Hereditary angioedema presenting as refractory urticaria: a case report

Abstract

Hereditary angioedema (HAE) is a rare autosomal dominant genetic disorder characterized by localized or diffuse swelling of an affected patient's face, neck, larynx, visceral organs, extremities, and trunk. There is also an acquired form of angioedema that has been described in patients with malignancies (i.e., lymphocytic leukemia) or collagen vascular disease, or in those who have developed C1 esterase inhibitor (C1-INH) autoantibodies. However, HAE is rarely associated with urticaria.