Myelomalacia and hypoglycorrhachia in malignant atrophic papulosis

A 25-year-old man with the skin lesions of malignant atrophic papulosis had clinical and electrodiagnostic evidence of a multifocal asymmetric myelomalacia or polyradiculopathy in association with elevated CSF protein and hypoglycorrhachia. Autopsy findings included widespread infarctions and necrosis of brain, brainstem, and spinal cord. The combined clinical and laboratory findings were similar to those seen in systemic lupus erythematosus, sarcoidosis, or meningeal carcinomatosis. Thus, malignant atrophic papulosis should be added to the differential diagnosis of either polyradiculopathy or myelomalacia.