Scleroderma in childhood report of 5 cases

Abstract

Five cases of scleroderma in childhood have been reviewed. All presented with cutaneous complaints. Raynaud's phenomenon occurred in 2 and Sjögren's syndrome in one. A patient with linear scleroderma and hemiatrophy developed subcutaneous calcification 5 years after the onset of disease. Of the 5 patients reviewed, 2 have been clinically well for several years, while the disease is progressive in the remaining 3. Because of the clinical and serological overlap, confusion is possible between dermatomyositis, scleroderma, juvenile rheumatoid arthritis, and systemic lupus erythematosus, particularly when these connective tissue disorders are so uncommon in children. It appears that scleroderma in children may be less severe than in adults and that systemic involvement is less common. The presence of Raynaud's phenomenon, antinuclear factor, lupus erythematosus (LE) cells, and a raised erythrocyte sedimentation rate indicate a poor prognosis.