Connective Tissue Disorders

Scleroderma is a syndrome in which genetic factors appear to predispose, and is characterized by inflammation associated with fibrosis and pathological remodeling of connective tissues. Pathological deposition of extracellular matrix in systemic sclerosis is most probably caused by changes in the regulation of dermal fibroblasts. The prevalence is 15/100,000. Affected patients are female in 80% of the cases. Early signs can appear under age of 3, but skin signs generally present in the forties.