De Arun K, Das Kallol, Sil Archan, Joardar Swarnali
Department of Pediatric Medicine, Medical College, Kolkata, India.
Indian J Dermatol. 2013 Sep;58(5):406. doi: 10.4103/0019-5154.117333.
Systemic sclerosis is a clinically heterogeneous systemic disease affecting the connective tissues of skin, walls of blood vessels and internal organs like lung, heart and kidneys. Systemic sclerosis is very unusual in pediatric population. Children represent fewer than 10% of all cases. We report a case of 11 years old girl of progressive systemic sclerosis presenting with features of cutaneous sclerosis, microstomia, mask-like facies, sclerodactyly, esophageal dysmotility, Raynaud's phenomenon, arthralgia and pulmonary fibrosis.
系统性硬化症是一种临床异质性的全身性疾病，会影响皮肤、血管壁以及肺、心脏和肾脏等内脏器官的结缔组织。系统性硬化症在儿童群体中非常罕见。儿童患者占所有病例的比例不到10%。我们报告一例11岁患有进行性系统性硬化症的女孩，其表现有皮肤硬化、小口症、面具脸、指硬皮病、食管动力障碍、雷诺现象、关节痛和肺纤维化。
