26-year-old woman presented with a 5-month history of painful oral erosions and a 1-month history of widespread flaccid vesicles over her face and torso. A biopsy specimen of a skin lesion revealed suprabasal acantholysis. The diagnosis of pemphigus vulgaris (PV) was confirmed by a positive intercellular (IC) antibody titer. The patient was treated with prednisolone (30-45 mg/d) combined with other immunosuppressive agents, including azathioprine (100 mg/d), cyclophosphamide (100 mg/d), methotrexate sodium (15 mg/wk), mycophenolate mofetil (1 g/d), and dapsone (100 mg/d), at different times over a 2-year period. The clinical course was complicated by episodes of severe flare, with hemorrhagic paronychia and onychomadesis that required pulse therapy with methylprednisolone, with or without cyclophosphamide, and intravenous immunoglobulin therapy. The disease proved to be recalcitrant to all these medications. The circulating IC antibody titer was 1:640. The treatments were associated with severe adverse effects, including iatrogenic Cushing syndrome, depression, severe infectious mononucleosis–like reaction with acute hepatitis, and cutaneous bacterial and fungal infections. The erosions on the face were particularly unresponsive to treatment, even with the addition of potent topical corticosteroids to the regimen.
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