Localized cutaneous scleroderma.

Abstract

Localized scleroderma in children includes morphea mainly on the trunk and linear scleroderma on the limbs, scalp, and face. Progressive systemic sclerosis is very rare in children and change from localized to progressive disease is extremely rare. Laboratory abnormalities occur with localized scleroderma, including eosinophilia, positive antinuclear factor (ANF), and increased immunoglobulin (Ig)G. The diseases are usually self limited, but involvement over bones may lead to marked functional impairment. Those on the face may be associated with underlying abnormalities and, occasionally, seizures. Treatment regimens are difficult to assess as there is no chemical marker, but penicillamine has gained wide use. It has numerous side effects and patients should be carefully monitored, particularly with regard to renal function. Other modalities have been used with variable success, but series of patients are small. Many medications produce skin softening in patients with progressive systemic sclerosis. The pathogenesis of skin sclerosis involves complicated interactions between vascular responses, lymphokines, and connective tissue proliferation. The etiology is completely unknown, despite a few cases associated with increased Borrelia titers.