Nervous system involvement in Degos disease.

[malignant atrophic papulosis]

Degos disease , or malignant atrophic papulosis , is a rare obstructive vasculopathy of unknown origin , characterized by distinctive skin lesions , visceral involvement , and an unfavorable outcome . The gastrointestinal tract and the central nervous system are most frequently affected , but cases limited to benign skin lesions have also been described . Neuroradiologic reports of this condition are exceptionally rare . We report the case of a 29 -year -old woman with central and peripheral nervous system involvement who presented with progressive clinical deterioration and a meningovascular pattern at cerebral MR imaging ; other organs were spared in this patient .