Central cloudy corneal dystrophy of Francois. A clinicopathologic study
C. L. Karp, I. U. Scott, W. R. Green, T. S. Chang and W. W. Culbertson
Department of Ophthalmology, Bassom Palmer Eye Institute, University of Miami School of Medicine, Fla., USA.
Central cloudy corneal dystrophy of Francois was first described in 1955 by
J. Francois; its pathophysiology remains unknown. An 80-year-old woman with
bilateral central cloudy corneal dystrophy of Francois was examined after
having undergone a combined penetrating keratoplasty and cataract
extraction. The corneal button was obtained. Light microscopy revealed
stromal staining for acid mucopolysaccharide. Transmission electron
microscopy revealed extracellular vacuoles, some of which had
fibrillogranular material and electron-dense deposits. Fibrillogranular
material was present in and around some keratocytes. Numerous endothelial
vacuoles contained light-staining fibrillogranular material and round
electron-dense granules. Our findings suggest that the opacities in
patients with central cloudy corneal dystrophy of Francois are due to the
extracellular accumulation of mucopolysaccharide and lipidlike material.
Further studies are needed to elucidate the nature of these deposits.
