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Vasculitis and calcinosis in juvenile dermatomyositis
1Rheumakinderklinik Garmisch-Partenkirchen, Germany.
Acta Universitatis Carolinae. Medica
|January 1, 1991
Summary
Juvenile dermatomyositis presents with vasculitic skin and organ involvement. Calcinosis is common but can regress, impacting functional outcomes in children.
Area of Science:
- Pediatric Rheumatology
- Dermatology
- Immunology
Background:
- Juvenile dermatomyositis (JDM) is an idiopathic inflammatory myopathy.
- It characteristically involves vasculitic skin lesions and can affect internal organs.
- Calcinosis is a frequent complication impacting long-term prognosis.
Purpose of the Study:
- To describe the clinical manifestations and complications of JDM in a cohort of 32 patients.
- To analyze the occurrence and progression of calcinosis in JDM.
- To evaluate factors influencing functional outcomes in JDM.
Main Methods:
- Retrospective case series of 32 patients diagnosed with juvenile dermatomyositis.
- Clinical data collection included skin and systemic manifestations, presence of calcinosis, and functional status.
- Longitudinal follow-up was used to assess calcinosis progression and regression.
Main Results:
- All patients exhibited vasculitic skin changes (e.g., facial erythema, Gottron's sign, ulcerations).
- Systemic vasculitis affected gastrointestinal, neurologic, and cardiac systems.
- Calcinosis developed in 21 patients, with regression observed in 6 cases after 1-5 years.
Conclusions:
- Juvenile dermatomyositis involves significant vasculitic manifestations affecting skin and internal organs.
- Calcinosis is a prevalent complication, but its potential for regression offers hope for improved outcomes.
- Functional outcome in JDM is primarily determined by the extent of calcinosis and muscle atrophy.