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Morphea profunda presenting as a neuromuscular mimic
N C Voermans1, S Pillen, E M de Jong
1Departments of Neurology, Neuromuscular Centre Nijmegen, Nijmegen Medical Centre, The Netherlands. n.voermans@neuro.umcn.nl
Journal of Clinical Neuromuscular Disease
|June 6, 2008
Summary
Localized scleroderma, specifically morphea profunda, can mimic neuromuscular disorders with muscle weakness and atrophy. Muscle biopsy is key to differentiating it from inflammatory myopathies.
Area of Science:
- Rheumatology
- Neurology
- Dermatology
Background:
- Localized scleroderma involves skin and connective tissue fibrosis.
- Deep morphea (morphea profunda) can affect underlying muscle.
- Neuromuscular disorders present with progressive muscle weakness and atrophy.
Observation:
- A patient with linear scleroderma developed progressive arm atrophy and weakness, resembling a neuromuscular condition.
- Muscle biopsy revealed myositis, consistent with morphea profunda.
- Skin sclerosis in this case was nonprogressive.
Findings:
- Morphea profunda can present as a neuromuscular mimic.
- Myositis in morphea profunda is typically localized, unlike diffuse inflammatory myopathies.
- Distinct skin and muscle biopsy findings differentiate morphea profunda from dermatomyositis.
Implications:
- Recognizing morphea profunda as a neuromuscular mimic is crucial for accurate diagnosis.
- Muscle biopsy carries sampling risks; electromyography and imaging may offer broader insights.
- Distinguishing morphea profunda from inflammatory myopathies impacts treatment and prognosis.
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