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Biber-Haab-Dimmer degeneration

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A localised form of amyloidosis with onset early in life (20-30 years of age), affecting both sexes. It is manifested by progressive corneal opacity with distinct borders and a network of lattice-like branching filaments. Round spots may be scattered throughout the cornea, but the intervening spaces are clear, and there may be extracellular deposits of amyloid material. Visual function usually remains fairly satisfactory for long time. Aetiology unknown. Inheritance is autosomal dominant.

The terms Bücklers' syndrome II and Reis-Bücklers syndrome are misnomers. Reis-Bücklers syndrome is an annular corneal dystrophy. See under Heinrich Maria Wilhelm Reis, German ophthalmologist, born 1872.


  • H. Biber:
    Über einige seltenere Hornhauterkrankungen. Zürich, 1890.
    Inaugural Dissertation, Zurich, 1890. Cited by O. Haab: Die gittrige Keratitis.
  • O. Haab:
    Die gittrige Keratitis.
    Zeitschrift für Augenheilkunde, Basel, 1899, 2: 235-246.
  • F. Dimmer:
    Über oberflächliche gittrige Hornhauttrübung.
    Zeitschrift für Augenheilkunde, Basel, 1899, 2: 354.
  • S. J. Kimura,M. C. Wayne:
    Retinal Diseases. Philadelphia, Lea & Febiger, 1966.

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