Pediatric scleroderma

Abstract

Scleroderma is a diverse group of conditions which have in common fibrosis of skin and other tissues. Although less common in children than in adults, these conditions are an important cause of morbidity and occasional mortality when they occur in the pediatric population. Children are more likely than adults to develop localized forms of scleroderma, and because of the impact on growth, these can result in major facial or limb asymmetry, flexion contractures, and disability. Management approaches must take into consideration the effect of medications on the child (for example, growth failure and osteoporosis from corticosteroids) as well as the psychosocial impact of chronic illness and physical deformity on the child and family. This article describes the types of scleroderma identified in children, reviews epidemiologic and etiologic factors, and discusses management options. Because this is a rare group of diseases managed by both dermatologists and rheumatologists, large series of patients are rare, and controlled studies of management are not available.