Scleromyxedema: a case clinically and histologically responsive to intravenous immunoglobulin.

The authors report a 60-year-old man who presented with a rash and developed ataxia, confusion, slurring of speech, and disorientation that prompted hospitalization. A diagnosis of generalized scleromyxedema with neurological features was eventually made and the patient was treated with intravenous immunoglobulin. The patient showed clinical response to intravenous immunoglobulin therapy, and repeat biopsy showed histological clearance of increased fibroblasts and mucin deposition, demonstrating both clinical and histological response to intravenous immunoglobulin.