Case Study: December 2010

History

  • A 35 year old male presents with bilateral corneal opacities
  • Examination shows a honeycomb-type dystrophy
  • A PK is performed
  • Figure 1 is the H&E
  • Figure 2 is a Masson trichrome
  • Figure 3 is a transmission electron micrograph of the pathology shown in
    Figures 1 and 2

Questions

  1. What are the key features on the H&E?
  2. What does the Masson trichrome show?
  3. What does the electron micrograph display?
  4. What is the likeliest diagnosis?
  5. What is the H&E differential diagnosis?
Click to show answers
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  1. Sub-epithelial eosinophilic diffuse deposit with some in the anterior stroma, with epithelial atrophy
  2. Masson’s trichrome positive deposits matching the distribution on the H&E
  3. Electron dense rhomboidal deposits
  4. This is a good example of Reiss Buckler dystrophy (BIG-H3 family), which is essentially an anterior variant of granular dystrophy
  5. The main H&E differential is Thiel Behnke dystrophy, secondary amyloid deposits and primary gelatinous drop-like dystrophy