首页|期刊导航|皮肤性病诊疗学杂志|448例局限性硬皮病患者的临床特征及诊疗现状分析

448例局限性硬皮病患者的临床特征及诊疗现状分析OA

Analysis of clinical characteristics and current status of diagnosis and treatment in 448 patients with localized scleroderma

中文摘要英文摘要

目的 分析局限性硬皮病(LS)患者的临床特征、诊疗现状及变化趋势,以提高对该病的认识.方法 收集南方医科大学皮肤病医院2019年1月至2024年12月期间确诊为LS患者的临床资料,采用横断面分析方法,对患者人口学特征(性别、年龄)、临床表现(皮损类型、分布部位、病程)、实验室检查、治疗方案等信息进行描述性统计,分析不同临床分型与发病部位的关系及治疗方案的差异.结果 共纳入448例局限性硬皮病患者,其中男121例,女327例,男女比例为1∶2.7.中位发病年龄为21(9,33)岁.主要临床亚型为局灶型(47.32%)、线状(40.00%)、泛发型(10.71%).不同类型LS在发病部位上差异具有统计学意义(P<0.001).极少数患者(1.56%)伴有临床症状(活动受限、麻木感).活动期患者较非活动期患者使用外用激素联合钙调磷酸酯抑制剂的时间更长、光疗频率较高(P<0.05).JAK抑制剂在临床中的使用比例较高.结论 LS好发于0~12岁儿童及19~40岁中青年人群,临床表现多样,诊断延迟普遍存在,活动期患者中系统性治疗应用较广泛,以JAK抑制剂为代表的新型靶向药物在临床中应用频率较高,但其远期疗效与安全性仍需进一步观察.

Objective To analyze the clinical characteristics,current management and trends of patients with localized scleroderma(LS),aiming to improve the understanding of this disease.Methods Clinical data of LS patients diagnosed at Dermatology Hospital,Southern Medical Uni-versity between January 2019 and December 2024 were collected.A cross-sectional analysis was conducted to describe patients' demographics(sex,age),clinical manifestations(lesion type,distribution,disease duration),laboratory findings,and treatment regimens.The differences in lesion site and treatment strategy across different clinical subtypes were evaluated.Results A to-tal of 448 LS patients were included,comprising 327 females and 121 males,with a median age at onset of 21 years(interquartile range:9,33),and a female-to-male ratio of 2.7∶1.The clinical subtypes of LS were predominantly plaque type(47.32%),followed by linear type(40.00%),and generalized type(10.71%).There was a significant variation in lesion distribution across these different subtypes(P<0.001).Only a small proportion of patients(1.56%)reported clin-ical symptoms such as joint limitation or numbness.Treatment approaches varied with the phase of the disease,with patients in the active phase more frequently receiving a combination of topical corticosteroids,calcineurin inhibitors,and phototherapy compared to those in the inactive phase(P<0.05).Additionally,Janus kinase(JAK)inhibitors were utilized relatively often in the clinical management of LS.Conclusions LS predominantly affects children aged 0-12 years and young to middle-aged adults aged 19-40 years.Clinical manifestations are heterogeneous.Systemic therapy is more widely applied in patients with active disease,and novel targeted agents,particu-larly JAK inhibitors,are increasingly utilized in clinical practice.However,their long-term effica-cy and safety require further investigation.

曹一帆;王宇

南方医科大学皮肤病医院,广东 广州 510091南方医科大学皮肤病医院,广东 广州 510091

局限性硬皮病临床特征诊疗现状

localized sclerodermaclinical featurescurrent status of diagnosis and treat-ment

《皮肤性病诊疗学杂志》 2026 (5)

347-355,9

10.3969/j.issn.1674-8468.2026.05.004

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