Sneddon syndrome and the diagnostic value of skin biopsies - three young patients with intracerebral lesions and livedo racemosa

Eur J Dermatol. 2008 May-Jun;18(3):322-8. doi: 10.1684/ejd.2008.0397. Epub 2008 May 13.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antibodies, Antiphospholipid / blood
  • Arteries / pathology*
  • Biopsy / methods
  • Brain Ischemia / diagnosis
  • Diagnosis, Differential
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Muscle, Smooth, Vascular / pathology
  • Nervous System Diseases / diagnosis*
  • Risk Factors
  • Skin / blood supply
  • Skin / pathology*
  • Skin Diseases, Vascular / pathology*
  • Sneddon Syndrome / blood
  • Sneddon Syndrome / diagnosis*
  • Tomography, X-Ray Computed

Substances

  • Antibodies, Antiphospholipid