RT Journal Article
SR Electronic
T1 Sneddon's syndrome: it is all in the ectoderm
JF Practical Neurology
JO Pract Neurol
FD BMJ Publishing Group Ltd
SP 300
OP 303
DO 10.1136/practneurol-2015-001341
VO 16
IS 4
A1 Rutter-Locher, Zoe
A1 Chen, Zhongbo
A1 Flores, Lorena
A1 Basu, Tanya
A1 Creamer, Daniel
A1 Weeks, Robert
A1 Arya, Roopen
A1 Nashef, Lina
YR 2016
UL http://pn.bmj.com/content/16/4/300.abstract
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.