Scleroderma en coup de sabre with central nervous system and ophthalmologic involvement: treatment of ocular symptoms with interferon gamma

J Am Acad Dermatol. 2003 Sep;49(3):543-6. doi: 10.1067/s0190-9622(03)00901-0.


Scleroderma en coup de sabre, a variant of localized scleroderma, is a disorder of unknown origin characterized by fibrosis of connective tissue. Rare complications of scleroderma en coup de sabre are orbital and intracerebral involvement. We describe a patient with scleroderma en coup de sabre in whom intracerebral and orbital lesions developed after 2 decades of disease duration. Clinically, she had epilepsy, impaired vision, and retro-ocular pain of the affected eye. A 12-month course of interferon-gamma stopped progression of visual symptoms caused by orbital fibrous tissue. To our knowledge, this is the first patient with scleroderma en coup de sabre complicated by orbital involvement who was successfully treated with interferon-gamma.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Brain Diseases / complications*
  • Brain Diseases / diagnosis
  • Brain Diseases / drug therapy
  • Drug Administration Schedule
  • Epilepsy / complications
  • Epilepsy / diagnosis
  • Epilepsy / drug therapy
  • Eye Diseases / complications*
  • Eye Diseases / diagnosis
  • Eye Diseases / drug therapy*
  • Female
  • Follow-Up Studies
  • Humans
  • Interferon-gamma / therapeutic use*
  • Long-Term Care
  • Magnetic Resonance Imaging
  • Risk Assessment
  • Scleroderma, Localized / complications*
  • Scleroderma, Localized / diagnosis
  • Scleroderma, Localized / drug therapy*
  • Severity of Illness Index
  • Treatment Outcome
  • Visual Acuity


  • Interferon-gamma