ABSTRACT
Hereditary angioedema (HAE) is a rare disease, but it can be life-threatening. HAE is caused by C1 esterase inhibitor deficiency or its dysfunction. Submucosal and cutaneous swelling, and abdominal pain are the most common symptoms of HAE, but urticaria and hypotension are rare. Any case of HAE presented with hypotension has not been reported in Korea. We experienced a case of HAE presenting with severe abdominal pain, hypotension and urticaria. A 44-year-old female visited our clinic due to severe lower abdominal pain, eyelid/lip swelling, urticaria, and hypotension. Symptoms persisted even after epinephrine injection. She had experienced similar symptoms 3 times before. We measured serum C1 esterase inhibitor, C1 esterase inhibitor activity, and complement 4 level, and confirmed all 3 measurements were decreased. With a diagnosis of HAE, an auto-injectable icatibant (bradykinin β 2 receptor antagonist) was prescribed. We report a rare case of HAE presented with urticaria and hypotension.