Nervous system involvement in Degos disease.
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.
Amato
Carmelo
C
Department of Neuroradiology, Oasi Institute for Research on Mental Retardation and Brain Aging, Troina, Italy.
Ferri
Raffaele
R
Elia
Maurizio
M
Cosentino
Filomena
F
Schepis
Carmelo
C
Siragusa
Maddalena
M
Moschini
Massimo
M
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