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You are seeing a 7 year old patient with his third lifetime episode of angioedema. He has swelling around both eyes and his right upper lip, but none of his tongue or pharynx, and he is maintaining his airway. He has a history of multiple food allergies. Besides lack of urticaria or itching, which of the following findings supports hereditary bradykinin-mediated angioedema over allergic histamine-mediated angioedema as the underlying pathophysiology?
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April 27, 2021 at 11:25 am
E) Abdominal or genitourinary swelling
Hereditary angioedema usually involves a mutation in the gene coding for C1 esterase inhibitor, and affects ~1:50,000 people. All of the answers listed are more indicative of allergic histamine-mediated angioedema except for abdominal, genitourinary, or peripheral swelling. The swelling of hereditary angioedema develops more slowly than histamine-mediated, and can last for several days. Low C4 and low C1 esterase inhibitor can confirm the diagnosis for most types of hereditary angioedema. Careful family history is important to suspect the diagnosis.