Light and Electron Microscopic Observations in A Superficial Corneal Dystrophy

Reis-Bücklers Dystrophy

“…Three key histological features have been demonstrated in our study: (1) There is variability in staining of the subepithelial membrane. Early cases show more of a fibrinous change, especially notable with Movat's and reticulin stains.…”

Clinical Conditions Affecting The Basement Membrane of The Corneal Epithelium

“…Biomicroscopically, the cornea contains multiple minute discrete opacities just below the epithelium (Griffith and Fine, 1967;Yanoff and Fine, 1975).…”

“…Ultrastructurally, Bowman's layer is replaced slowly by scarring and proii'eration of collagenous tissue (Yanoff and Fine, 1975). There is an abrupt cessation of epithelial cell hemi-desmosomes and of the adjacent basement membrane (Griffith and Fine, 1967;Fogle, Green and Kenyon, 1974;Yanoff and Fine, 1975) and this appears to lead to recurrent desquamations or erosions of the epithelium with subsequent additional trauma to Bowman's layer (Yanoff and Fine, 1975). It was Kenyon (1969) who suggested that deficiencies in the formation of basement membrane hemi-desmosomes and attachment fibrils account for the erosive events encountered in Reis-Bucklers' dystrophy.…”

Groenouw's macular dystrophy: Histochemistry and ultrastructure of the cornea

“…: = Osmiophilic granules H : =-Histiocyte in Descemet dk : ~ Disrupted keratocyte DISCUSSION The macular corneal dystrophy, Groenouw, Type II, can be clearly defined as a disease originating from the stromal keratocytes. It differs from the Reis-Btickler disease (GRIEFITH et al, 1967, RICE et al, 1968, JONES & STAUFFER, 1970, nOCAN & WOOD, 1971, AKIYA & BROWN, 1971 where epithelial cytolysis and Bowman's membrane erosion are prominent, flom the lattice dystrophy, a localised amyloidosis (KUNTWORTH 1971) and Fuch's dystrophy involving primarily the endothelium (SCrIROEDER, 1972, MALBRAN 1972. A comparison between the pathological changes in these corneal dystrophies is summarised in Table II. The macular corneal dystrophy is considered to be a localised mucopolysaccharidosis involving a ~-Galactosidase deficiency (cogTIER et al, 1972)very different from the generalized, systemic Marateaux-Lamy syndrome, MPS VI, where a deficiency in ~3-Galactosidase activity provokes both intra-and extracellular storage material (KENYON 1972).…”