Fleck corneal dystrophy
J. J. Purcell Jr, J. H. Krachmer and T. A. Weingeist
Fourteen members of four families with fleck systrophy of the corneal
stroma were examined and studied. Corneal and lens changes were noted in
all patients. Corneal sensation was normal. Corneal biopsies were performed
on two patients and a corneal button of a patient with fleck dystrophy and
keratoconus was studied. Light and electron microscopy and histochemical
studies showed this dystrophy to consist of abnormal keratocytes with
variable numbers of membrane-limited intracytoplasmic vacuoles containing a
granular to fibrogranular material that stains positively for
mucopolysaccharide. This appears to be the first reported incidence of a
dominantly inherited disorder of mucopolysaccharides affecting the eyes.
