Sneddon syndrome

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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.

Clinical presentation

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

Pathology

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.

Radiographic features

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.

Differential diagnoses

  • 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

Treatment and prognosis

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