[Panniculitis associated with severe alpha 1-antitrypsin deficiency]

Genetically determined deficiency of alpha-1-antitrypsin, the primary serum protease inhibitor, can result in pulmonary emphysema or liver cirrhosis, and panniculitis may be an early symptom. A case of recurring panniculitis in a 29 year-old woman is described, which led to the diagnosis of severe alpha-1-antitrypsin deficiency (Pi ZZ). Serum alpha-1-antitrypsin levels should be measured in patients presenting with panniculitis.