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Myopathy and scleromyxedema
Antoine Soulages1, Hoang Marie-Ange Tang1, Anne Pham-Ledard2
1Department of Neurology, Nerve-Muscle Unit, CHU Bordeaux (Groupe Hospitalier Pellegrin), University of Bordeaux, Place Amélie Raba-Léon, 33000, Bordeaux, France.
Journal of Neurology
|May 23, 2019
Summary
Scleromyxedema, a rare skin disorder, can present with vacuolar myopathy, a muscle condition. Early recognition is key, as treatments like immunosuppressants may improve symptoms.
Area of Science:
- Neurology
- Dermatology
- Immunology
Background:
- Scleromyxedema is a rare chronic disorder linked to monoclonal gammopathy, characterized by mucin buildup in the skin.
- Systemic symptoms, particularly neuromuscular issues, are common in scleromyxedema patients.
Observation:
- A case report details a 71-year-old man with known scleromyxedema who developed vacuolar myopathy.
- This observation is compared with nineteen similar cases from existing medical literature.
Findings:
- The association between scleromyxedema and vacuolar myopathy can be diagnostically challenging, as myopathy may appear before characteristic skin changes.
- Unlike other vacuolar myopathies, some symptoms in this context may show partial improvement with immunomodulatory or immunosuppressant therapies.
Implications:
- Clinicians should consider vacuolar myopathy in patients with scleromyxedema, even with subtle skin findings.
- The potential for therapeutic response to immunomodulatory treatments offers a new avenue for managing scleromyxedema-associated myopathy.
