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Behnke-Thiel dystrophy

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A slowly progressive disease of the anterior basement membrane that leads to scarring, pain, and photophobia. There are subepithelial corneal opacities with a clear limbal zone, and a honeycomb-shaped opacity pattern, Previously this form of corneal dystrophy has erroneously been classified as Reis-Bücklers' dystrophy. The clinical symptoms hardly differ from those of Reis-Bücklers' dystrophy, but the histological appearance is clearly distinct.

Inheritance is autosomal dominant. In 1965 Behnke and Thiel described a form of juvenile epithelial corneal dystrophy and showed that all cases in Schleswig-Holstein were members of one kindred traced back to 1620. In the 4 living generations, 120 cases were demonstrated. Thiel-Behnke is now considered separate entity and a variant of Reis-Bücklers.

Bibliography

  • H. Behnke, H. J. Thiel:
    Ueber die hereditäre Epitheldystrophie der Hornhaut (Typ Meesman-Wilke) in Schleswig-Holstein.
    Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1965, 147: 662-672.
  • R.W. Yee, L.S. Sullivan, H.T. Lai, E.L. Stock, Y. Lu, M.N. Kahn, S. P. Daiger:
    Linkage mapping of Thiel-Behnke corneal dystrophy (CDTB) to chromosome 10q23-q24.
    Genomics, San Diego, 1997, 46: 152-154.
  • Egon G Weidle:
    Honeycomb-shaped corneal dystrophy of Thiel and Behnke. Reclassification and distinction from Reis-Bücklers' corneal dystrophy.
    Augenheilkunde, 1999, 214 (3): 125-135.

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