Sk. Dowd et al., EFFECTIVE TREATMENT WITH ALPHA(1)-PROTEASE INHIBITOR OF CHRONIC CUTANEOUS VASCULITIS ASSOCIATED WITH ALPHA(1)-ANTITRYPSIN DEFICIENCY, Journal of the American Academy of Dermatology, 33(5), 1995, pp. 913-916
The primary serum proteinase inhibitor is alpha(1)-antitrypsin, and de
ficiency 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.