Sneddon syndrome and the diagnostic value of skin biopsies - three young patients with intracerebral lesions and livedo racemosa.
Sneddon syndrome is a rare disorder characterised by generalised livedo racemosa of the skin with extracutaneous neurological symptoms like headache, vertigo, transient ischaemic attacks (TIA), stroke, and seizures. Diagnosis of Sneddon syndrome is based on these clinical features and positive findings in skin biopsies, namely the histological proof of occlusion of arterioles by intimal proliferation. We describe three cases of young patients with clinical characteristics of Sneddon syndrome, but in only two cases could this diagnosis be confirmed by skin biopsies. These cases stress the difficulty of diagnosing Sneddon syndrome and show the additive value of skin biopsies in this process.
Legierse
Catharina M
CM
Department of Dermatology, Academic Medical Center, Department of Dermatology P.O. Box 22700, 1100 DE Amsterdam, The Netherlands. c.m.legierse@amc.uva.nl
Canninga-Van Dijk
Marijke R
MR
Bruijnzeel-Koomen
Carla A F M
CA
Kuck-Koot
Veronica C M
VC
D000328:Adult; D017152:Antibodies, Antiphospholipid / Q000097:blood; D001158:Arteries / Q000473:pathology*; D001706:Biopsy / Q000379:methods; D002545:Brain Ischemia / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009131:Muscle, Smooth, Vascular / Q000473:pathology; D009422:Nervous System Diseases / Q000175:diagnosis*; D012307:Risk Factors; D012867:Skin / Q000098:blood supply / Q000473:pathology*; D017445:Skin Diseases, Vascular / Q000473:pathology*; D018860:Sneddon Syndrome / Q000097:blood / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed
D017152:Antibodies, Antiphospholipid