Abstract
Hereditary angioedema is a rare autosomal dominantly inhereted severe disease which is characterized by the formation of painless subcutaneous and submucosa edema without itching. Due to mutation regulatory protein C1 inhibitor is either absent or functionless in patients suffering from hereditary angioedema.
Its prevalence is estimated to be from 1 : 30 000 up to 1 : 50 000. Hereditary angioedema is developing eihter due to increased catabolic degradation of C1 inhibitor molecules or therir neutralisation in the presence of specific autoantibodies.
In addition, in patients treated with angiotensin-converting enzyme inhibitors angioedema is induced as a result of decreased biodegradation of vasoactive kinins, such as bradykinin.