hereditary angioedema

angioedema /an·gio·ede·ma/ (-ĕ-de´mah) a vascular reaction involving the deep dermis or subcutaneous or submucosal tissues, representing localized edema caused by dilatation and increased permeability of the capillaries, and characterized by the development of giant wheals.

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hereditary angioedema  an autosomal dominant disorder of C1 inhibitor (C1 INH), which causes uncontrolled activation of the classical complement pathway, manifested as recurrent episodes of edema of the skin and upper respiratory and gastrointestinal tracts with increased levels of several vasoactive mediators of anaphylaxis. It may be mediated by such factors as minor trauma, sudden changes in environmental temperature, and sudden emotional stress.

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Hereditary angioedema

A complement deficiency characterized by lymphatic vessel blockages that cause temporary swelling (edema) of areas of the skin, mucous membranes, and, sometimes, internal organs.

Mentioned in: Complement Deficiencies

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hereditary angioedema,

an inherited autosomal-dominant disorder characterized by the episodic appearance of nonpitting edema involving any part of the body, including mucosal surfaces. The attacks last 48 to 72 hours and can be life-threatening if edema obstructs the airway.

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angioedema

a condition characterized by the sudden and temporary appearance of large areas of painless swelling in the subcutaneous tissue or submucosa, with or without pruritus. Caused by immunological reactions, usually immediate type hypersensitivities. Sometimes referred to as angioneurotic edema.

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hereditary angioedema

in humans, the periodic occurrence of angioedema caused by a deficiency of the complement regulatory protein.