Abstract
Hereditary angioedema (HAE) is an uncommon genetic disorder, with patients experiencing unpredictable episodes of angioedema. Subtypes include HAE with a deficiency of C1 esterase inhibitor levels (HAE-C1INH) and HAE with normal serum and functional levels of C1-INH (HAE-nl-C1INH). The etiology, pathophysiology, and prevalence of HAE-nl-C1INH are not well understood. We present a case series of an unusual clinical symptom in patients with HAE-nl-C1INH.
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