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Dermatomyositis and polymyositis in childhood
1Department of Pediatrics, University Hospital, Uppsala, Sweden.
Brain & Development
|January 1, 1990
Summary
This review covers childhood dermatomyositis and polymyositis, detailing skin, muscle, and tendon symptoms. Early, high-dose corticosteroid treatment, potentially long-term, is key, with physiotherapy and antimetabolites as adjuncts.
Area of Science:
- Pediatric Rheumatology
- Dermatology
- Neuromuscular Disorders
Background:
- Childhood dermatomyositis and polymyositis are rare inflammatory myopathies.
- Clinical presentation includes characteristic skin rashes and proximal muscle weakness.
Purpose of the Study:
- To review clinical findings, diagnostic procedures, and treatment strategies for pediatric inflammatory myopathies.
- To emphasize long-term management and potential sequelae.
Main Methods:
- Retrospective review of 40-50 childhood cases over 30 years.
- Analysis of clinical manifestations, diagnostic approaches, and treatment outcomes.
- Evaluation of therapeutic responses to corticosteroids and antimetabolites.
Main Results:
- Key symptoms include skin eruptions (knuckles, elbows, knees) and proximal muscle weakness.
- Corticosteroids are the primary treatment, requiring high initial doses and potential long-term use.
- Physiotherapy is crucial, and antimetabolites may be added for refractory cases.
Conclusions:
- Early and aggressive treatment with corticosteroids is essential for managing pediatric dermatomyositis and polymyositis.
- Long-term management, including physiotherapy, is often necessary for optimal outcomes.
- Most children survive with minimal or no long-term complications with appropriate care.