Nervous system involvement in Degos disease

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)646-649
Number of pages4
JournalAmerican Journal of Neuroradiology
Volume26
Issue number3
Publication statusPublished - 2005

Fingerprint

Malignant Atrophic Papulosis
Nervous System
Central Nervous System
Skin
Peripheral Nervous System
Gastrointestinal Tract

ASJC Scopus subject areas

  • Clinical Neurology
  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology

Cite this

Nervous system involvement in Degos disease. / Amato, Carmelo; Ferri, Raffaele; Elia, Maurizio; Cosentino, Filomena; Schepis, Carmelo; Siragusa, Maddalena; Moschini, Massimo.

In: American Journal of Neuroradiology, Vol. 26, No. 3, 2005, p. 646-649.

Research output: Contribution to journalArticle

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AU - Ferri, Raffaele

AU - Elia, Maurizio

AU - Cosentino, Filomena

AU - Schepis, Carmelo

AU - Siragusa, Maddalena

AU - Moschini, Massimo

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AB - 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.

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