Hereditary angioedema (HAE) is a rare disease, which shows a preponderance of female sufferers. There are various types of HAE, with or without C1 inhibitor deficiency but estrogens may worsen the course of the disease in all the types. It is thus mandatory to know this sensitivity to estrogens in order to improve the management of women with HAE during their child-bearing ages. Contraceptive progestin can help to reduce the frequency and severity of the attacks. Pregnancies can be associated with worsening or improvement. Some other situations where endogenous or exogenous estrogens concentration can increase are possibly associated with an aggravation of the attacks (ART, tamoxifène).
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