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Abstract：
BACKGROUND: Juvenile localized scleroderma (JLS) is a rare pediatric disease characterized by inflammation and skin thickening. JLS is associated with deep tissue and extracutaneous involvement that often results in functional impairment and growth disturbances. This article provides an overview of the disease with a focus on active features and treatment.
METHODS: We searched databases including PubMed, Elsevier and MedLine and Wanfang, reviewing publications from 2013 to 2019. Selected earlier publications were also reviewed.
RESULTS: Linear scleroderma is the most common JLS subtype. Several lines of evidence suggest that JLS is an autoimmune disease. Extracutaneous involvement is common and can present before the onset of skin disease. Multiple skin features are associated with disease activity, and activity can also manifest as arthritis, myositis, uveitis, seizures, and growth impairment. Systemic immunosuppressive treatment, commonly methotrexate with or without glucocorticoids, greatly improves outcome and is recommended for treating JLS patients with active disease and moderate or higher severity. Long term monitoring is needed because of the disease\'s chronicity and the high frequency of relapses off of treatment.
CONCLUSIONS: JLS is associated with a risk for disabling and disfiguring morbidity for the growing child. Identifying active disease is important for guiding treatment, but often difficult because of the paucity of markers and lack of a universal skin activity feature. More studies of JLS pathophysiology are needed to allow the identification of biomarkers and therapeutic targets. Comparative effectiveness treatment studies are also needed to work towards optimizing care and outcome.
METHODS: We searched databases including PubMed, Elsevier and MedLine and Wanfang, reviewing publications from 2013 to 2019. Selected earlier publications were also reviewed.
RESULTS: Linear scleroderma is the most common JLS subtype. Several lines of evidence suggest that JLS is an autoimmune disease. Extracutaneous involvement is common and can present before the onset of skin disease. Multiple skin features are associated with disease activity, and activity can also manifest as arthritis, myositis, uveitis, seizures, and growth impairment. Systemic immunosuppressive treatment, commonly methotrexate with or without glucocorticoids, greatly improves outcome and is recommended for treating JLS patients with active disease and moderate or higher severity. Long term monitoring is needed because of the disease\'s chronicity and the high frequency of relapses off of treatment.
CONCLUSIONS: JLS is associated with a risk for disabling and disfiguring morbidity for the growing child. Identifying active disease is important for guiding treatment, but often difficult because of the paucity of markers and lack of a universal skin activity feature. More studies of JLS pathophysiology are needed to allow the identification of biomarkers and therapeutic targets. Comparative effectiveness treatment studies are also needed to work towards optimizing care and outcome.
摘要：
背景：青少年局限性硬皮病（JLS）是一种罕见的儿科疾病，其特征是炎症和皮肤增厚。JLS与深部组织和皮外受累有关，通常会导致功能损害和生长障碍。本文提供了该疾病的概述，重点是主动特征和治疗。
方法：我们搜索了包括PubMed、爱思唯尔、MedLine和万方，审查2013年至2019年的出版物。还审查了选定的早期出版物。
结果：线性硬皮病是最常见的JLS亚型。一些证据表明JLS是一种自身免疫性疾病。皮肤外受累是常见的，并且可以在皮肤病发作之前出现。多种皮肤特征与疾病活动有关，活动也可以表现为关节炎，肌炎,葡萄膜炎,癫痫发作,和增长减值。全身免疫抑制治疗，通常甲氨蝶呤有或没有糖皮质激素，极大地改善了预后,建议用于治疗患有活动性疾病和中度或更严重的JLS患者.由于疾病的慢性性和治疗后复发的高频率，需要长期监测。
结论：JLS与成长中儿童致残和毁容的风险相关。识别活动性疾病对指导治疗具有重要意义,但通常很难，因为缺乏标记和缺乏通用的皮肤活动特征。需要对JLS病理生理学进行更多的研究以鉴定生物标志物和治疗靶标。还需要进行比较有效性治疗研究，以优化护理和结果。
方法：我们搜索了包括PubMed、爱思唯尔、MedLine和万方，审查2013年至2019年的出版物。还审查了选定的早期出版物。
结果：线性硬皮病是最常见的JLS亚型。一些证据表明JLS是一种自身免疫性疾病。皮肤外受累是常见的，并且可以在皮肤病发作之前出现。多种皮肤特征与疾病活动有关，活动也可以表现为关节炎，肌炎,葡萄膜炎,癫痫发作,和增长减值。全身免疫抑制治疗，通常甲氨蝶呤有或没有糖皮质激素，极大地改善了预后,建议用于治疗患有活动性疾病和中度或更严重的JLS患者.由于疾病的慢性性和治疗后复发的高频率，需要长期监测。
结论：JLS与成长中儿童致残和毁容的风险相关。识别活动性疾病对指导治疗具有重要意义,但通常很难，因为缺乏标记和缺乏通用的皮肤活动特征。需要对JLS病理生理学进行更多的研究以鉴定生物标志物和治疗靶标。还需要进行比较有效性治疗研究，以优化护理和结果。
