Successful perinatal management of hereditary angioedema with normal C1 esterase inhibitor and factor XII mutation using C1 esterase inhibitor therapy

Hereditary angioedema (HAE) is a rare, autosomal dominant condition that consists of a deficiency or dysfunction of C1 esterase inhibitor (C1-INH). Various triggers can lead to self-limiting or life-threatening airway edema.1 Owing to its nonhistaminergic pathophysiologic features, HAE subtypes do not respond to epinephrine, corticosteroids, or antihistamine. Supplementation of C1-INH is an accepted and common treatment for HAE attacks and HAE prophylaxis.

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