Angioedema

doi:10.1016/j.jaad.2004.09.032

Angioedema

2005-09-01

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DOI：10.1016/j.jaad.2004.09.032

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Although first described more than 130 years ago, the pathophysiology, origin, and management of the several types of angioedema are poorly understood by most dermatologists. Although clinically similar, angioedema can be caused by either mast cell degranulation or activation of kinin formation. In the former category, allergic and nonsteroidal anti-inflammatory drug-induced angioedema are frequently accompanied by urticaria. Idiopathic chronic angioedema is also usually accompanied by Urticaria, but can occur without hives. In either case, an autoimmune process leading to dermal mast cell degranulation Occurs in some patients. In these patients, histamine-releasing IgG anti-Fc epsilon R1 autoantibodies are believed to be the cause of the disease, removal or Suppression by immunomodulation being followed by remission. Angiotensin-converting enzyme inhibitor-induced angioedema is unaccompanied by hives, and is caused by the inhibition of enzymatic degradation of tissue bradykinin. Hereditary angioedema, caused by unchecked tissue bradykinin formation, is recognized biochemically by a low plasma C4 and low quantitative or functional C1 inhibitor. Progress has now been made in understanding the molecular genetic basis of the two isoforms of this dominantly inherited disease. Recently, a third type of hereditary angioedema has been defined by several groups. Occurring exclusively in women, it is not associated with detectable abnormalities of the complement system. Angioedema caused by a C1 esterase inhibitor deficiency can also be acquired in several clinical settings, including lymphoma and autoimmune connective tissue disease. It can also occur as a consequence of specific anti-C1 esterase autoantibodies in some patients. We have reviewed the clinical features, diagnosis, and management of these different subtypes of angioedema.

Keywords:

CHRONIC IDIOPATHIC URTICARIA

ANGIOTENSIN-CONVERTING-ENZYME

HEREDITARY ANGIONEUROTIC-EDEMA

EXERCISE-INDUCED ANAPHYLAXIS

ANTI-IGE AUTOANTIBODIES

FC-EPSILON-RI

ACQUIRED C1-INHIBITOR DEFICIENCY

INHIBITOR-ASSOCIATED ANGIOEDEMA

DRUG-INDUCED URTICARIA

C1 INHIBITOR

Angioedema

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