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Polyneuropathy in juvenile dermatomyositis
S A Vogelgesang1, J Gutierrez, G L Klipple
1Department of Medicine, Walter Reed Army Medical Center, Washington, DC 20307-5000, USA.
The Journal of Rheumatology
|July 1, 1995
Summary
Juvenile dermatomyositis (DM) can rarely cause polyneuropathy, a nerve disorder. This condition may be linked to skin lesions and endothelial damage, leading to nerve ischemia.
Area of Science:
- Neurology
- Rheumatology
- Pathology
Background:
- Juvenile dermatomyositis (DM) is an idiopathic inflammatory myopathy.
- Neurological complications are rare in juvenile DM.
Observation:
- Two patients with juvenile DM presented with distinct clinical polyneuropathies.
- Associated symptoms included ulcerative skin lesions and elevated Factor VIII related antigen.
- Muscle and nerve biopsies were performed for detailed analysis.
Findings:
- Muscle biopsies showed perifascicular atrophy and microinfarcts, characteristic of juvenile DM.
- Sural nerve biopsies revealed axonal degeneration.
- Electron microscopy identified capillary endothelial inclusions, indicative of early endothelial injury.
Implications:
- Polyneuropathy is a rare but significant complication of juvenile DM.
- The findings suggest nerve ischemia secondary to endothelial damage as the underlying mechanism.
- This highlights the importance of considering neurological involvement in juvenile DM patients with specific clinical presentations.