption is rarely confined to one area of the body.

Report of a Case.—  A 29-year-old woman was examined in November 1975 for pruritus vulvae of seven months' duration. Numerous small, flat, white papules and thickened lichenified skin were found on the vulvar surface. A skin biopsy specimen of a papule showed suprabasilar clefts, villi, and acantholytic epidermal cells characteristic of FBCP. The results of the rest of the skin examination were noncontributory. Fungal and yeast cultures grew no organisms, and pelvic and breast examination results were normal. There was no family history of recurrent bullous disease. The FBCP has not responded to steroid creams, antimonilial creams and suppositories, antifungal creams, steroid antibiotic combination cream,