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Progressive Systemic Sclerosis (Scleroderma)

General Considerations

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 may have a role. Symptoms usually appear in the third to fifth decades, and women are affected two to three times as frequently as, scleroderm, sclerderma, sleroderma, Sjorgrens, Sjorgren's syndrome, Sorgrens.

Scleroderma may be localized or systemic. Localized scleroderma—morphea, linear scleroderma—is 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 diffuse systemic scleroderma in having skin tightening limited to the hands and face (versus the trunk), a lower risk of renal involvement, a higher risk of pulmonary hypertension, and an overall better prognosis.

Rapid progression of visceral organ disease leading to death within a few years is much more common in diffuse systemic scleroderma than in CREST syndrome.

Clinical Findings

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

Laboratory Findings: Mild anemia is often present, and it is occasionally hemolytic because of mechanical damage to

Differential Diagnosis

Eosinophilic fasciitis is a rare disorder presenting with skin changes that appear to be like those in diffuse systemic scleroderma. The inflammatory abnormalities, however, are limited to the fascia rather than the dermis and epidermis.

The eosinophilia-myalgia syndrome was first noted in patients who ingested tryptophan, an essential amino acid that was sold—until banned by the Food and Drug Administration—as an over-the-counter remedy for insomnia and premenstrual

Case reports have suggested that silicone breast implants can cause scleroderma or other rheumatic diseases, including


Treatment of progressive systemic sclerosis is symptomatic and supportive. Severe Raynaud's syndrome may respond to calcium channel blockers, eg, nifedipine, 30–60 mg/d. Intravenous iloprost, a prostacyclin analog that causes vasodilation and platelet inhibition, is moderately effective in healing digital ulcers. Patients with esophageal disease should take medications in liquid or crushed form. Esophageal reflux can be reduced and scarring prevented by