An unusual presentation of scleromyxedema as inflammatory myopathy

Kavadisseril Vivekanandan Vysakha 1, Rajalakshmi Poyuran 2, Sruthi S Nair 1 and Muralidharan Nair 1

1 Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India; 2 Department of Pathology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India

Scleromyxedema is a rare cutaneous mucinosis with frequent extracutaneous manifestations. Myopathy in scleromyxedema is a poorly recognized syndrome among neurologists and can mimic idiopathic and connective tissue disease-associated inflammatory myopathy. Diagnosis is suspected by the characterization of the skin lesions and clinched by skin and muscle biopsies. Here, we report a patient with scleromyxedema and myopathy with the characteristic histopathological feature of mucin deposition in skin biopsy. Her muscle biopsy showed a picture consistent with scleromyxedema myopathy with vacuolar and inflammatory changes. The association with paraproteinemia, propensity to life-threatening central nervous system disease and good response to intravenous immunoglobulin necessitate the accurate diagnosis of this condition.

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