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.

This study will test the efficacy and tolerance of patients with a high score of induration (modified Rodnan score > 20/54) Comparison : 34 patients with severe forms of cutaneous involvement will be evaluated in a double blind RCT comparing imatinib 400mg/j and placebo in a 6 month period. Efficacy will be assessed using a cutaneous induration scale and skin biopsy, and quality of life questionnaires.

Inclusion Criteria:

• More than 18 years old
• Documented diagnostic of scleroderma (systemic or cutaneous)
• Severe cutaneous sclerodermia or systemic sclerodermia with m-Rodnan score > 20/51
• Ejection fraction of more than 45 per cent at cardiac ultrasound pre-inclusion study
• Woman with efficient contraceptive method during trail treatment and during 3 month after the end of trial treatment
• All female patients with reproductive potential must have a negative pregnancy test (serum or urine) within the 7 days prior to enrolment
• Affiliated or profit patient of a social security system
• Signed informed consent

Exclusion Criteria:

• new systemic treatment, potentially interfering with disease progression, beginning 3 months prior the start trial treatment
• Patient with isolated cutaneous scleroderma treated with a drug potentially interfering with the course of the disease 4 weeks before starting the trial (Systemic corticosteroids, methotrexate, cyclophosphamide, bosentan)
• Scleroderma " en coup de sabre "
• Severe organ failure or anomaly of blood chemistry/hematology (bilirubin, SGOT, SGPT, creatinine > 1,5 ´ upper normal limit, polymorphonuclear granulocytes less than 1*10*9/l or platelets less than 50*10*9/l),
• Ongoing cancer
• Ejection fraction ≤ 45 per cent at cardiac ultrasound pre inclusion study
• myocardial infarction of less than 6 mois at pre inclusion visit
• Non controlled chronic illness (diabetes, chronic kidney failure, chronic hepatitis, HIV infection),
• Major surgery less than two weeks before inclusion
• Pregnancy or lactation
• Absence of validated contraception in childbearing women.
• Contraindication to imatinib mesylate treatment as specified in product specifications
• Non observance anticipated and absence of informed consent