We report a case of angioedema associated with C1 inhibitor deficiency. A 58-year-old man first noticed swelling of the hands in his twenties, which was spontaneously resolved within a few days. He suffered severe facial edema triggered by a dental treatment ten years ago. Subsequently, he had repeated episodes of swelling of the face, hands, feet, or genitalia, and had several attacks of abdominal pain due to an unknown cause. On January 2004, he visited the emergency department in our hospital, complaining of perioral edema after carelessly biting the lower lip. Computerized tomography revealed narrowing of the upper respiratory tract due to laryngeal edema during the stroke. The symptoms subsided within several days. Findings of blood examinations disclosed remarkably low titers of C1 inhibitor function (<25%, normal: 70~130%) and C4 (<2 mg/dl, normal : 14~39 mg/dl). The patient was diagnosed as angioedema associated with C1 inhibitor deficiency. Based on the normal value of serum C1q (10.0 mg/dl, normal : 8.8~15.3 mg/dl), hereditary angioedema was suspected, although his family had no previous experience of angioedema. He was treated with danazol at an initial dose of 200 mg daily, which provided symptomatic relief. Danazol was tapered down to the minimal effective dose of 150 mg daily.
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