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Review

Updates on the pathophysiology of dermal sclerosis

Pages 559-568 | Published online: 10 Jan 2014
 

Abstract

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.

Financial & competing interests disclosure

This work was supported in part by grants-in-aid for research on intractable diseases from the Ministry of Health, Labour and Welfare, and for scientific research from the Ministry of Education, Science and Culture of Japan (#16591090). The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

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