Benign Familial Chronic Pemphigus

September 14, 2005
Jonathan S. Crane, DO

,
Ronald P. Benjamin, MD

,
Carla Dibenedetto, PA-C

,
Johnathan S. Crane, MD

,
Patricia Hood, PA-C

For 6 years, a 32-year-old man had a recurring rash on his back, shoulders, and chest. He stated that the rash appears in the spring, itches, and enlarges into ringlike areas. Previously, when treated with cephalexin, the rash had cleared within several weeks. Antifungal medication (econazole cream and oral terbinafine) had failed to resolve the rash.

For 6 years, a 32-year-old man had a recurring rash on his back, shoulders, and chest. He stated that the rash appears in the spring, itches, and enlarges into ringlike areas. Previously, when treated with cephalexin, the rash had cleared within several weeks. Antifungal medication (econazole cream and oral terbinafine) had failed to resolve the rash.

Biopsy samples of the patient's lesions revealed acantholysis. Hailey-Hailey disease-benign familial chronic pemphigus-was diagnosed.

Jonathan S. Crane, DO, Ronald P. Benjamin, MD, Carla DiBenedetto, PA-C, Patricia Hood, PA-C, and Kelly Mulligan, PA, of Wilmington, NC, write that Hailey-Hailey disease-initially described in 1939-is characterized by persistent blisters that typically involve the neck, axillae, and flexural surfaces. These eruptions may remain localized or become widespread. Frequently, one of the blisters ruptures and an erosion is seen followed by a thick crusted area, which resembles impetigo. The lesions often spread peripherally, develop an erythematous border around a central clearing, and produce circular patterns. They appear in crops for several weeks and then may go into remission.

Summertime sweating and sun exposure often aggravate the lesions. Bacterial infection, trauma, and fungal infections may trigger flares. Biopsy of the lesions is essential for diagnosis. Histopathologic findings demonstrate acantholysis, which on microscopic examination looks like a “dilapidated brick wall.&148;1

The treatment of Hailey-Hailey disease is difficult. Cephalexin and other antistaphylococcal antibiotics are often helpful in reducing the duration of the rash, in decreasing the size of the rash, and in increasing the duration between flares. Topical and oral corticosteroids may reduce symptoms. Cyclosporine, oral and topical retinoids, and dapsone, as well as dermabrasion and laser vaporization, have also been shown to be effective. Most patients respond to a course of topical corticosteroids and antibiotics, as was the case with this patient.

References:

REFERENCE:


1.

Odom RB, James WD, Berger TG, eds.

Andrew's Diseases of the Skin: Clinical Dermatology.

9th ed. Philadelphia: WB Saunders Co; 2000:699-701.

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