October 2006 | Volume 5 | Issue 9 | Original Article | 848 | Copyright © October 2006
Michael M. Sachse MD, Amor Khachemoune MD CWS, Kjetil K. Guldbakke MD, Michael Kirschfink DVM PhD
Hereditary angioedema (HAE) is a rare autosomal dominant disorder caused by a C1-inhibitor deficiency. It is characterized
by potentially life-threatening recurrent episodes of angioedema of the skin and mucosa. Several recent studies have further
elucidated the immunology of HAE implicating bradykinin, the key mediator of the contact system. This article reviews the
pathophysiology, subtypes, and clinical features of HAE. Therapeutic approaches for various clinical situations (emergency
and prophylactic regimens) are also discussed.