Abstract
Background
In women with sporadic recurrent angioedema with an unknown cause who are unresponsive
to antihistamines and have normal C1 inhibitor activity and a negative family history
of angioedema, it is unclear whether they have idiopathic angioedema or hereditary
angioedema with normal C1 inhibitor, and what impact exogenous estrogens have on their
angioedema.
Methods
A cohort of 147 women was analyzed for F12 exon 9 mutations and for the influence of oral contraceptives, hormonal replacement
therapy, and pregnancy on their angioedema.
Results
A total of 142 women had idiopathic angioedema unresponsive to antihistamines. Five
women had an F12 mutation and thereby hereditary angioedema with F12 mutations. Among the women with idiopathic angioedema, 63 had never taken estrogens.
There was no estrogen impact in 42 women, a moderate impact in 15 women, and a severe
impact in 22 women. The type and dose of estrogens did not differ in women with and
without an estrogen impact. In 5 women, idiopathic angioedema disappeared after desogestrel
use. Among the 5 women with hereditary angioedema with F12 mutations, angioedema symptoms occurred during 4 pregnancies, whereas no symptoms
occurred during any of the 58 pregnancies in women with idiopathic angioedema.
Conclusions
Women with recurrent angioedema unresponsive to antihistamines may have idiopathic
angioedema or, more rarely, hereditary angioedema with F12 mutations. Both conditions may be provoked or aggravated by exogenous estrogens.
In idiopathic angioedema, treatment with progestins may be helpful.
Keywords
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Article info
Footnotes
Funding: None.
Conflict of Interest: None.
Authorship: All authors had access to the data and a role in writing the manuscript.
Identification
Copyright
© 2013 Published by Elsevier Inc.
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- Progestins are Efficient Agents in Estrogen-sensitive Nonhistaminic AngioedemaThe American Journal of MedicineVol. 127Issue 10
