Fuchs endothelial dystrophy

Topic Completed: 1 October 2013

Minor changes: 23 December 2020

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Nat Pernick, M.D.
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Cite this page: Pernick N. Fuchs endothelial dystrophy. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/eyecorneafuchsdystrophy.html. Accessed December 8th, 2021.
Definition / general
  • Type of primary endothelial dystrophy or endothelial decompensation
  • Most common endothelial dystrophy and common indication for corneal transplantation in US
  • Damage (decompensation) to endothelium, which normally pumps out fluid from corneal stroma, leads to chronic edema of stroma and epithelium, pain from rupture of epithelial bullae, eventually diffuse scarring and reduced vision
  • Women ages 50+
  • Some cases are autosomal dominant
  • Some cases are due to mutation in COL8A2 at 1p34.3-p32.3
Gross description
  • Bilateral
  • Often asymmetric
Microscopic (histologic) description
  • Corneal edema and cysts due to endothelial damage with paucity of endothelial cells and thickening of Descemet membrane
  • Epithelium is separated from Bowman layer
  • Papillary excrescences (guttae) in middle of cornea (must bisect cornea at equator and embed at equator to see)
  • If severe, may see edema of basal cells, bullae formation and pannus formation (fibrovascular ingrowth between Bowman layer and epithelium)
Differential diagnosis
  • Interstitial keratitis: inconspicuous ghost vessels in most posterior corneal stroma
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