Farber's Disease: Light and Electron Microscopic Study of the Eye

Marco A. Zarbin, W. Richard Green, Sebastian J. Morton, Hugo W. Moser

Research output: Contribution to journalArticlepeer-review

21 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)73-80
Number of pages8
JournalArchives of Ophthalmology
Volume103
Issue number1
DOIs
StatePublished - Jan 1985
Externally publishedYes

ASJC Scopus subject areas

  • Ophthalmology

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