Eosinophilic vasculitis affecting multiple middle-sized arteries in a patient with Kimura's disease: A case report and literature review
Eosinophilic vasculitis affecting multiple middle-sized arteries in a patient with Kimura's disease: A case report and literature review
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木村患者的嗜酸粒细胞性血管炎累及多条中型动脉
DOI：
10.1016/j.alit.2018.04.010
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发表时间：
2018
影响因子：
6.8
通讯作者：
Nakajima H.
中科院分区：
文献类型：
--
作者：
Furuya H;Ikeda K;Suzuki J;Suzuki K;Nakamura K;Furuta S;Tamachi T;Suzuki K;Hirose K;Nakajima H.
木村氏病，或皮下嗜酸性淋巴肉芽肿，是一种罕见的良性慢性炎症性疾病，主要影响年轻的亚洲男性，其特征是在头部和颈部区域的复发性皮下肿块，显着的嗜酸性粒细胞增多，IgE水平升高。虽然有12e16%的患者报告同时存在肾脏疾病，1但很少累及其他器官系统，因此Kimura病预后良好。我们在此报告一个木村氏病并发嗜酸性血管炎的病例。我们也回顾先前报告的类似并发症的病例，并强调其共同的临床特征。一名25岁的日本男性，有12年的木村病病史，接受泼尼松龙10 mg/天治疗数年，入院时主诉左腿肿胀1年，右脚进行性麻木和指尖变黑4周，发热3天。由于他的Kimura病，他的右颈部有一个皮下肿块，多年来大小没有变化，持续性嗜酸性粒细胞增多（约1000/ml）和血清IgE升高（约10，000 IU/ml）。他没有其他病史，包括支气管哮喘或鼻窦炎，也没有吸烟史。入院时，他有发烧（38.7摄氏度），坏疽，左手第三指化脓性溃疡（图1A），双侧桡动脉和足背动脉搏动消失，左大腿青斑和肿胀（图1 B）和右侧胫骨前肌无力（手动肌肉测试4级）。血液检查显示明显的嗜酸性粒细胞增多（6679/ml）、血小板计数低（110，000/ml）、肝酶升高（AST 88 IU/l、ALT 78 IU/l、ALP 508 IU/l、g-GTP 167 IU/l）、CRP（2.9 mg/dl）和IgE（10，053 IU/ml），而自身抗体包括PR 3-ANCA和MPO-ANCA均为阴性。对比增强CT显示肝脏低增强区（图1C），血管造影显示多个中等大小动脉闭塞（图1D，E）。神经传导研究显示存在多发性单神经炎，皮肤活检显示血管炎，其特征为血管壁中大量嗜酸性粒细胞浸润，无肉芽组织（图1 F）。骨髓检查未见形态学和核型异常，荧光原位杂交（FISH）显示FIP 1 L1-PDGFRa、PDGFRb和FGFR 1基因重排缺失。我们诊断他患有嗜酸性血管炎。作为抗生素治疗，阿司匹林、肝素和前列腺素E1类似物仅改善了他的发热和局部感染体征。
Kimura's disease, or subcutaneous eosinophilic lymphoid granuloma, is a rare benign chronic inflammatory disorder that affects predominantly young Asian men and is characterized by recurrent subcutaneous masses in the head and neck region, marked eosinophilia, and elevated IgE levels. Although renal disease was reported to coexist in 12e16% of the patients, 1 the other organ systems are rarely involved and thus Kimura's disease has good prognosis. We herein report a case of Kimura's disease complicated by eosinophilic vasculitis. We also review previously reported cases with similar complication and highlight their common clinical features. A 25-year-old Japanese man with a 12-year history of Kimura's disease treated with prednisolone 10 mg/day for several years was admitted to our hospital, complaining of a one-year history of swelling in his left leg, a four-week history of progressing numbness in his right foot and blackening in his fingertips, and a threeday history of fever. Due to his Kimura's disease, he had a subcutaneous mass in his right neck, which had not changed in size for years and persistent eosinophilia (around 1000/ml) and serum IgE elevation (around 10,000 IU/ml). He had no other medical history including bronchial asthma or sinusitis and had no smoking history either.On admission, he had a fever (38.7 C), gangrene with suppurative ulceration in his left 3rd finger (Fig. 1 A), the loss of pulsation in his bilateral radial and dorsalis pedis arteries, livedo and swelling in his left thigh (Fig. 1 B), and weakness (manual muscle test grade 4) in his right tibialis anterior muscle. Blood test revealed marked eosinophilia (6679/ml), a low platelet count (110,000/ml), and elevated liver enzymes (AST 88 IU/l, ALT 78 IU/l, ALP 508 IU/l, g-GTP 167 IU/l), CRP (2.9 mg/dl), and IgE (10,053 IU/ml), while autoantibodies including PR3-ANCA and MPO-ANCA were negative. Contrast-enhanced CT revealed hypoenhanced areas in the liver (Fig. 1 C) and angiography revealed occlusion in multiple middlesized arteries (Fig. 1 D, E). Nerve conduction study showed the presence of mononeuritis multiplex and skin biopsy revealed vasculitis characterized by massive infiltrations of eosinophils in vessel walls without granulation (Fig. 1 F). Bone marrow examination exhibited no abnormality in morphology or karyotype, and fluorescence in situ hybridization (FISH) revealed the absence of FIP1L1-PDGFRa, PDGFRb and FGFR1 gene rearrangement. We diagnosed him as having eosinophilic vasculitis. As treatment with antibiotics, aspirin, heparin, and prostaglandin E1 analogue only improved fever and local infectious signs in his