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Sneddon's syndrome: it is all in the ectoderm
  1. Zoe Rutter-Locher1,
  2. Zhongbo Chen1,
  3. Lorena Flores1,
  4. Tanya Basu2,
  5. Daniel Creamer2,
  6. Robert Weeks1,
  7. Roopen Arya3,
  8. Lina Nashef1
  1. 1Department of Neurology, King's College Hospital, London, UK
  2. 2Department of Dermatology, King's College Hospital, London, UK
  3. 3Department of Haematology, King's College Hospital, London, UK
  1. Correspondence to Dr Zoe Rutter-Locher, Department of Neurology, King's College Hospital, Denmark Hill, London SE5 9RS, UK; Zrutter-locher{at}nhs.net

Abstract

A 51-year-old man gave a 2-year history of worsening mobility, cognitive decline and headaches. He had a history of thromboembolic stroke, recurrent transient ischaemic attacks and a spontaneous intraventricular haemorrhage. On examination, he had livedo reticularis and perniosis and a systolic murmur. Catheter cerebral angiography showed peripheral small-vessel and medium-vessel vasculopathy resulting in pruning of the distal cortical vessels and tortuous irregular distal collaterals. Skin biopsy showed subtle vasculopathy with ectasia of capillaries and postcapillary venules but no frank vasculitis or arterial thrombosis. Repeated serum antiphospholipid antibody titres were negative. The clinical features, skin biopsy and angiogram findings strongly supported a diagnosis of Sneddon's syndrome. Clinicians should consider Sneddon's syndrome in patients with livedo reticularis and stroke. There are treatment dilemmas in this situation when ischaemic and haemorrhagic cerebral events coexist.

  • STROKE
  • CEREBROVASCULAR DISEASE
  • RHEUMATOLOGY
  • VASCULITIS
  • HAEMATOLOGY

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