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Images in Dermatology
January 11, 2023

Benign Familial Pemphigus in Skin of Color

Author Affiliations
  • 1University of Florida College of Medicine, Gainesville
  • 2Department of Dermatology, University of Florida College of Medicine, Gainesville
JAMA Dermatol. 2023;159(3):331. doi:10.1001/jamadermatol.2022.5117

An African American man in his 40s presented with a 10-year history of pruritic intertriginous eruption. The patient had no improvement with treatment with triamcinolone, 0.1%, ointment. Physical examination showed friable erythematous plaques on the neck, inguinal folds, buttocks, bilateral axillae, and bilateral antecubital fossae. Punch biopsy results of the right axilla revealed acantholysis, confirming the diagnosis of benign familial pemphigus. Treatment with oral naltrexone, 4.5 mg, once daily, was initiated, which was followed by substantial clinical improvement within 6 weeks. An acute flare secondary to medication noncompliance prompted the addition of clobetasol, 0.05%, and mupirocin, 2%, external ointments to the treatment regimen for 4 weeks and on an as-needed basis after. Symptoms were controlled for 9 months until he developed a flare in the bilateral axillae and inguinal folds (Figure), at which point treatment with isotretinoin, 40 mg, once daily, was initiated in addition to continuing use of low-dose naltrexone.

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