Farber's disease. Light and electron microscopic study of the eye
M. A. Zarbin, W. R. Green, H. W. Moser and S. J. Morton
A 35-month-old girl had Farber's disease (disseminated lipogranulomatosis)
manifested clinically by macular cherry-red spots. The pathologic changes
consisted of intracellular inclusions of varying morphologic features and
density. The most frequently encountered inclusion was 1.2 micron wide and
consisted of flattened stacks of osmophilic lamellae (2.1 to 2.3 nm thick,
with 4.4-nm periodicity) oriented in parallel or oblique array
("zebra-body" configuration) and enclosed by a focally discontinuous unit
membrane. Some of the inclusions contained curved tubular profiles
resembling curvilinear tubular bodies. The retinal ganglion cells were
grossly distended with inclusions and showed the greatest pathologic
changes.