PT  - JOURNAL ARTICLE
AU  - Zoe Rutter-Locher
AU  - Zhongbo Chen
AU  - Lorena Flores
AU  - Tanya Basu
AU  - Daniel Creamer
AU  - Robert Weeks
AU  - Roopen Arya
AU  - Lina Nashef
TI  - Sneddon's syndrome: it is all in the ectoderm
AID  - 10.1136/practneurol-2015-001341
DP  - 2016 Aug 01
TA  - Practical Neurology
PG  - 300--303
VI  - 16
IP  - 4
4099  - http://pn.bmj.com/content/16/4/300.short
4100  - http://pn.bmj.com/content/16/4/300.full
SO  - Pract Neurol2016 Aug 01; 16
AB  - 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.