diagnosis, differential

• Hereditary angioedema (HAE) is a rare autosomal dominant
disorder characterized by recurrent episodes of painful (and
usually asymmetric) swelling without urticaria that leads to
substantial morbidity and even mortality (in the case of
laryngeal involvement) if left untreated.
• Delayed diagnosis and misdiagnosis of HAE are common,
particularly during pregnancy and the postpartum period.
• Hereditary angioedema should be considered in the differential
diagnosis of any patient presenting with unexplained
abdominal pain and recurrent episodes of angioedema
(particularly if asymmetric in nature) without urticaria.
• Tests to confirm the diagnosis of HAE include measurement of
C4 and C1 inhibitor (INH) antigen and function.
• Successful pregnancy and delivery are possible in HAE with
proper medical management, which includes plasma-derived
C1-INH and collaboration with HAE specialists.