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Esclerodermia cutânea localizada juvenil : aspectos clínicos e epidemiológicos de pacientes diagnosticados de 2006-2017 no ambulatório de dermatologia do HUB

  • Figueira, Renata Borges Fortes da Costa
Publication Date
Dec 06, 2018
Repositório Institucional da Universidade de Brasília
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Background: Localized scleroderma is a benign disease restricted to the skin and subcutaneous tissue. However, the disease course is unpredictable, and considerable functional and aesthetic abnormalities can develop, especially in childhood. In addition, although rare, extracutaneous manifestations – primarily articular, neurologic and ocular – are more frequent among the paediatric population. Delayed diagnosis and systemic treatment may be associated with poorer functional and aesthetic prognosis in childhood. Aims: To analyse the clinical and epidemiological profiles of children and adolescents with localized scleroderma receiving care at a health service in the Federal District, Brazil, and to investigate the prevalence of extracutaneous symptoms/signs and unaesthetic deformities, as well as their association with the clinical disease subtypes. We also analysed the diagnostic delay and its effects on the patients’ functional capacity and aesthetics. Methods: Prevalence study with 32 children and adolescents diagnosed with localized scleroderma, followed over 11 years at the paediatric dermatology outpatient clinic of a university hospital in the Federal District. Results: The ratio of affected girls to boys was 3.5:1; linear scleroderma was the most frequent subtype (47%). The prevalence of extracutaneous symptoms and signs was 68.7%; that of unaesthetic deformities was 68.7%. A significant association was found between articular symptoms/signs and the linear and mixed subtypes and between neurologic symptoms/signs and linear scleroderma. We also detected a significant association between unaesthetic deformities and linear scleroderma. The diagnostic delay was 2,2 years. However, no significant association was observed between diagnostic delay and extracutaneous symptoms/signs or unaesthetic deformities. Conclusions: The high prevalence of extracutaneous symptoms/signs and non-aesthetic deformities found is noteworthy and suggests that paediatric scleroderma may involve considerable morbidity. Although no association was found between diagnostic delay (>2 years) and functional/aesthetic disability, early disease recognition and treatment onset are crucial to individual patients' clinical prognoses.

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