Hereditary angioedema [HAE]: study of 30 cases

ABSTRACT

Hereditary angioedema [HAE] is not a rare condition in Iran. HAE is inherited as an autosomal dominant trait. We studied 30 cases of HAE from 1987 to 1992, twenty females and 10 males, with a mean age of 24.8 years. Clinical presentation was nonpitting edema of the face in all cases. In HAE type I, screening of complement profile revealed a C1 inhibitor deficiency in 60% and C4 deficiency in 66% of cases; but in type II HAE, the complement profile seems to be normal. Complement component study is a good tool for the diagnosis of type I HAE