The etiology of systemic scleroderma is still unknown. This disease is well individualized by its usual forms, i.e. acrosclerosis and diffuse form, whose features and prognosis differ. The description of new entities has generated further interest, particularly through a different diagnostic and therapeutic approach. No specific biologic or immunologic criteria of the disease have as yet been discovered. Prognosis depends mainly upon the severity of visceral involvement. There have been little improvements in therapy which rests upon presumptive physiopathological hypotheses.