Scleroderma in Children and Adolescents: Localized Scleroderma and Systemic Sclerosis

Abstract

Scleroderma is a rare disease that has two main forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are chronic diseases, can present in different patterns (subtypes), and are associated with extracutaneous involvement in pediatric patients. Morbidity and mortality is much worse for juvenile SSc with patients at risk for life-threatening lung, heart, and other visceral organ fibrosis and vasculopathy. Mortality is extremely rare in juvenile LS, but morbidity is common, with patients at risk for severe disfigurement and functional impairment. Scleroderma treatment is directed towards controlling inflammation and managing specific problems. Early diagnosis can greatly improve outcome.

Keywords: Extracutaneous involvement; Juvenile localized scleroderma; Juvenile systemic sclerosis; Morbidity; Morphea; Pediatric rheumatology; Pediatric scleroderma.