title: Nervous system involvement in Degos disease.
abstract: 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.
authors.affiliation: Department of Neuroradiology, Oasi Institute for Research on Mental Retardation and Brain Aging, Troina, Italy.
authors.forename: Carmelo
authors.initials: C
authors.lastname: Amato
authors.forename: Raffaele
authors.initials: R
authors.lastname: Ferri
authors.forename: Maurizio
authors.initials: M
authors.lastname: Elia
authors.forename: Filomena
authors.initials: F
authors.lastname: Cosentino
authors.forename: Carmelo
authors.initials: C
authors.lastname: Schepis
authors.forename: Maddalena
authors.initials: M
authors.lastname: Siragusa
authors.forename: Massimo
authors.initials: M
authors.lastname: Moschini
mesh_terms: D000328:Adult; D001808:Blood Vessels / Q000473:pathology; D002490:Central Nervous System / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008578:Meninges / Q000098:blood supply / Q000473:pathology; D010525:Peripheral Nerves / Q000473:pathology*; D012867:Skin / Q000473:pathology; D017444:Skin Diseases, Papulosquamous / Q000175:diagnosis* / Q000473:pathology