Linear scleroderma 'en coup de sabre' associated with cerebral and ocular vasculitis

Scand J Rheumatol. Sep-Oct 2006;35(5):402-4. doi: 10.1080/03009740600556126.


Linear scleroderma 'en coup de sabre' (LSCS) has been reported in association with intracranial abnormalities. We report the case of an 11-year-old boy with LSCS who presented with recurrent headaches. Cranial magnetic resonance imaging (MRI) and angiography were consistent with the diagnosis of a cerebral vasculitis. In addition, retinal examination showed an exudative inflammatory lesion consistent with vasculitis.

Publication types

  • Case Reports

MeSH terms

  • Cerebral Angiography
  • Child
  • Headache / etiology
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Retinal Vasculitis / complications*
  • Retinal Vasculitis / diagnosis
  • Retinal Vasculitis / pathology
  • Scleroderma, Localized / complications*
  • Scleroderma, Localized / diagnosis
  • Scleroderma, Localized / pathology
  • Vasculitis, Central Nervous System / complications*
  • Vasculitis, Central Nervous System / diagnosis
  • Vasculitis, Central Nervous System / pathology