Definition: an inherited, autosomal-dominant disease characterized by episodic appearance of brawny nonpitting edema, most often affecting the limbs, but capable of involving other parts of the body, including mucosal surfaces such as those of the intestine (causing abdominal pain) or respiratory tract (causing asphyxia, which can require intubation to avoid fatal outcome). Associated with deficiency of inhibitor of first component of complement pathway (C1). Epinephrine is used in emergency treatment, long-term treatment with a variety of agents is effective.
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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.