Systemic Sclerosis and Localized Scleroderma in Childhood

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Juvenile scleroderma syndromes, including the systemic and the localized varieties, represent the third most frequent chronic rheumatic conditions in pediatric rheumatology practice. In children, systemic sclerosis shows a significantly less frequent involvement of all organs, a higher prevalence of arthritis and myositis, and a better outcome than in adults. Recently, new classification criteria were proposed, which help improve patient care by enabling earlier, more definite diagnoses and by standardizing the conduct of clinical trials. Localized scleroderma is the more frequent subtype of scleroderma in childhood. It comprises a group of distinct conditions that involve mainly the skin and subcutaneous tissues. They range from small plaques of fibrosis involving only the skin to diseases causing significant functional deformity with various extracutaneous features.

Section snippets

Juvenile systemic sclerosis

Juvenile systemic sclerosis (JSSc) is a chronic multisystemic connective tissue disease characterized by symmetrical thickening and hardening of the skin, associated with fibrous changes in internal organs. Although rare in children, it represents one of the most severe rheumatic conditions in pediatric rheumatology practice.

Juvenile localized scleroderma

JLS, also know as morphea, comprises a group of conditions that involve essentially the skin and subcutaneous tissues. They have various features and range from very small plaques to extensive fibrotic lesions that may cause significant functional and cosmetic deformity.

Although JLS is relatively uncommon, it is far more common than systemic sclerosis in childhood, by a ratio of at least 10:1 [20]. There is a mild female predilection (female to male ratio 2.4: 1) [21]. The mean age at disease

Summary

JLS is a challenging but uncommon disorder among children. A new classification is now available that recognizes that many children have more than one type of LS (morphea and linear) and that a small subset of patients have evidence of internal organ disease. New techniques are available to assess and follow lesions. Therapy is based on open-labeled experiences, and controlled trials using new guidelines are needed to determine the best approach to disease modification.

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