Foeldvari I, Wulffraat N
Paediatric Rheumatology Clinic, AK-Eilbek, Hamburg, Germany.
Paediatr Drugs. 2001;3(8):575-83. doi: 10.2165/00128072-200103080-00002.
Juvenile scleroderma is a rare disease of childhood. The 2 main forms are localised and systemic scleroderma, although the pathogenesis of both forms is still unclear. The main clinical characteristic is the presence of skin sclerosis. Localised scleroderma can be divided into 3 different subtypes: morphoea, linear scleroderma and 'coup de sabre' lesions. The incidence is 0.2 to 0.4 per 100,000. 25 to 40% of patients are rheumatoid factor positive and 23 to 67% antinuclear antibody positive. An increased number of eosinophils occur in 31% of patients. Systemic sclerosis is an even rarer disease; approximately 10% of patients evolve the disease before the age of 18 years. The clinical presentation and disease course differ from the adult disease. This is reflected in the much better outcome. With regard to therapeutic options, there is still no gold standard for either form of the disease.
青少年硬皮病是一种罕见的儿童疾病。主要有局限性和系统性硬皮病两种形式，尽管两种形式的发病机制仍不清楚。主要临床特征是皮肤硬化。局限性硬皮病可分为3种不同亚型：硬斑病、线状硬皮病和“剑击伤”皮损。发病率为每10万人中有0.2至0.4例。25%至40%的患者类风湿因子呈阳性，23%至67%的患者抗核抗体呈阳性。31%的患者嗜酸性粒细胞数量增加。系统性硬化症是一种更为罕见的疾病；约10%的患者在18岁之前发病。其临床表现和病程与成人疾病不同。这反映在其预后要好得多。关于治疗选择，对于这两种形式的疾病都仍没有金标准。
