The fibrosis is especially prominent in the diffuse cutaneous form of SSc, where excessive connective tissue accumulation is due to overproduction of the extracellular matrix by fibroblasts and myofibroblasts, activated by soluble factors such as transforming growth factor beta (TGF-β) [22–26] and connective tissue growth factor [27]. Here, TGFB1 is linked to systemic sclerosis.