A 70-year-old white woman presented to a local dermatology clinic with a 2-month history of blisters that had begun in the mouth and had progressed to involve the scalp and flexural areas, including the bilateral inframammary regions and the groin. These flaccid bullae ruptured easily, producing painful erosions. She was found to be otherwise healthy except for a long history of hypertension and hypercholesterolemia, which were controlled with nifedipine, quinapril hydrochloride, bisoprolol fumarate–hydrochlorothiazide, and atorvastatin. She had been taking these medications for more than 2 years. She denied any family history of similar eruptions or recent overseas travel. Several mucocutaneous biopsy specimens had been obtained at another medical center, including 2 from the gingiva, which revealed neutrophil-rich intraepithelial and, in some areas, suprabasilar acantholytic blisters. However, the results of direct and indirect immunofluorescence and enzyme-linked immunosorbent assay (ELISA) were repeatedly negative for desmogleins 1 and 3. Approximately 1 week before the patient presented,to our clinic, treatment was initiated with prednisone (1.5 mg/kg per day [80 mg/d]), azathioprine (1.75 mg/kg per day [100 mg/d]), and dapsone (50 mg/d).
Sibley Hash K, Rencic A, Hernandez MI, Hashimoto T, Nousari HC. Aggressive Immunosuppressive Therapy for a Refractory Case of IgA Pemphigus. Arch Dermatol. 2002;138(6):744-746. doi:10.1001/archderm.138.6.744