Updates on hereditary angioedema in pediatrics

Abstract

Introduction Angioedema is characterized by an asymmetric nondependent swelling that is generally not pruritic. The pathogenesis of angioedema results from increased vascular permeability, with leakage of plasma into the deeper skin layers in patients with angioedema. Hereditary angioedema (HAE) is a rare genetic life-long disabling disease that predisposes an individual to develop vasogenic edema. HAE is an autosomal dominant disease, and most patients with HAE have a positive family history of angioedema. The prevalence of HAE is estimated to be between 1:30,000 and 1:80,000 in the general population, and there is no evidence of sex, ethnic, or racial differences in the prevalence of HAE. 1 Awareness and recognition of this disease is important as HAE is often misdiagnosed as allergic angioedema or acute abdomen (especially acute appendicitis). This may often lead to inappropriate use of antihistamines, corticosteroids, adrenaline and sometimes even surgical interventions.