Juvenile localized and systemic scleroderma

Abstract

Juvenile localized scleroderma and juvenile systemic sclerosis (jSSc) are sclerotic disorders which differ greatly in their extent of involvement and prognosis. Juvenile localized scleroderma, also called morphea, involves the skin and subcutaneous tissues. It occurs in 4.7-20 per 100,000 children and is ten times more frequent than jSSc. Lesions range from very small plaques to extensive fibrotic lesions which may cause significant functional impairment and cosmetic deformity. Extracutaneous signs are most often seen in linear, generalized, and pansclerotic morphea. Disease onset is usually during childhood or adolescence, although onset in early infancy and even at birth has been described. For lesions that cross joints or cause functional or cosmetic impairment, methotrexate (MTX), initially in combination with corticosteroids, is the treatment of choice. Juvenile systemic sclerosis (jSSc) is a multisystem connective tissue disease, with an estimated incidence of 0.27 per million of children. Approximately 5-10 % of all adults with systemic sclerosis (SSc) report the onset of the disease during childhood. It has a variety of clinical manifestations, sometimes different from the adult form. Mixed connective tissue disease (MCTD) and overlap syndromes have features of jSSc and sometimes even fulfill the criteria of jSSc.