Document Detail


Infantile neurological Degos disease.
MedLine Citation:
PMID:  20659809     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Degos disease, or malignant atrophic papulosis, is a rare vasculopathy of uncertain aetiology manifesting as a primary dermatological disorder in most cases, but with widespread systemic involvement developing in an undefined proportion of patients. Reported neurological features of Degos disease include ischaemic and haemorrhagic stroke, subdural effusion, seizures, neuropathy, transverse myelitis, and optic atrophy. The description of contrast enhancement of the leptomeninges possibly indicates a defect of blood vessel integrity likely explaining the pleiotropic neurological manifestations. Degos disease is usually considered a disorder of adulthood, although a small number of infantile cases have been described. Here, we report a female who demonstrated a neonatal onset of Degos disease, eventually showing the highly characteristic skin lesions together with ptosis and a generalized weakness as part of her systemic disorder. Subsequent exacerbations led to an inexorable neurodevelopmental and physical decline. CT scan revealed intracranial calcification, a feature described in two previous cases. Our report highlights the need to consider Degos disease in the differential diagnosis of childhood neurological disease with skin involvement.
Authors:
Tong Hong Yeo; Grace Vassallo; Mary Judge; Nigel Laycock; Anna Kelsey; Yanick J Crow
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Publication Detail:
Type:  Journal Article     Date:  2010-07-24
Journal Detail:
Title:  European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society     Volume:  15     ISSN:  1532-2130     ISO Abbreviation:  Eur. J. Paediatr. Neurol.     Publication Date:  2011 Mar 
Date Detail:
Created Date:  2011-03-11     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9715169     Medline TA:  Eur J Paediatr Neurol     Country:  England    
Other Details:
Languages:  eng     Pagination:  167-70     Citation Subset:  IM    
Copyright Information:
Copyright © 2010 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
Affiliation:
Department of Paediatric Neurology, Royal Manchester Children's Hospital, Manchester M13 9WL, UK.
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