Acquired angioedema

In the aquired angioedema, an aquired deficiency of the plasma protease C1 inhibitor (C1-INH) causes transient and recurrent subcutaneous and/or submucosal edemas with abdominal pain and swelling. These edemas are insensitive to histamine and cortisol.

The disease does not occur until the age of 30 years. The family history is negative, as the AAE is not inherited. Affected patients complain of white, non-itchy edemas that persists for up to 72 hours and recurs at varying intervals. The edema is mostly localized to the lips, tongue, pharynx, and larynx, but may also occur on the hands, feet, arms, legs, scrotum, and digestive tract.

Occurrence in the digestive tract produces a clinical picture similar to an intestinal obstruction. With a false treatment of the edemas in the larynx there is a possibility of death in 25% of the cases. The cause of the edema is an increased permeability of the blood vessels through an increased concentration of bradykinin, which is a result of a C1-INH deficiency.

Acquired angioedema can be classified as AAE I, a concomitant disease of B lymphocyte disorder, and AAE II, autoantibodies to C1-INH.