[Some aspects of systemic and localized (limited) sclerosis in children]

Sclerosis is a rare connective tissue disease. Uncontrolled process of fibrosis and collagen accumulation in the tissues leads to functional impairment and structural changes in the skin and internal organs. A leading role in the pathogenesis of sclerosis is played by the interrelated mechanism of vascular injury, immune changes, and fibroblast activation. Fibrosis of the connective tissue of the skin and internal organs determines two forms of the disease: systemic sclerosis and localized (often called limited) sclerosis. Despite similarities in the histopathology of the skin, these two types differ fundamentally as to course and prognosis. The prevalence of sclerosis among children has not been determined. 2% to 11.5% of adults with systemic sclerosis noted the onset of the disease before the age of 16 years. The onset was prior to 10 years of age in 1% to 2% of adult patients. According to some authors, localized sclerosis is found approximately 2.7 per 100,000 thousand of children, 3 to 4 times more often in girls especially in the preschool and early school age. Localized sclerosis is seen 9 to 10 times more often than systemic sclerosis. This work discussed diagnostic criteria, differential diagnosis, and prognosis of sclerosis in children were.