European Journal of Cardiovascular Medicine

Hereditary angioedema (HAE) is an autosomal dominant disease due to C1 esterase inhibitor deficiency (C1-INH). Patients present with non-pruritic subcutaneous and sub mucosal edema in the absence of urticaria in the first or second decade of life. Even though the disease causes significant morbidity and mortality, the diagnosis is often late or even completely missed. Diagnosis of HAE is made by assessing C1-INH levels in blood. In a populated country like India, the disease prevalence would be high. This case series emphasizes the importance of early diagnosis and prompt treatment of a potentially fatal disease.