摘要
儿童局限性硬皮病(juvenile localized scleroderma,JLS)是儿童风湿性疾病的罕见类型,以炎症和皮肤硬化为特征。与成人局限性硬皮病不同,JLS患儿更易出现深部组织和皮肤外组织受累,且病程更长。文献报道30%~38%的JLS患儿有功能损害甚至致畸,严重影响患儿生命质量。目前尚无特效疗法,主要针对炎症反应、纤维化和免疫调节进行治疗,方法包括局部治疗、光疗、系统治疗以及出现畸形后的手术治疗。
Juvenile localized scleroderma(JLS)is a rare type of rheumatic disease in children,which is characterized by inflammation and skin sclerosis.Different from adult localized scleroderma,JLS patients are more likely to have deep tissues and extra skin tissues involvement,and the course of the disease is longer.It is reported that 30%~38%of JLS patients have functional damage or even deformity,which seriously affects the quality of life.While there is no specific treatment at present.The current therapies are mainly focused on the aspects of inflammatory response,fibrosis,and immune regulation,including local treatment,phototherapy,systemic treatment,and surgical treatment after deformity.
作者
罗鸯鸯
曾迎红
肖嵘
周斌
LUO Yang-yang;ZENG Ying-hong;XIAO Rong(Department of Dermatology,Hunan Children’s Hospital,Changsha 410007,China)
出处
《实用皮肤病学杂志》
2022年第6期360-363,共4页
Journal of Practical Dermatology
基金
湖南卫生健康委科研计划项目(B2019023)
湖南省自然科学基金项目(2021JJ40273)
关键词
硬皮病
局限性
儿童
病情评估
治疗
Scleroderma,localized,juvenile
Disease evaluation
Treatment
