Sneddon syndrome is a type of systemic non-inflammatory vasculopathy characterised by livedo reticularis and progressive and occlusive cerebrovascular thrombosis involving the medium-sized arteries.
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Epidemiology
Sneddon syndrome is more common in females, and tends to affect a young adult population between the ages of 20-42 years 4.
Clinical presentation
- livedo reticularis and livedo racemosa 4
- progressive ischaemic strokes, and less commonly intracerebral haemorrhage 4
- cognitive impairment 4
Pathology
It is characterised by intimal hyperplasia of medium-sized arteries with unknown aetiology. The condition is usually sporadic, with at least half of the patients also have antiphospholipid syndrome, however familial forms have also been described 4. Skin biopsy is diagnostic 4.
Radiographic features
MRI typically shows non-specific multi-territory infarcts and cortical atrophy, however, less commonly may also show haemorrhagic changes such as cerebral microhaemorrhages 4,5. 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, severe leukoaraiosis and juvenile stroke
- cerebral angiography 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.
History and etymology
The condition is named after Ian Bruce Sneddon (1915–1987), a British dermatologist who published a seminal case series describing the condition in 1965 6.