Hereditary angioedema is a congenital defect of the C1 inhibitor system of complement. It ranks among the primary immunodeficiencies.
This article summarizes the pathogenesis, clinical symptoms and sings, manifestations (edema of the skin and mucosa), differential diagnosis (differentiation from acquired angioedema) and reports on the current diagnostic (immunological and genetic) options. It also discusses the current therapeutic recommendations and procedures, including acute and prophylactic treatment as well as the therapeutic options for the treatment of patients in the Czech Republic.
The article also mentions the problems faced immunologists when providing such treatment.