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Scleroderma and fasciitis in children
1Royal Free Hospital, London, UK.
Insights
Childhood scleroderma primarily affects localized skin and soft tissues, differing from adult forms. Juvenile eosinophilic fasciitis is rare, with unclear causes and overlapping symptoms with other scleroderma types.
Area of Science:
- Pediatric Rheumatology
- Dermatology
- Immunology
Background:
- Scleroderma encompasses a range of disorders that can manifest in childhood.
- Childhood scleroderma predominantly presents as localized disease affecting skin and soft tissues, unlike the more common generalized forms in adults.
- Juvenile-onset eosinophilic fasciitis is a rare condition with limited existing literature.
Purpose of the Study:
- To review the clinical presentation of scleroderma in children.
- To explore the etiologic, immunologic, and pathogenic factors contributing to juvenile-onset scleroderma.
- To discuss the similarities and overlaps between childhood eosinophilic fasciitis, morphea, and linear scleroderma.
Main Methods:
- Literature review of childhood scleroderma, focusing on eosinophilic fasciitis.
- Analysis of clinical patterns in juvenile-onset scleroderma.
- Discussion of potential multifactorial etiopathogenesis.
Main Results:
- Localized scleroderma is more common in children than generalized forms.
- Childhood eosinophilic fasciitis shares similarities with adult presentations, including blurred diagnostic lines with morphea and linear scleroderma.
- The etiopathogenesis of juvenile scleroderma is largely unknown and likely multifactorial.
Conclusions:
- Childhood scleroderma exhibits distinct patterns compared to adult disease, with localized manifestations being predominant.
- Further research is needed to elucidate the complex etiopathogenesis of juvenile-onset scleroderma.
- The heterogeneity suggests juvenile scleroderma may comprise multiple distinct disease entities.
Abstract:
Scleroderma is a spectrum of disorders, all of which may occur in childhood. Childhood disease differs from adult disease in that localized forms predominate, with major problems confined to the skin and underlying soft tissues; generalized scleroderma, whether diffuse or limited, is less common. Childhood eosinophilic fasciitis is rare, and the literature is scant. The pattern appears to be similar to that in adults, and as in the adult form, the overlap between eosinophilic fasciitis, morphea, and linear scleroderma is blurred. The etiopathogenesis of juvenile-onset scleroderma is unknown but almost certainly multifactorial. The heterogeneity of its clinical expression may argue for it being more than one disease. This review attempts to document the expression of scleroderma in childhood and relate it to etiologic, immunologic, and pathogenic considerations.