中国麻风皮肤病杂志 ›› 2023, Vol. 39 ›› Issue (2): 106-108.doi: 10.12144/zgmfskin202302106

• 病例报告 • 上一篇    下一篇

硬皮病样表现伴关节挛缩的慢性移植物抗宿主病一例

李艳艳,张帆,刘红,张福仁   

  1. 山东第一医科大学附属皮肤病医院（山东省皮肤病医院），山东省皮肤病性病防治研究所，山东济南，250022
  • 出版日期:2023-02-15 发布日期:2022-12-27

Chronic graft-versus-host disease with scleroderma-like manifestations and hand-foot joint contracture：a case report

LI Yanyan, ZHANG Fan, LIU Hong, ZHANG Furen   

  1. Shandong Provincial Hospital for Skin Diseases ＆ Shandong Provincial Institute of Dermatology and Veneorology, Shandong First Medical University ＆ Shandong Academy of Medical Sciences, Jinan 250022, China
  • Online:2023-02-15 Published:2022-12-27

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摘要： 患儿，男，5岁。双手足质硬、红斑、关节挛缩2年。患儿自幼反复罹患肺脓肿并伴有发热，4年前行基因检测，诊断为JAK3基因相关重型联合免疫缺陷症，遂行异基因造血干细胞移植。2年前先于足部出现脱皮，后双手足背、腕、肘、膝及髋关节处出现红斑、脱皮、硬化，伴手足关节挛缩。皮肤组织病理示移植物抗宿主反应并病毒感染。

关键词: 慢性移植物抗宿主病, 异基因造血干细胞移植

Abstract: A 5-year-old boy presented with hard texture, erythema and joint contractures of both hands and feet for 2 years. Suffered from repeated lung abscesses with fever since childhood, genetic testing was performed 4 years ago. He conducted allogeneic hematopoietic stem cell transplantation following up the diagnosis of “JAK3 gene-related severe combined immunodeficiency”. Peeling occurred on the feet 2 years ago, and then erythema, peeling, and sclerosis appeared on the back of both hands, wrists, elbows, knees and hip joints. Histopathology of skin lesions revealed graft-versus-host reaction and viral infection.

Key words: chronic graft-versus-host disease, allogeneic hematopoietic stem cell transplantation