Myopathy and scleromyxedema.

Abstract

Scleromyxedema is a chronic, idiopathic disorder associated with monoclonal gammopathy, and characterized by dermal mucin deposition. However, systemic manifestations are frequent, including neuromuscular symptoms. We herein present a 71-year-old man who developed a vacuolar myopathy in a context of a known scleromyxedema, and we compare our observation with the nineteen other cases found in the medical literature. Such an association (especially with suggestive skin abnormalities) has to be known for two reasons. First, this diagnosis might be quite challenging because the myopathy may precede the typical skin changes. Secondly, conversely to other forms of vacuolar myopathy, some of the symptoms may respond (even partially) to immunomodulatory and/or immunosuppressant therapeutics.

• Authors: Antoine Soulages, Hoang Marie-Ange Tang, Anne Pham-Ledard, Marie-Laure Négrier-Leibreich, Alexandre Cosnard, Fanny Duval, Guilhem Solé, Louis Carla, Gwendal Le Masson, Stéphane Mathis
• Journal: Journal of neurology (J Neurol) Vol. 266 Issue 8 Pg. 2051-2059 (Aug 2019) ISSN: 1432-1459 [Electronic] Germany
• PMID: 31115676 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Immunoglobulins, Intravenous

Topics

• Aged
• Humans
• Immunoglobulins, Intravenous (administration & dosage)
• Lysosomal Storage Diseases (complications, diagnostic imaging, drug therapy)
• Male
• Muscular Diseases (complications, diagnostic imaging, drug therapy)
• Scleromyxedema (complications, diagnostic imaging, drug therapy)