CHILDHOOD SCLERODERMA AND ITS PECULIARITIES

Based on a very large series of childhood scleroderma followed for mor e than ten years, the authors describe various subsets, which, in seve ral aspects, differ significantly from the adult forms. Of special imp ortance is a higher prevalence of linear scleroderma resulting in defo rmities 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 pathogen esis. Critical discussion on a possible transitional form between loca lized and systemic disease and visceral involvement in cutaneous forms is followed by clinically applicable laboratory studies and managemen t, including new therapeutic modalities.