嗜酸性粒细胞筋膜炎5例临床病理分析并文献复习  

Eosinophilic fasciitis:a clinicopathological analysis of 5 cases and lirature review

作　　者：曾艳平[1] 柳胤 解燕春[1] 梁静静[1] 卢祖能[1] ZENG Yanping;LIU Yin;XIE Yanchun;LIANG Jingjing;LU Zuneng(Department of Neurology,Renming Hospital of Wuhan University,Hubei Province,Wuhan 430060,China)

机构地区：[1]武汉大学人民医院神经内科,430060

出　　处：《疑难病杂志》2019年第5期511-514,518,共5页Chinese Journal of Difficult and Complicated Cases

基　　金：武汉大学自主科研项目(2042018kf0128)

摘　　要：目的总结嗜酸性粒细胞筋膜炎(EF)的临床表现、病理特征和诊治方法。方法收集2017年4月—2018年9月武汉大学人民医院神经内科诊治的5例EF患者,均符合2014年Pinal-Femandez和2017年Masatoshi Jinnin嗜酸性粒细胞筋膜炎的诊断标准,对其危险因素、临床表现、实验室检查、肌电图、MR、病理组织活检表现及治疗进行分析、结果嗜酸性粒细胞筋膜炎主要表现为对称性的肢体肿胀、僵硬、疼痛,Groove征以及肌腱挛缩;实验室检查外周血嗜酸性粒细胞增加,高丙种球蛋白血症,红细胞沉降率和C反应蛋白升高,血清肌酸激酶正常;肌肉MR检查显示筋膜增厚;肌电图均表现为肌源性损害;全程皮肤、筋膜、肌肉连续活检,肉眼可见筋膜明显增厚,组织学检查显示5例患者皮肤及筋膜均以淋巴细胞为主的炎性细胞浸润,可见嗜酸性粒细胞,4例患者肌肉组织有炎性细胞浸润;激素治疗有明显疗效。结论嗜酸性粒细胞筋膜炎除皮肤和筋膜的表现外,还有肌肉受损的表现;诊断除临床、实验室、影像学等检查外,主要依靠活组织检查;早期诊断和激素治疗效果较好。Objective To summarize the clinical manifestations, pathological features, diagnosis and treatment of eosinophilic fasciitis(EF). Methods Five EF patients were collected from April 2017 to September 2018 in the Department of Neurology, People’s Hospital of Wuhan University. They all met the diagnostic criteria of Pinal Fernandez in 2014 and Masatoshi Jinnin eosinophilic fasciitis in 2017. The risk factors, clinical manifestations, laboratory examinations, electromyography, MR, pathological biopsy and treatment were analyzed. Results Eosinophilic fasciitis is mainly characterized by symmetrical limb swelling, stiffness, pain, Groove sign and muscle contracture;laboratory examination of peripheral blood eosinophilia, hypergammaglobulinemia, erythrocyte sedimentation rate and C response Protein increased, serum creatine kinase was normal;muscle MR examination showed fascia thickening;electromyography showed myogenic damage;continuous skin, fascia, muscle biopsy, visible thickening of fascia, histology Examination showed that the skin and fascia of 5 patients were infiltrated by lymphocytes-based inflammatory cells, and eosinophils were observed. Four patients had inflammatory cell infiltration in muscle tissue;hormone therapy had obvious curative effect. Conclusion Eosinophilic fasciitis has not only skin and fascia manifestations, but also muscle damage manifestations;besides clinical, laboratory and imaging examinations, diagnosis mainly depends on biopsy;early diagnosis and hormone treatment are better.

关 键 词：嗜酸性粒细胞筋膜炎 临床表现 活组织检查 病理 

分 类 号：R593.2[医药卫生—内科学] R747.9[医药卫生—临床医学]