Reis-Bücklers dystrophy: a study of eight cases

Perry, H.D.; Fine, B.S.; Caldwell, D.R.

Archives of Ophthalmology 97(4): 664-670


ISSN/ISBN: 0003-9950
PMID: 85445
DOI: 10.1001/archopht.1979.01020010320007
Accession: 068520071

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Seven corneas from 8 patients with Reis-Bucklers dystrophy were examined by light and electron microscopy. In all cases the now characteristic changes of degeneration of Bowman's membrane and the development of an irregular subepithelial membrane were found. Four cases also had changes limited to the anterior 3rd of the corneal stroma. Six of the 7 patients examined by EM showed involvement of all three levels (subepithelial, Bowman's layer, and anterior stroma) by masses of peculiar curley filaments with a diameter of about 100 .ANG. and of indeterminate length. The curly configuration seems to be due to the sectioning of longer wavy filaments. The EM appearance of these peculiar curly filaments seems to be highly characteristic of Reis-Bucklers corneal dystrophy and has not been observed in any other corneal dystrophy.