ABSTRACT
Pediatric scleroderma includes localized scleroderma (LSc) and juvenile systemic sclerosis (JSSc). The predominant form of childhood scleroderma is LSc, also known as morphea, which principally involves the skin and underlying musculoskeletal structures. The onset of JSSc is usually insidious, and the interval between onset and diagnosis in childhood is often long. Diagnosis of childhood scleroderma is based mainly on clinical features and a histopathology may be indicated when there is an ambiguity in clinical diagnosis. Individuals with LSc during childhood may have an impaired quality of life and are more likely to develop autoimmune diseases during adulthood. A number of immunosuppressive or immunomodulatory agents are used with varying success in systemic sclerosis. Systemic therapy is indicated when there is a significant risk of morbidity, such as in deep pansclerotic morphea, progressive linear morphea overlying a joint, and generalized morphea.
