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Sneddon syndrome

Dr Henry Knipe and Dr M Venkatesh et al.

Sneddon syndrome is a type of systemic non-inflammatory vasculopathy characterized by livedo reticularis and progressive and occlusive cerebrovascular thrombosis involving the medium-sized arteries. 

  • livedo reticularis as a skin manifestation 
  • progressive cerebral strokes and hemorrhage
  • cerebral dysfunction

It is characterized by intimal hyperplasia of medium-sized arteries with unknown etiology. Half of the patients also have antiphospholipid syndrome. Skin biopsy is diagnostic. 

CT and MRI show non-specific infarcts and cortical atrophy. Angiography shows stenosis or occlusion of medium-sized arteries and large networks of collateral small vessels.

  • Divry van Bogaert syndrome
    • juvenile-onset of progressive cognitive impairment / juvenile dementia, severe leukoaraiosis / juvenile stroke
    • cerebral angiogram shows extensive, multifocal occlusions of peripheral small and medium-sized cerebral arteries and a network of small corkscrew-like collaterals

There is no effective treatment to control disease progression but long-term antiplatelet therapy and anticoagulation are commonly used in cases with cerebral manifestations. 

Article information

rID: 25503
Section: Syndromes
Tag: cases, cases
Synonyms or Alternate Spellings:

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