Morphea profunda is a rare disease that mainly affects young women and often has a progressive course with physical and psychological sequelae. The skin becomes sclerotic after an initial inflammatory reaction and joint contractures can develop. The aetiology is unknown. Until now, no successful therapy has been proven for this morphea variant. On the basis of new insights into the key role of effector T cells in scleroderma, in particular Th-17, T-cell directed therapies are expected to have promising effects. We report here the first two cases of morphea profunda treated with abatacept. Abatacept had a clinical effect on the active disease, in addition to softening old sclerotic lesions.