Systemic scleroderma or progressive systemic sclerosis is a connective-tissue disease characterized by vascular alterations and severe fibrosis. The disease has a predilection for female patients and affects all races and ages, although it is more common during the third and fourth decades of life. The sites most frequently involved are the skin, esophagus, lung, and kidney. Patients with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) seem to have a more benign clinical course than those with the diffuse form of the disease, which has a more serious prognosis owing to the high incidence of severe pulmonary fibrosis and kidney insufficiency.
The pathophysiology of scleroderma remains poorly understood. Two schools of thought prevail: some believe that the primary event is vascular damage, and others propose a derangement in collagen metabolism as the initial event.1 More recently, emphasis has been placed on the presence of mononuclear cell infiltrates
Fleischmajer R, Perlish JS, Duncan M. SclerodermaA Model for Fibrosis. Arch Dermatol. 1983;119(12):957-962. doi:10.1001/archderm.1983.01650360003004