RT Journal Article
SR Electronic
T1 Nervous System Involvement in Degos Disease
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 646
OP 649
VO 26
IS 3
A1 Amato, Carmelo
A1 Ferri, Raffaele
A1 Elia, Maurizio
A1 Cosentino, Filomena
A1 Schepis, Carmelo
A1 Siragusa, Maddalena
A1 Moschini, Massimo
YR 2005
UL http://www.ajnr.org/content/26/3/646.abstract
AB 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.