Kawaguchi Yasuhisa, Miyazaki Yasushi, Kuriyama Kazutaka, Tomonaga Masao
Department of Hematology, Molecular Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University School of Medicine.
Rinsho Ketsueki. 2003 Oct;44(10):1020-5.
On March 16, 2000, a 37-year-old male was admitted to another hospital for fever, erythema of the limbs, and swelling of the right lower leg. The leukocyte count was 19,800/microliter, and the ratio of eosinophils was 61%, suggesting marked eosinophilia. Thoracic computed tomography (CT) revealed pneumonia in the left lung. However, the patient was negative for autoantibodies or parasitic antibodies. Administration of prednisolone at 80 mg/day resulted in a marked improvement of the symptoms and the eosinophilia. For diagnosis, detailed examination, and treatment, the patient was referred and admitted to our department on March 28. The dose of prednisolone was gradually decreased. On April 15, the agent was discontinued. Eosinophilia was not observed, however erythema of the limbs and swelling of the right lower leg recurred. Skin biopsy revealed in mild edema of the corium and eosinophilic infiltration, suggesting episodic angioedema associated with eosinophilia (EAE). In 1984, Greich et al. reported 4 patients with repeated angioedema, hives, and marked eosinophilia, and proposed the term EAE. Since then, more than 50 patients have been reported in Japan. Only 4 of these patients were males. We report on the present male patient together with the pathological findings.
2000年3月16日，一名37岁男性因发热、四肢红斑及右下肢肿胀入住另一家医院。白细胞计数为19,800/微升，嗜酸性粒细胞比例为61%，提示明显的嗜酸性粒细胞增多。胸部计算机断层扫描（CT）显示左肺肺炎。然而，患者自身抗体和寄生虫抗体检测均为阴性。给予泼尼松龙80毫克/天治疗后，症状和嗜酸性粒细胞增多明显改善。为进行诊断、详细检查及治疗，患者于3月28日转诊并入住我科。泼尼松龙剂量逐渐减少。4月15日，药物停用。虽未观察到嗜酸性粒细胞增多，但四肢红斑及右下肢肿胀复发。皮肤活检显示真皮轻度水肿及嗜酸性粒细胞浸润，提示与嗜酸性粒细胞增多相关的发作性血管性水肿（EAE）。1984年，格雷希等人报告了4例反复出现血管性水肿、荨麻疹及明显嗜酸性粒细胞增多的患者，并提出了EAE这一术语。从那时起，日本已报告了50多例患者。其中男性仅4例。我们报告该男性患者及病理检查结果。
