Sneddon syndrome
Hakima Elmahi, Fatima Zahra mernissi
The Pan African Medical Journal. 2019;34:9. doi:10.11604/pamj.2019.34.9.11903

Innovations in Measles Elimination Innovations in Measles Elimination
"Better health through knowledge sharing and information dissemination "

Images in medicine

Sneddon syndrome

Cite this: The Pan African Medical Journal. 2019;34:9. doi:10.11604/pamj.2019.34.9.11903

Received: 05/02/2017 - Accepted: 07/03/2017 - Published: 04/09/2019

Key words: Sneddon syndrome, skin disease, antiphospholipid antibodies

© Hakima Elmahi et al. The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Available online at: http://www.panafrican-med-journal.com/content/article/34/9/full

Corresponding author: Hakima Elmahi, Department of Dermatology, University Hospital Hassan II, Fez, Morocco (elmahi.hakima@gmail.com)


Sneddon syndrome

Hakima Elmahi1,&, Fatima Zahra Mernissi1

 

1Department of Dermatology, University Hospital Hassan II, Fez, Morocco

 

 

&Corresponding author
Hakima Elmahi, Department of Dermatology, University Hospital Hassan II, Fez, Morocco

 

 

Image in medicine    Down

Sneddon's syndrome (SS) is a rare non-inflammatory thrombotic vasculopathy characterized by the combination of cerebrovascular disease with livedo racemosa(LR). It has been estimated that the incidence of SS is 4 per 1 million per annum in general population and generally occurs in women between the ages of 20 and 42 years. Approximately 80% of Sneddon's syndrome patients have an antiphospholipid antibody marker. LR may precede the onset of stroke by years and the trunk and/or buttocks are involved in nearly all patients. The cerebrovascular manifestations are mostly secondary to ischemia (transient ischemic attacks and cerebral infarct). Other neurological symptoms range from headache, cerebral hemorrhage, seizures, cognitive and psychiatric disturbances. The involved internal organs include heart, kidney, and eyes. Histological findings of skin are characteristic and the involved vessels are small to medium-sized arteries at the border of dermis to subcutis with a distinct histopathological time course. The main diagnostic criteria are general LR with typical histopathological findings on skin biopsy and focal neurological deficits. The pathogenesis is related to hypercoagulable state and intrinsic small-vessel vasculopathy. The optimal management remains an unsolved problem and long-term anticoagulation have been recommended for cerebral ischemic events based on the presumed pathogenesis. There are controversial results in treatment of SS with immunomodulatory agents. We reported a case of a 49-year old women patient with recurrent ischemic cerebrovascular accidents as the first manifestation, accompanied by livedo reticularis in trunk and limbs and mitral valvulopathy without antiphospholipid antibody.

 

 

Figure 1: A) livedo reticularis in trunk and limbs; B) livedo reticularis in arms

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 


The Pan African Medical Journal articles are archived on Pubmed Central. Access PAMJ archives on PMC here

Volume 34 (September - December 2019)

Article tools

PDF (315 Kb)
Contact the corresponding author
Download to Citation Manager
EndNote
Reference Manager
Zotero
BibTex
ProCite


This article authors

On Pubmed
On Google Scholar

Navigate this article

Keywords

Sneddon syndrome
Skin disease
Antiphospholipid antibodies

Rate this article

Altmetric

PAMJ is a member of the Committee on Publication Ethics
PAMJ Authors services
Next abstract

PAMJ is published in collaboration with the African Field Epidemiology Network (AFENET)
Currently tracked by: DOAJ, AIM, Google Scholar, AJOL, EBSCO, Scopus, Embase, IC, HINARI, Global Health, PubMed Central, PubMed/Medline, Ulrichsweb, More to come . Member of COPE.

ISSN: 1937-8688. © 2019 - Pan African Medical Journal. All rights reserved