系统性硬皮病属于结缔组织疾病,分肢端硬皮病(95％)和弥漫性硬皮病。临床上有皮肤、关节、肌肉和内脏的表现,疾病的预后主要与内脏损害有关。二种类型硬皮病均有内脏病变,但在弥漫性硬皮病中更为常见。一、皮肤表现活动型硬皮病易于识别,皮肤呈特殊萎缩性硬化,面部消瘦,皮肤菲薄,紧贴深部组织,无褶纹,表情呆滞,鼻细长,鼻孔狭小,眼睑挛缩,口唇薄而僵硬,严重者影响张口。四 Systemic scleroderma is a connective tissue disease, scleroderma (95%) and diffuse scleroderma. Clinical manifestations of the skin, joints, muscles and viscera, the prognosis of the disease is mainly related to visceral damage. Both types of scleroderma have visceral lesions, but are more common in diffuse scleroderma. First, the skin manifestations of active scleroderma easily identifiable, the skin showed a special atrophic sclerosis, facial weight loss, thin skin, close to deep tissue, no folds, dull expression, slender nostrils, narrow nostrils, eyelid contracture, lip thin Stiff, severe affect the mouth. four