Some patients may develop angioedema during periods of estrogen excess. This may be related to a defect in kinin formation and/or inactivation.
General features:
(1) clinical angioedema
(2) normal levels and function of C1-INH
(3) occurrence during times of estrogen excess (menstruation, pregnancy, hormone therapy)
Types:
(1) hereditary (familial), with autosomal dominant inheritance
(2) acquired
Almost all patients are female. 1 male with the acquired form developed angioedema when androgen deficient (relative estrogen excess).
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Specialty: Immunology/Rheumatology
ICD-10: ,