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Two newly described subtypes of angioedema, often estrogen related, occur in women. The closely related conditions resemble angioedema caused by deficiency or dysfunction of the C1 esterase inhibitor (C1-INH). Estrogen-dependent angioedema runs in families and occurs only during pregnancy, oral contraceptive use, or estrogen replacement therapy. A second type, estrogen-associated angioedema, may also be heritable but can occur without obvious estrogen provocation.
Investigators studied four women who experienced attacks of angioedema of the pharynx and limbs. Some episodes were apparently precipitated by local trauma, upper respiratory tract infections, and pregnancy. A maternal aunt of one patient had suffocated during an attack at age 20. …