Localized scleroderma.

Abstract

Localized scleroderma has no recognized internal organ involvement. It may rarely coexist with a systemic connective tissue disease, but is not thought to progress to systemic sclerosis. Although never fatal, localized scleroderma may cause considerable disability from joint contractures and subcutaneous atrophy. Facial lesions are particularly disfiguring and may be accompanied by hemiatrophy. Serum auto-antibodies are common but do not imply systemic disease. The presence of ANAs or antibodies to ssDNA correlates directly with more extensive and prolonged disease. Blood eosinophilia may be striking, and its presence correlates with active disease. Patients with elevated serum IgG appear to be at a greater risk for the subsequent development of contractures. These laboratory abnormalities are useful in determining prognosis and during follow-up. No accepted and effective treatment exists. Physical therapy should be instituted immediately in all patients at risk for contractures. Systemic corticosteroids, used early in patients with active and widespread involvement, can be expected to improve the inflammatory phase. A variety of systemic agents have been reported to be helpful, but no controlled studies exist on their effectiveness.