Degos disease: A radiological-pathological correlation of the neuroradiological aspects of the disease.
Adolescent
Antibodies, Monoclonal, Humanized
/ therapeutic use
Biological Factors
/ therapeutic use
Central Nervous System
/ diagnostic imaging
Cranial Nerve Diseases
/ diagnosis
Disease Progression
Drug Therapy, Combination
Fatal Outcome
Female
Gait Disorders, Neurologic
/ diagnosis
Humans
Magnetic Resonance Imaging
/ methods
Malignant Atrophic Papulosis
/ complications
Nervous System Diseases
/ diagnosis
Nitriles
Pyrazoles
/ therapeutic use
Pyrimidines
Skin
/ pathology
Thrombotic Microangiopathies
/ etiology
Urinary Incontinence
/ diagnosis
Degos
Microangiopathy
Neuroradiology
Papulosis
Spinal cord
Journal
Annals of diagnostic pathology
ISSN: 1532-8198
Titre abrégé: Ann Diagn Pathol
Pays: United States
ID NLM: 9800503
Informations de publication
Date de publication:
Aug 2020
Aug 2020
Historique:
received:
17
05
2020
accepted:
19
05
2020
pubmed:
9
6
2020
medline:
23
6
2021
entrez:
8
6
2020
Statut:
ppublish
Résumé
Malignant atrophic papulosis (Degos disease) is an unusual thrombotic microangiopathy of uncertain etiology. The disease characteristically involves the skin and internal organs, with nervous system involvement more common in children. We present a case with diverse neurological manifestations including cranial nerve palsies, gait instability, and urinary incontinence. The patient also developed white papular lesions on her lower extremities and back. Magnetic resonance imaging (MRI) demonstrated progressive intracranial and spinal abnormalities. Despite treatment with numerous biologic agents, the patient had persistent clinical deterioration and expired one month after admission. We highlight the extensive neurologic manifestations of Degos disease correlated with neuroradiological imaging and pathological features. Nervous system involvement in Degos disease requires careful neurologic and dermatologic exam with central nervous system (CNS) magnetic resonance imaging to distinguish it from non-organic etiologies of similar symptoms.
Identifiants
pubmed: 32505971
pii: S1092-9134(20)30086-1
doi: 10.1016/j.anndiagpath.2020.151545
pii:
doi:
Substances chimiques
Antibodies, Monoclonal, Humanized
0
Biological Factors
0
Nitriles
0
Pyrazoles
0
Pyrimidines
0
ruxolitinib
82S8X8XX8H
tocilizumab
I031V2H011
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
151545Informations de copyright
Copyright © 2020 Elsevier Inc. All rights reserved.