Reis-Bucklers corneal dystrophy


Reis-Bucklers corneal dystrophy
Reis-Bucklers corneal dystrophy
Classification and external resources

Reis-Bücklers corneal dystrophy. Reticular opacity in the superficial cornea
OMIM 608470

Reis-Bucklers corneal dystrophy (or Reis-Bücklers corneal dystrophy), also known as Corneal dystrophy of Bowman layer, type I, is a rare form of human corneal dystrophy in which the Bowman's layer undergoes disintegration. The disease has been associated with mutations in TGFBI gene. Symmetrical reticular opacities, usually appearing bilaterally in the upper cornea by the age of 4 or 5 years, elevate the corneal epithelium, leading eventually to its erosions that prompt attacks of ocular hyperemia, pain, and photophobia.

The dystrophy was described in 1917 by Reis[1] and in 1949 by Bucklers.[2]

Reis-Bücklers corneal dystrophy. Light microscopy of cornea showing characteristic red stained deposits of mutated transforming growth factor beta induced protein in the superficial corneal stroma. Masson's trichrome stain.

See also

References

  1. ^ Reis W: Fämiliare, fleckige Hornhautetartung. Dtsch Med Wochenschr 1917, 43:575.
  2. ^ Bücklers M: Über eine weitere familiare Hornhautdystrophie (Reis). Klin Monatsbl Augenheilkd 1949, 114:386-397.

External links



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