Hereditary angioedema. A hereditary disorder in the synthesis of the complement system
Vanderstock, L.; Vander Eecken, P.; Vermeersch, H.
Acta Oto-Rhino-Laryngologica Belgica 36(3): 418-431
1983
ISSN/ISBN: 0001-6497 PMID: 6613563 Document Number: 205656
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.