Course: Benign Familial Pemphigus
CME Credits: 1.00
Released: 2022-01-12
A woman in her 50s presented with recurrent painful, pruritic, and malodorous rashes of the underarms and groin that were exacerbated by extensive sweating. Her family history had similar findings. Past unsuccessful treatment included aluminum chloride, glycopyrrolate, and triamcinolone, 0.1%, cream. Physical examination showed well-defined, macerated, erythematous plaques with stellate erosions within the bilateral axillary vaults (). With characteristic epidermal acantholysis on histology and a positive family history, a diagnosis of benign familial pemphigus was confirmed. Initial treatment included doxycycline, 100 mg, twice daily; naltrexone, 3 mg, daily; and halobetasol-tazoretene lotion, daily, as needed, which led to complete resolution of the clinical findings with postinflammatory pigment alteration within 2 months. She continued to receive maintenance therapy with treatment with low-dose naltrexone, to which calcipotriene, 0.005%, ointment was added, and she transitioned from use of doxycycline to narrow-spectrum sarecycline. To control the primary axillary hyperhidrosis and limit its association with the disease, 50 units of onabotulinumtoxinA were injected intradermally to each axilla.
Educational Objective
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