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Article
January 1985

Farber's DiseaseLight and Electron Microscopic Study of the Eye

Author Affiliations

From The Wilmer Ophthalmological Institute (Drs Zarbin, Green, and Morton), The John F. Kennedy Institute (Dr Moser), and the Departments of Pathology (Drs Zarbin, Green, and Morton), Neurology (Dr Moser), and Pediatrics (Dr Moser), Johns Hopkins Medical Institutions, Baltimore.

Arch Ophthalmol. 1985;103(1):73-80. doi:10.1001/archopht.1985.01050010077025
Abstract

• 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 μm 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.

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