ABSTRACT
The clinical picture of systemic sclerosis (SSc) may be variable at presentation. The predominant cutaneous involvement and the resultant clinical features can be categorized as those due to fibrosis and the others due to vasculopathy. Cutaneous appendages are involved in the fibrotic process, clinically appreciable as dry, lustreless skin surface and sparse body hair. Clinically evident renal dysfunction is not as common in SSc as in other collagen vascular disorders. The clinical course of SSc is chronic and progressive without evidence of sudden flares, classically described as the “monophasic course.” The classification system for SSc categorizes the entity into two subsets. The basis of this categorization is the extent of fibrosis on extremities; the limited cutaneous SSc and diffuse cutaneous SSc. The bound-down skin on the cheeks, peri-nasal, and perioral areas give rise to some of the characteristic changes of scleroderma-facies.
