Clinical, biochemical, and genetic characterization of type III hereditary angioedema in 13 Northwest Spanish families.

Background: A new variant of hereditary angioedema has been reported during the last decade. Three main characteristics distinguish it from classic hereditary angioedema: normal C1 inhibitor activity, predominance in women, and different genetic alterations.

Objective: To assess the symptoms, laboratory findings, and treatment of a population with type III hereditary angioedema from Northwest Spain.