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An otherwise healthy Black woman in her 30s presented to the dermatology clinic for evaluation of hypopigmented, intermittently pruritic patches. Patches were confined to her face and neck and occurred intermittently after her last pregnancy approximately 10 years ago. She had no history of eczema, atopic skin conditions, or autoimmune conditions and denied family history of such. In previous evaluations, the areas were treated with petroleum-based creams, low- to medium-potency topical steroids, pimecrolimus, and topical antifungals, without consistent improvement. Physical examination identified multiple hypopigmented macules and subtle patches symmetrically distributed across the lower aspect of the forehead, cheeks, nasofacial sulci, and nasolabial folds; and along the jawline and neck (Figure, A and B). Many had a slight overlying scale that was tested with potassium hydroxide and was negative for hyphae. No hair or nail abnormalities were noted. A 4-mm punch biopsy of a hypopigmented patch on the right cheek was performed (Figure, C and D).
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A. Pemphigus foliaceous
Biopsy revealed focal epidermal parakeratosis with minimal acanthosis. The stratum corneum was uniformly lifted off the granular layer, with subtle portions still attached. The dermis showed mild perivascular lymphocytes and melanin incontinence (Figure, C and D). Periodic acid-Schiff stain result was negative for fungus. Subsequent enzyme-linked immunosorbent assay (ELISA) for antibodies against desmoglein (Dsg) 1 and 3 was positive against Dsg 1. Histology demonstrating a subcorneal blister and positive ELISA for antibodies to Dsg 1 supported the diagnosis of pemphigus foliaceus (PF). The patient began receiving a prednisone taper with transition to mycophenolate mofetil, resulting in improvement.
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Corresponding Author: Kristina Burke, MD, Wenatchee Dermatology, 707 N Emerson, Wenatchee, WA 98801 (email@example.com).
Published Online: July 14, 2021. doi:10.1001/jamadermatol.2021.2466
Conflict of Interest Disclosures: None reported.
Disclaimer: The views expressed in this article are those of the authors and do not reflect the official policy of the Uniformed Services University, Department of the Army/Navy/Air Force, Department of Defense, or US government.
Additional Contributions: We thank the patient for granting permission to publish this information.
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