Hereditary angioedema: a broad review for clinicians

UC Nzeako, E Frigas, WJ Tremaine - Archives of internal …, 2001 - jamanetwork.com
Hereditary angioedema (HAE) is an autosomal dominant disease that afflicts 1 in 10 000 to
1 in 150 000 persons; HAE has been reported in all races, and no sex predominance has
been found. It manifests as recurrent attacks of intense, massive, localized edema without
concomitant pruritus, often resulting from one of several known triggers. However, attacks
can occur in the absence of any identifiable initiating event. Historically, 2 types of HAE have
been described. However, a variant, possibly X-linked, inherited angioedema has recently …