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Dermatomyositis and polymyositis in childhood
1Department of Pediatrics, University Hospital, Uppsala, Sweden.
Insights
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.
Abstract:
Personal experience of 30 years work with 40-50 cases of dermatomyositis and polymyositis in childhood is reviewed, stressing the clinical findings of skin eruptions on the knuckles, elbows and knees (except in the 10 per cent of patients without any skin involvement), weakness of particularly proximal muscles and tightness of tendons. Special diagnostic procedures are reviewed as well as treatment. The basic treatment is corticosteroids, which must be started at a high dose and as soon as possible be given at intervals of 48 hours to diminish the side-effects. In many, though not all, patients the treatment must be continued for years. The parents should always know that extended treatment may be necessary. Physiotherapy should be started cautiously and be slowly increased, close co-operation between the physician and the physiotherapist being necessary. If the patient does not respond to corticosteroids, antimetabolites may be added. Most patients survive with no or only minor sequelae.