title: Severe neurologic involvement of Degos disease in a pediatric patient.
abstract: A 14-year-old male presented with paresthesias on the right upper and lower extremities, headache, and vomiting. In addition to worsening paresthesia and weakness on the right side of his body, blurred vision, fever, and skin lesions developed. He also had skin lesions characterized with 3-10 mm papules with a white atrophic center surrounded by pink rim mostly on the trunk and lower extremities. Brain magnetic resonance imaging showed chronic subdural effusion and encephalomalacia of the left cerebral hemisphere. Cerebrospinal fluid (CSF) examination revealed increased protein levels. Electromyography was consistent with diffuse polyradiculoneuropathy. Skin biopsy confirmed the diagnosis of a rare vasculopathy: Degos disease. A case presenting with chronic subdural effusion, encephalomalacia, elevated CSF protein, and polyradiculopathy should be carefully examined for skin lesions, which may suggest the diagnosis of Degos disease.
authors: 1Department of Pediatric Neurology, Dokuz Eylul University Medical School, Izmir, Turkey.
authors: Pakize
authors: P
authors: Karaoğlu
authors: Yasemin
authors: Y
authors: Topçu
authors: Erhan
authors: E
authors: Bayram
authors: Uluç
authors: U
authors: Yis
authors: Sevgi
authors: S
authors: Akarsu
authors: Ezgi
authors: E
authors: Atalay
authors: Tolga
authors: T
authors: Koroğlu
authors: Handan
authors: H
authors: Cakmakçi
authors: Erdener
authors: E
authors: Ozer
authors: Semra
authors: S
authors: Hız
mesh_terms: D000293:Adolescent; D001921:Brain / Q000000981:diagnostic imaging; D005528:Foot / Q000473:pathology; D006801:Humans; D017254:Leukemic Infiltration; D008279:Magnetic Resonance Imaging; D008297:Male; D054853:Malignant Atrophic Papulosis / Q000150:complications*; D009422:Nervous System Diseases / Q000175:diagnosis / Q000209:etiology*; D011859:Radiography; D012867:Skin / Q000473:pathology; D015898:Tomography Scanners, X-Ray Computed
keywords: Degos disease; encephalomalacia; polyradiculoneuropathy; subdural effusion; vasculopathy