title: Sneddon's syndrome: it is all in the ectoderm.
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.
authors.affiliation: Department of Neurology, King's College Hospital, London, UK.
authors.forename: Zoe
authors.initials: Z
authors.lastname: Rutter-Locher
authors.affiliation: Department of Neurology, King's College Hospital, London, UK.
authors.forename: Zhongbo
authors.initials: Z
authors.lastname: Chen
authors.affiliation: Department of Neurology, King's College Hospital, London, UK.
authors.forename: Lorena
authors.initials: L
authors.lastname: Flores
authors.affiliation: Department of Dermatology, King's College Hospital, London, UK.
authors.forename: Tanya
authors.initials: T
authors.lastname: Basu
authors.affiliation: Department of Dermatology, King's College Hospital, London, UK.
authors.forename: Daniel
authors.initials: D
authors.lastname: Creamer
authors.affiliation: Department of Neurology, King's College Hospital, London, UK.
authors.forename: Robert
authors.initials: R
authors.lastname: Weeks
authors.affiliation: Department of Haematology, King's College Hospital, London, UK.
authors.forename: Roopen
authors.initials: R
authors.lastname: Arya
authors.affiliation: Department of Neurology, King's College Hospital, London, UK.
authors.forename: Lina
authors.initials: L
authors.lastname: Nashef
mesh_terms: D002533:Cerebral Angiography; D002543:Cerebral Hemorrhage / Q000175:diagnosis*; D004475:Ectoderm / Q000473:pathology*; D006801:Humans; D002546:Ischemic Attack, Transient; D008297:Male; D008875:Middle Aged; D018860:Sneddon Syndrome / Q000150:complications / Q000175:diagnosis*
keywords: CEREBROVASCULAR DISEASE; HAEMATOLOGY; RHEUMATOLOGY; STROKE; VASCULITIS