硬皮病样表现伴关节挛缩的慢性移植物抗宿主病一例  

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

作　　者：李艳艳 张帆 刘红[1] 张福仁[1] LI Yanyan;ZHANG Fan;LIU Hong;ZHANG Furen(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)

机构地区：[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　　处：《中国麻风皮肤病杂志》2023年第2期106-108,共3页China Journal of Leprosy and Skin Diseases

基　　金：山东省高等学校青创科技支持计划(编号:2019KJL003)。

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。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.

关 键 词：慢性移植物抗宿主病 异基因造血干细胞移植 

分 类 号：R457.7[医药卫生—治疗学]