ABSTRACT

Systemic sclerosis (SSc), or systemic scleroderma, is a chronic multisystem connective tissue disease characterized by cutaneous and visceral fibrosis and a widespread obliterative vasculopathy affecting small arteries and capillaries. SSc is the most serious form of scleroderma (thickening of the skin), a heterogeneous group of idiopathic fibrosing skin disorders that also includes localized scleroderma variants in which systemic involvement rarely occurs (1) (Table 1). Moreover, a number of other diseases, some caused by known environmental exposures, resemble scleroderma clinically (pseudoscleroderma syndromes) and may provide clues to pathogenesis (2) (Table 2).