A 31-year-old man presented in November 1998 with spontaneously arising bullae on his dorsal hands and fingers, which expanded into tender ulcers. A biopsy revealed a dense neutrophilic infiltrate with papillary dermal edema without vasculitis. Laboratory studies revealed leukocytosis, neutrophilia, and elevated liver function test results. Findings from serum protein electrophoresis, urinalysis, and urine porphyrins were normal. Bullous Sweet syndrome was diagnosed. He began treatment with prednisone, 60 mg/d, and topical Polysporin, with initial improvement. His disease flared over the next 6 months during any attempt to taper prednisone therapy, and he did not tolerate treatments with colchicine or dapsone. Flares responded briefly to treatment with IV methylprednisolone sodium phosphate. Minocycline hydrochloride therapy was added in August 1999 without significant benefit. A reevaluation in October 1999 revealed violaceous ulcers on the right first and left fourth fingers (2 cm) and the left dorsal hand (5 cm). A second biopsy (Table 1) revealed vasculitis, and his condition was now interpreted as atypical PG. Findings from colonoscopy and bone marrow biopsies were normal. Treatment with topical tacrolimus was not helpful, and 3-month trials of cyclosporine and thalidomide failed. By May 2000, lesions had developed on his ears and shoulders, which were poorly controlled with prednisone therapy, 40 mg/d, with methylprednisolone pulses every 3 to 4 weeks. Cyclophosphamide, 500 mg, was administered IV 4 times over 2 months with improvement, allowing for tapering of prednisone therapy to 25 mg/d. Therapy with saturated solution of potassium iodide was added in October 2000. He developed indurated papules on his forearms, and a biopsy confirmed lymphocytic infiltrate of Jessner. Therapy with hydroxychloroquine sulfate, 200 mg/d, was added. His disease flared despite increased use of corticosteroid, and in December 2001, cyclophosphamide was resumed monthly for 5 months, allowing for tapering of prednisone therapy to 20 mg/d. Cyclophosphamide therapy was subsequently discontinued because of concern for toxic effects, and he began methotrexate therapy in July 2002, at a weekly dose of 17.5 mg, allowing for tapering of prednisone therapy to 10 mg/d. His disease was well controlled in May 2003, at the time of his accidental death.