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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

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【作者】 夏颖杨秀奇李慎秋曹育春邹耘刘冬先陈岚

【Author】 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;

【机构】 华中科技大学同济医学院附属同济医院皮肤科

【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。

【Abstract】 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
【Key words】 IgG4-related diseaseshead nodule
  • 【文献出处】 临床皮肤科杂志 ,Journal of Clinical Dermatology , 编辑部邮箱 ,2020年09期
  • 【分类号】R758.2
  • 【下载频次】176
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