Lababidi H M, Nasr F W, Khatib Z
Department of Internal Medicine, American University Medical Center, Beirut, Lebanon.
J Rheumatol. 1991 Jun;18(6):885-8.
Five cases of juvenile progressive systemic sclerosis (SSc) are reported (4 girls and 1 boy). The age of onset of the disease ranged from 4 to 13 years. The clinical features included Raynaud's phenomenon present in 4 of 5 cases; hyperpigmentation, skin tightening and contractures of the large joints were noted in all 5 cases. One patient initially diagnosed as having eosinophilic fasciitis developed SSc 3 months later. Another patient was diagnosed initially as having juvenile rheumatoid arthritis. There was one case of pulmonary fibrosis and another of mild restrictive lung disease. Two cases of esophageal and intestinal hypomotility were reported. Scleroderma nephropathy was absent in all 5 cases.
报告了5例青少年进行性系统性硬化症（SSc）（4名女孩和1名男孩）。发病年龄在4至13岁之间。临床特征包括5例中有4例出现雷诺现象；所有5例均有色素沉着、皮肤紧绷和大关节挛缩。1例最初诊断为嗜酸性筋膜炎的患者在3个月后发展为SSc。另1例患者最初诊断为青少年类风湿性关节炎。有1例肺纤维化，另1例为轻度限制性肺病。报告了2例食管和肠道运动功能减退。所有5例均无硬皮病肾病。
