摘要 : Scleroderma is characterized by vascular injury and increased production and accumulation of extracellular matrix proteins by activated fibroblasts in the skin of patients with immunologic abnormalities. A growing body of evidence... 展开 Scleroderma is characterized by vascular injury and increased production and accumulation of extracellular matrix proteins by activated fibroblasts in the skin of patients with immunologic abnormalities. A growing body of evidence has demonstrated that extracellular matrix overproduction in the sclerotic dermis by scleroderma fibroblasts results from complex interactions among endothelial cells, immunocytes and fibroblasts, mediated by various cytokines, chemokines and their receptors. Recently, novel signaling pathways leading to fibrosis have been clarified and genomic analysis have also been progressing. In tandem with investigation of human scleroderma, animal models are indispensable for a better understanding of the pathomechanisms of scleroderma, and a number of animal models have been developed. In this review, current insights into the pathophysiology of dermal sclerosis and therapeutic approaches are discussed. 收起