Glossary

Angioedema (angioneurotic edema): recurring episodes of noninflammatory swelling.

Autosomal: a chromosome other than a sex chromosome, normally occurring in pairs in somatic cells and singly in gametes.

C1-INH: Complement 1 inhibitor esterase, a serpin protease that inhibits numerous chemicals; C1-INH is the only inhibitor of C1r and C1s in the classical complement pathway. It also regulates kinin generation via inactivation of factors XIIa plasma and plasma kallikrein. In sum, C1-INH regulates the complement, contact, fibrinolytic, and coagulation cascades.

Erythema Marginatum: Occasionally seen in HAE and is a non-raised rash that can be confused with serpiginous or annular hives

Hereditary angioedema (HAE): an autosomal dominant disease manifested by recurring episodes of edema of the skin, mucous membranes of viscera, and associated with either a deficiency of an esterase inhibitor of one of the components of complement or a functionally inactive form of the inhibitor.

Laryngeal HAE attacks: angioedema affecting or near the larynx (airway); laryngeal attacks can be fatal and emergency tracheotomy is sometimes necessary to obtain a patent airway.

Pitting (pitting edema): formation of well-defined, relatively deep depressions in a surface.

Urticaria: skin condition characterized by welts that itch intensely, caused by an allergic reaction, an infection, or a nervous condition.

For more information about HAE see: