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Pediatric scleroderma
1Department of Pediatrics, University of California at San Francisco, 94143-0105, USA.
Insights
Scleroderma in children involves skin and tissue fibrosis, often localized, causing asymmetry and disability. Pediatric management requires careful consideration of medication side effects and psychosocial impacts.
Area of Science:
- Pediatric Rheumatology
- Dermatology
- Systemic Autoimmune Diseases
Background:
- Scleroderma encompasses diverse conditions characterized by fibrosis of skin and other tissues.
- While less common in children, scleroderma significantly impacts pediatric morbidity and mortality.
- Localized forms are more prevalent in children, potentially leading to growth issues, asymmetry, and functional disability.
Purpose of the Study:
- To describe types of scleroderma in children.
- To review epidemiologic and etiologic factors of pediatric scleroderma.
- To discuss management strategies for childhood scleroderma.
Main Methods:
- Literature review of scleroderma in pediatric populations.
- Analysis of existing case series and clinical observations.
- Synthesis of current understanding of disease presentation and progression.
Main Results:
- Children predominantly develop localized scleroderma, unlike adults.
- Potential complications include facial/limb asymmetry, contractures, and functional impairment.
- Management must balance treatment efficacy with adverse effects like growth failure and osteoporosis.
Conclusions:
- Childhood scleroderma presents unique challenges, particularly localized forms impacting growth and development.
- Multidisciplinary management is crucial, addressing both physical and psychosocial aspects.
- Further controlled studies are needed due to the rarity of large patient cohorts.
Abstract:
Scleroderma is a diverse group of conditions which have in common fibrosis of skin and other tissues. Although less common in children than in adults, these conditions are an important cause of morbidity and occasional mortality when they occur in the pediatric population. Children are more likely than adults to develop localized forms of scleroderma, and because of the impact on growth, these can result in major facial or limb asymmetry, flexion contractures, and disability. Management approaches must take into consideration the effect of medications on the child (for example, growth failure and osteoporosis from corticosteroids) as well as the psychosocial impact of chronic illness and physical deformity on the child and family. This article describes the types of scleroderma identified in children, reviews epidemiologic and etiologic factors, and discusses management options. Because this is a rare group of diseases managed by both dermatologists and rheumatologists, large series of patients are rare, and controlled studies of management are not available.

