Amato Carmelo, Ferri Raffaele, Elia Maurizio, Cosentino Filomena, Schepis Carmelo, Siragusa Maddalena, Moschini Massimo
Department of Neuroradiology, Oasi Institute for Research on Mental Retardation and Brain Aging, Troina, Italy.
AJNR Am J Neuroradiol. 2005 Mar;26(3):646-9.
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.
德戈斯病，又称恶性萎缩性丘疹病，是一种病因不明的罕见阻塞性血管病，其特征为独特的皮肤病变、内脏受累及不良预后。胃肠道和中枢神经系统最常受累，但也有仅表现为良性皮肤病变的病例报道。关于这种疾病的神经放射学报告极为罕见。我们报告一例29岁女性患者，其中枢和周围神经系统受累，在脑部磁共振成像上表现为进行性临床恶化和脑膜血管型；该患者其他器官未受累。
