Systemic Sclerosis And Localized Scleroderma

The scleroderma spectrum of disorders encompasses a wide variety of disparate conditions whose unifying feature is the clinical presence of hard, tight skin, and pathological deposition of excessive collagen. Children and adolescents with scleroderma make up a very small subgroup of patients seen in a general rheumatology clinic, and the type and pattern of scler-oderma is different to that seen in adults. Localized scleroderma is far more common in pediatrics than systemic sclerosis, by a ratio of at least 4:1 (94). There is a significant association with trauma, not seen in adults (94). Childhood-onset scleroderma resembles adult disease in its female predominance, racial predisposition, histological findings, and heterogeneity of clinical expression, but differs in a number of ways. In contrast to the adult disease, childhood onset scleroderma is associated with normal parameters of vascular activation (such as von Willebrand factor, angio-tensin-converting enzyme, endothelial-1 and E-selectin), T cell activation

(interleukin-2 receptors) and collagen synthesis (carboxy-terminal type I, amino terminal type III). It is also distinguished by a notable lack of anticentromere antibodies, and normal coagulation indices (94). Scleroderma in childhood, particularly the linear form of localized scler-oderma, causes growth defects and muscle wasting not seen in adults. In adults, Raynaud's phenomenon occurs in more than 95% of cases, but occurs less commonly in childhood scleroderma, seen in 82% of those with diffuse disease and only 14% of those with localized scleroderma (94).

There have been recent changes in the classification of scleroderma with continued work in this field. Since children with localized scleroderma will have disease into their adolescent or adult years, here follows a description of the juvenile forms.

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