以嗜酸性粒细胞增多伴血管淋巴样增生为皮肤表现的IgG4相关性疾病  

Immunoglobulin G4-related disease:a case report

作　　者：夏颖 杨秀奇 李慎秋[1] 曹育春[1] 邹耘[1] 刘冬先[1] 陈岚[1] XIA Ying;YANG Xiu-qi;LI Shen-qiu;CAO Yu-chun;ZOU Yun;LIU Dong-xian;CHEN Lan(Department of Dermatology,Tongji Hospital,Tongji Medical college,Huazhong University of Science and Technology,Wuhan 430022,China)

机构地区：[1]华中科技大学同济医学院附属同济医院皮肤科,湖北武汉430000

出　　处：《临床皮肤科杂志》2020年第9期545-549,共5页Journal of Clinical Dermatology

摘　　要：报告1例表现为嗜酸性粒细胞增多伴血管淋巴样增生的IgG4相关性疾病。患者男,56岁。头部多发肿块4年余伴右颈部肿块1年。血Ig G4水平和IgG4/IgG比值升高,磁共振成像(MRI)和正电子发射计算机断层显像技术(PET)检查提示腮腺肿大,颈部淋巴结增多。两次组织病理检查结果分别为:嗜酸性粒细胞增多伴血管淋巴样增生样表现;真皮内大量浆细胞、淋巴细胞,伴纤维结缔组织增生。免疫组化:IgG4阳性细胞最多区域约160个/HP,IgG4/IgG>0.80。结合临床表现及实验室检查最终诊断为IgG4相关性疾病。予以泼尼松30 mg/d口服,目前在随访中。A case of Immunoglobulin G4-related disease(IgG4-RD)with eosinophilia and angiolymphoid hyperplasia is reported.The patient had multiple head nodules for more than 4 years and right neck masses for 1 year.The level of IgG4 and the ratio of IgG4/IgG were increased.MRI and PET showed that the parotid gland was enlarged and the lymph nodes in neck were increased.Histopathology:eosinophilia with angiolymphoid proliferation,a large number of plasma cells and lymphocytes in dermis with fibroconnective tissue proliferation.Immunohistochemistry:the most areas of IgG4 positive cells are about 160/HP,IgG4/IgG>0.80.Combined with clinical manifestations and laboratory examination,IgG4 related diseases were finally diagnosed.Prednisone(30 mg/D)was given,and it is still in follow-up.

关 键 词：IGG4相关性疾病 结节 头部 

分 类 号：R758.2[医药卫生—皮肤病学与性病学]