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Reis-Bücklers dystrophy

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A peculiar familial form of a bilateral subepithelial corneal dystrophy, characterized by annular grayish lesions of the Bowman membrane with secondary involvement of the epithelium and superficial part of the stroma. Infrequent episodes of eye pain, reduction of vision. Onset variable age from 8 to 20 years. Increased severity after 40 years of age. Inheritance is autosomal dominant. First described by Reis on the basis of a single case.

Grayson-Wilbrandt syndrome is a variant with variable effect on vision, partial corneal involvement, and normal corneal sensitivity.

Bücklers and Reis-Bücklers syndrome has been used as a synonym for Biber-Haab-Dimmer degeneration, which is an inheritable corneal lattice dystrophy. See under Hugo Biber, Swiss ophthalmologist, 1864-1918.

See also Thiel-Behnke dystrophy, or honeycomb dystrophy of the cornea, under Hans-Jürgen Thiel, German ophthalmologist.


  • W. Reis:
    Familiäre, fleckige Hornhautentartung.
    Deutsche Medizinische Wochenschrift, Berlin, 1917, 43: 575.
  • M. Bücklers:
    Über eine weitere familiäre Hornhautdystrophie (Reis).
    Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1949, 114: 386-397.
  • M. Grayson, H. Wilbrandt:
    Dystrophy of the anterior limiting membrane of the cornea (Reis-Bücklers type).
    American Journal of Ophthalmology, Chicago, 1966, 61: 345-349.

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