Severe deficiency of alpha 1-antitrypsin associated with cutaneous vasculitis, rapidly progressive glomerulonephritis, and colitis.

The association of vasculitis with severe deficiency of alpha 1-antitrypsin is rare. This report describes a 44-year-old man with severe deficiency of alpha 1-antitrypsin associated with diffuse vasculitis involving skin, kidney (rapidly progressive glomerulonephritis), and colon (colitis). Colitis has not previously been reported in association with deficiency of alpha 1-antitrypsin. Other reported cases are reviewed and the possible immunologic mechanisms underlying the association are discussed.