[Angioneurotic edema in hereditary alpha-1-antitrypsin deficiency].

A young man with angioedema presented with normal concentration and function of C1-esterase inhibitor, but with a hereditary deficiency of alpha-1 antitrypsin (Pi-ZZ type). The pathogenetic relevance of this defect is discussed with respect to the partial deficiency of the complement component C4 (C4BQ0), which was found in addition.