RESUMO
BACKGROUND: Hereditary angioedema is an autosomal dominant inherited condition that is characterized by the local development of edema of the skin, subcutaneous tissue, and the walls of almost any organ, including the gastrointestinal and upper respiratory tracts. OBJECTIVE: To describe the clinical characteristics, diagnosis and treatment of six controlled patients with hereditary angioedema. PATIENTS AND METHODS: This paper presents clinical characteristics of six patients, four of them members of the same family, and two, isolated, non-family related cases. This contribution is a review of the latest literature related to diagnosis and treatment of hereditary angioedema. RESULTS: Hereditary angioedema is a rare clinical entity that has a complicated manifestation profile that requires to discard a wide series of differential diagnosis. Deeper knowledge of its genetics and physiopathology will allow us to explore new treatment alternatives in addition to those already available. CONCLUSIONS: By diffusion of isolated cases or familial cases series, as presented in this paper, the disease recognition and timely treatment will be reached.