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Hereditary angioedema with normal C1 inhibitor

Eveline Wu, MD
Michael M Frank, MD
Section Editor
Sarbjit Saini, MD
Deputy Editor
Anna M Feldweg, MD


Hereditary angioedema (HAE) is characterized by recurrent, self-limited episodes of swelling primarily involving the skin and the mucosa of the gastrointestinal tract and upper airway. There are several subtypes. The clinical manifestations, pathogenesis, diagnosis, and management of HAE with normal C1 inhibitor (HAE with normal C1INH, previously called type III HAE) will be reviewed here.

HAE types I and II, which are characterized by abnormalities in the level and/or function of the inhibitor of complement component 1 (C1 inhibitor or C1INH), are discussed separately:

(See "Hereditary angioedema: Epidemiology, clinical manifestations, exacerbating factors, and prognosis".)

(See "Hereditary angioedema: Pathogenesis and diagnosis".)

(See "Hereditary angioedema: General care and long-term prophylaxis".)

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Literature review current through: Nov 2017. | This topic last updated: Aug 18, 2017.
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