Craniofacial linear scleroderma associated with retinal telangiectasia and exudative retinal detachment

J AAPOS. 2017 Jun;21(3):251-254. doi: 10.1016/j.jaapos.2016.12.004. Epub 2017 May 18.

Abstract

Linear scleroderma is a characteristic form of scleroderma that typically affects children. Ocular manifestations may be present, especially when the frontoparietal area of the head is affected. We present the case of a 5-year-old boy with craniofacial linear scleroderma ("en coup de sabre") who developed exudative retinal detachment. Angiographic and neuroimaging findings are presented, and the importance of regular fundus examination is highlighted.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Drug Therapy, Combination
  • Enzyme Inhibitors / therapeutic use
  • Exudates and Transudates
  • Facial Dermatoses / complications*
  • Facial Dermatoses / diagnosis
  • Fluorescein Angiography
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Magnetic Resonance Imaging
  • Male
  • Methotrexate / therapeutic use
  • Methylprednisolone / therapeutic use
  • Mycophenolic Acid / therapeutic use
  • Retinal Detachment / diagnosis
  • Retinal Detachment / etiology*
  • Retinal Telangiectasis / diagnosis
  • Retinal Telangiectasis / etiology*
  • Scalp Dermatoses / complications*
  • Scalp Dermatoses / diagnosis
  • Scleroderma, Localized / complications*
  • Scleroderma, Localized / diagnosis

Substances

  • Enzyme Inhibitors
  • Glucocorticoids
  • Immunosuppressive Agents
  • Mycophenolic Acid
  • Methylprednisolone
  • Methotrexate