Les angiœdème héréditaires de type III : nouvelle maladie ou nouveau diagnostic ?

Abstract

Type III hereditary angiœdema (HAE) presents the same symptoms as type I and type II ones, which are associated with C1Inh deficiency. Women are principally affected. First symptoms often appear during pregnancies or with women taking combined pills. C1Inh and C4 assays are normal. Some of type III HAE can be associated with mutations of the Hageman factor gene. Type III HAE diagnosis is difficult. Kininogenase activity assay and the Hageman factor gene analysis will help us in the future. Treatment strategy is not known: tranexamic acid could be used. Also, C1Inh concentrate could be administrated for laryngeal attacks.