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Paediatric morphoea: a holistic review. Part 1: epidemiology, aetiopathogenesis and clinical classification
1Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
Insights
This review covers pediatric morphoea (localized scleroderma) in children, detailing its epidemiology, causes, and subtypes. It also outlines diagnostic approaches, activity markers, and treatment strategies for this fibrosing skin disorder.
Area of Science:
- Dermatology
- Pediatrics
- Rheumatology
Background:
- Morphoea, or localized scleroderma, is a fibrosing disorder affecting skin and subcutaneous tissues.
- Pediatric-onset morphoea is common, characterized by frequent relapses and complex pathogenesis.
- The condition presents with diverse clinical subtypes across all pediatric age groups.
Purpose of the Study:
- To provide a comprehensive overview of current literature on pediatric morphoea.
- To elucidate the epidemiology, aetiopathogenesis, and clinical classification of the disease in children.
- To review diagnostic criteria, disease activity markers, management, and natural history in Part 2.
Main Methods:
- A systematic literature search was conducted using PubMed, MEDLINE, and Google Scholar.
- Keywords included 'pediatric morphea', 'juvenile localised scleroderma', and 'juvenile systemic sclerosis'.
- Relevant studies (RCTs, reviews, original research) were selected and analyzed.
Main Results:
- Part 1 of the review summarizes epidemiology, aetiopathogenesis, and clinical classification.
- The disease affects children of all ages with complex mechanisms and varied subtypes.
- Research is actively focused on understanding pathophysiology and disease activity measures.
Conclusions:
- Pediatric morphoea is a significant condition requiring further research into its complex nature.
- Understanding epidemiology and classification is crucial for effective management.
- This review serves as a foundation for further exploration of diagnosis, activity markers, and treatment.
Abstract:
Morphoea, also known as localized scleroderma, is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Paediatric-onset disease is not uncommon and is associated with frequent relapses. The disease has complex pathogenetic mechanisms and multiple clinical subtypes, and affects children of all ages. Recent research has focused on elucidating the disease pathophysiology and identifying measures of disease activity. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Relevant studies, including randomized trials, reviews of standard current guidelines and original research articles, were selected, and results were analysed before being summarized. In the first of this two-part review, we provide a bird's-eye view of the current literature concerning the epidemiology, aetiopathogenesis and clinical classification of paediatric morphoea; in Part 2, we review the diagnosis, markers of disease activity, management and natural history.
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