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Effective treatment with alpha 1-protease inhibitor of chronic cutaneous vasculitis associated with alpha 1-antitrypsin deficiency.

The primary serum proteinase inhibitor is alpha 1-antitrypsin, and deficiency of this enzyme has been associated with a variety of systemic and cutaneous disorders. We report cutaneous vasculitis in a 49-year-old man with alpha 1-antitrypsin deficiency. His condition persisted despite treatment with colchicine, prednisone, and antibiotics but has been controlled by the administration of alpha 1-protease inhibitor.