Reis-Bücklers Dystrophy

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Abstract
• Seven corneas from eight patients with Reis-Bücklers 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 third of the corneal stroma. Six of the seven patients examined by electron microscopy showed involvement of all three levels (subepithelial, Bowman's layer, and anterior stroma) by masses of peculiar "curly" filaments with a diameter of about 100 A and of indeterminate length. The curly configuration seems to be due to the sectioning of longer wavy filaments. The electron microscopic appearance of these peculiar curly filaments seems to be highly characteristic of Reis-Bücklers corneal dystrophy and has not been observed in any other corneal dystrophy.

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