[Hereditary angioedema. A hereditary disorder in the synthesis of the complement system]

Hereditary angioedema is characterized by recurrent attacks of painless, non itching edema of the face and limbs and sometimes by abdominal symptoms. It is due to a deficiency of functional inhibitor of the first component of complement (C1 Inh). We present a case where a normal antigenic level of C1 Inh was found but no functional activity was present (B variant). A short review is given of the pathogenesis, heredity, diagnosis and treatment of the disease.