Acquired angioedema (AAE)

Besides hereditary angioedema (HAE) there are further, extremely rare forms of  bradykinin-mediated angioedema,the so called acquired angioedemas (AAE), which characterised by a deficiency in C1-esterase inhibitor (C1-INH). Unlike HAE, however, AAE is not caused by a genetic mutation but by certain disorders or pathological processes in the body that either consume C1-INH or inactivate this protein. As a result, there is too little C1-INH available to halt the excess production of bradykinin: the bradykinin level rises and swelling of the skin and mucous membranes may occur.

Also distinct from HAE, the age of onset of this rare form of bradykinin-mediated angioedema is usually past the 30th year of life. In addition, other family members are not affected. AAE can be associated with another underlying disorder, which must, of course, also be investigated by your doctor.