A 62-year-old woman presented with red nodules on her thigh and an acute respiratory infection for which she was taking clemastine and phenylpropanolamine. Similar lesions had developed 25 years earlier in the 34th week of a pregnancy. At that time, the patient was treated with a sulfonamide but developed angioedema. She then received intramuscular penicillin, but the skin lesions enlarged and ulcerated. A cesarean section was done at 38 weeks' gestation without complications. After parturition, the patient was treated with prednisone, 60 mg/d; this therapy was discontinued when the ulcerations rapidly enlarged. After several months, the skin lesions gradually healed.

On this most recent occasion, therapy with clemastine and phenylpropanolamine was discontinued and respiratory symptoms improved with amoxicillin. -1-Antitrypsin deficiency, phenotype PiZZ, was diagnosed after the -1-antitrypsin level was found to be 24 mg/dL (normal, 93 to 224 mg/dL). The panniculitis progressed, however, and edema developed on both legs. The patient was treated with doxycycline for 3 weeks, but the edema became incapacitating and began to extend to her upper thighs and abdomen. Because the patient became unable to ambulate for several weeks, a single infusion of -1 protease inhibitor (60 mg/kg of body weight) was given. The response was dramatic. Within 24 hours, the patient could walk; after three weekly infusions, the massive edema and panniculitis resolved.

Balk and colleagues [1] described a man with PiZZ phenotype and progressive edema of the extremities and abdominal wall who died of hypovolemic shock and sepsis. Smith and coworkers [2] described two patients with panniculitis who were receiving dapsone and who rapidly improved after infusions of -1 protease inhibitor.