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Progressive systemic sclerosis is a chronic disorder characterized by the development of diffuse fibrosis of the skin and internal organs. The cause of scleroderma remains unknown; however, autoimmunity, fibroblast disregulation, and occupational exposure to silica and silicone may have a role. Symptoms usually appear in the third to fifth decades.
Scleroderma may be localized or systemic. Localized sclerodermaCmorphea, linear sclerodermaCis not associated with visceral organ involvement and is therefore benign. Two forms of systemic scleroderma are generally recognized: diffuse (20% of patients) and limited (80%). Patients with limited systemic scleroderma frequently have calcinosis cutis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasia (CREST syndrome). Patients with CREST syndrome differ from those with systemic sclerosis.
Rapid progression of visceral organ disease leading to death within a few years.
Symptoms and Signs: Most frequently, the disease makes its appearance in the skin, although visceral involvement may precede cutaneous alteration. Polyarthralgia and Raynaud's phenomenon (present in 90% of patients) are early manifestations. Subcutaneous edema, fever, and malaise are common. With time the skin becomes thickened and hidebound, with loss of normal folds. Telangiectasia, pigmentation, and depigmentation are
Treatment
Treatment of progressive systemic sclerosis is symptomatic and supportive. Severe Raynaud's syndrome may respond to calcium channel blockers, eg, nifedipine, 30B60 mg/d. Intravenous iloprost, a prostacyclin analog that causes vasodilation and platelet inhibition, is moderately effective.