Patterned dystrophies of the retinal pigment epithelium
R. C. Hsieh, B. S. Fine and J. S. Lyons
Three varieties of patterned dystrophies of the retinal pigment epithelium
have been described in the literature: They are reticular dystrophy,
macroreticular dystrophy, and butterfly-shaped pigment dystrophy of the
fovea (macula). They are all inherited and are bilateral and symmetric.
Results of retinal-function tests in all three varieties were essentially
normal, except for the electro-oculogram in the butterfly-shaped pigment
dystrophy. We have observed within a single family one member manifesting
butterfly-shaped pigment dystrophy, one member with macroreticular
dystrophy, and one member with probable reticular dystrophy.
Electrophysiologic studies of the retina in these patients were in accord
with those already reported, except for supernormal scotopic responses in
the latter two patients. This family demonstrates the linkage necessary to
tie together a number of entities heretofore considered as separate.
