Angioedema is characterized by localized, rapid and severe swelling of the subcutaneous and submucosal tissues. Angioedema of the upper airway can lead to life-threatening airway obstruction. Acquired angioedema (AAE) is known to be caused by a deficiency or dysfunction of C1-esterase inhibitor (C1-INH) related to underlying diseases. We report a case of acute angioedema that was strongly suspected, but could not be confirmed, as a case of AAE. A 70-year-old man presented to us with sudden non-pitting edema of the floor of his mouth, tongue and neck. The swelling appeared within a few hours after the patient had bitten his own tongue. He was treated with tranexamic acid and the symptom improved promptly. He had experienced two episodes of acute angioedema in the past, and the first episode was when he was 68 years of age. There was no family history of angioedema. Based on the findings, we considered that this was not a case of hereditary angioedema (HAE). Blood test revealed reduced serum levels of C4, C1-INH, and C1q, and we strongly suspected that the patient had AAE. However, we could not detect any underlying diseases that could predispose to the development of AAE. Five months later, the patient developed the fourth episode of AE. Treatment with tranexamic acid failed to improve the symptom, and we had to perform tracheostomy for upper airway obstruction. The treatment of first choice for patients with AAE is control of the underlying disease. However, when the cause of the AAE remains unclear, AAE should be treated according to the treatment principles for HAE.
|Translated title of the contribution
|A case of oral and cervical edema strongly suspected as acquired angioedema
|Number of pages
|Published - 2021
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