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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 26:646-649, March 2005
© 2005 American Society of Neuroradiology

Case Report
BRAIN

Nervous System Involvement in Degos Disease

Carmelo Amato^a, Raffaele Ferri^b, Maurizio Elia^b, Filomena Cosentino^b, Carmelo Schepis^c, Maddalena Siragusa^c and Massimo Moschini^d

^a Department of Neuroradiology, Oasi Institute for Research on Mental Retardation and Brain Aging, Troina, Italy
^b Department of Neurology, Oasi Institute for Research on Mental Retardation and Brain Aging, Troina, Italy
^c Department of Dermatology, Oasi Institute for Research on Mental Retardation and Brain Aging, Troina, Italy
^d Department of Neuroradiology, Catholic University, Rome, Italy

Address correspondence to Carmelo Amato, MD, Department of Neuroradiology, Oasi Institute, I-94018 Troina, Italy

Summary: 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.