Treatment of scleromyxedema with IVIg
© Moser and Griffin; licensee BioMed Central Ltd. 2012
Published: 13 July 2012
Report findings in a case of Scerlomyxedema.
Bone marrow aspirate showed mild hypocellularity with 2-3% plasma cells (polyclonal). Serum IgE level was 884 IU/mL, however serum protein electrophoresis and immunoelectrophoresis were normal. Electromyography (EMG) was consistent with a sensory/motor peripheral polyneuropathy with demyelinating and axonal features involving the arms. Additional laboratory workup for underlying autoimmune, thyroid, neurologic, infectious and neoplastic disease was negative. Based on histopathology and clinical features, he was diagnosed with Scleromyxedema, and therapy with intravenous immunoglobulin (IVIg) was initiated with moderate symptomatic improvement after the first infusion. After three infusions, there was complete resolution of symptoms, and he had a normal physical exam without rash or neurologic abnormalities. He was treated with IVIg for a total of 6 months, and has not experienced symptoms suggesting relapse during the 6 months after discontinuation of IVIg. Repeat EMG testing, 3 and 9 months post-presentation showed significant improvement, but persistence of very mild peripheral neuropathy.
To our knowledge, this patient is the youngest male reported in the literature to have Scleromyxedema without an associated autoimmune disease, and this case supports the benefit of IVIg for the treatment of Scleromyxedema.
David W. Moser: None; Thomas A. Griffin: None.
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