Recognition and management of scleroderma in children

Abstract

Juvenile scleroderma is a rare disease of childhood. The 2 main forms are localised and systemic scleroderma, although the pathogenesis of both forms is still unclear. The main clinical characteristic is the presence of skin sclerosis. Localised scleroderma can be divided into 3 different subtypes: morphoea, linear scleroderma and 'coup de sabre' lesions. The incidence is 0.2 to 0.4 per 100,000. 25 to 40% of patients are rheumatoid factor positive and 23 to 67% antinuclear antibody positive. An increased number of eosinophils occur in 31% of patients. Systemic sclerosis is an even rarer disease; approximately 10% of patients evolve the disease before the age of 18 years. The clinical presentation and disease course differ from the adult disease. This is reflected in the much better outcome. With regard to therapeutic options, there is still no gold standard for either form of the disease.