In vitro studies have shown that imatinib 1mM inhibits strongly the growth of cutaneous
fibroblasts. The hypothesis is that imatinib inhibits PDGFR which is known to be a potential
target for the molecule, as recently also proposed after the discovery of autoantibodies
activating the PDGF receptors. Recent data indicate that TGFb is also a potential target of
imatinib. Cutaneous scleroderma is characterized by progressive cutaneous fibrosis caused by
hyperactive dermal fibroblasts. Since no established treatment for skin sclerosis in
scleroderma is currently available. This study will test the safety and efficacy of imatinib
in the treatment of patients with scleroderma and severe cutaneous involvement.