硬皮病样皮肤移植物抗宿主病24例临床特征分析

目的:分析异基因造血干细胞移植后硬皮病样皮肤移植物抗宿主病（GVHD）的临床特征和危险因素。方法:回顾2014—2019年间就诊于北京大学人民医院皮肤科硬皮病样皮肤GVHD患者24例的临床资料，分析临床特征、治疗、预后及发展为硬皮病样皮肤GVHD的可能因素。结果:24例硬皮病样皮肤GVHD患者中男11例，女13例，年龄...

Clinical features of 24 cases of scleroderma-like cutaneous graft-versus-host disease

Objective To investigate clinical features of and risk factors for scleroderma-like cutaneous graft-versus-host disease(GVHD)after allogeneic hematopoietic stem cell transplantation.Methods Clinical data were collected from 24 patients with scleroderma-like cutaneous GVHD in Department of Dermatology,Peking University People's Hospital from 2014 to 2019.Clinical features,treatment,prognosis,and possible factors influencing the development of scleroderma-like cutaneous GVHD were analyzed retrospectively.Results Among the 24 patients,11 were males,and 13 were females,aged 33±12 years;20 were human leukocyte antigen(HLA)-identical recipients,4 were HLA-haploidentical recipients;GVHD occurred 18.5(8.0,30.9)months after transplantation.Nineteen patients had discontinued anti-rejection therapy or received low-dose anti-rejection drugs before the onset of GVHD.Fifteen patients presented with generalized scleroderma-like lesions,1 with linear scleroderma-like lesions,5 with morphea-like lesions,and 3 with fasciitis-like lesions.None of the 15 patients with generalized scleroderma-like GVHD had Raynaud syndrome.Thirteen patients were accompanied by graft rejection in other systems,8 had joint mobility limitations,and 1 developed cutaneous squamous cell carcinoma secondary to chronic skin ulcers.All patients were treated with systemic glucocorticoids and immunosuppressive agents,and 11 also with topical glucocorticoids.An intensive follow-up was carried out in 11 patients,of whom 3 achieved marked improvement,4 achieved improvement,2 experienced exacerbation,and 2 died.A total of 223 patients with non-sclerodermatous cutaneous GVHD admitting during the same period served as controls,and the proportion of HLA-identical patients was significantly higher in the scleroderma-like cutaneous GVHD group(20/24,83.3%)than in the non-sclerodermatous cutaneous GVHD group(47/223,21.1%;P<0.001).Conclusions Scleroderma-like cutaneous GVHD commonly occurs late,and can mimic clinical manifestations of all 4 types of spontaneous scleroderma.HLA-identical transplants,premature discontinuation or excessive dose reduction of anti-rejection drugs may be risk factors for scleroderma-like cutaneous GVHD.