Childhood scleroderma and its peculiarities

Based on a very large series of childhood scleroderma followed for more than ten years, the authors describe various subsets, which, in several aspects, differ significantly from the adult forms. Of special importance is a higher prevalence of linear scleroderma resulting in deformities and disabilities, as well as impairment of bone development in facial hemiatrophy. The immune abnormalities and vascular involvement leading to fibrosis are presented as the basic events in the pathogenesis. Critical discussion on a possible transitional form between localized and systemic disease and visceral involvement in cutaneous forms is followed by clinically applicable laboratory studies and management, including new therapeutic modalities.