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Primary orbital lymphomatoid granulomatosis

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Lymphomatoid granulomatosis (LYG) is a rare angiocentric and angiodestructive Epstein–Barr virus (EBV)-associated B cell lymphoproliferative disorder that involves multiple organs including the lung, skin, kidney and central nervous system. Ocular LYG is an extremely rare tumour, which appears as ocular granulomatous inflammation (conjunctival infiltration,1 posterior uveitis,2 choroidal vasculitis,3 optic neuropathy,4 and retinal detachment5), with only a few reports in the medical literature. Furthermore, there are no reports of primary orbital LYG without pulmonary LYG. We describe the first case of primary orbital LYG diagnosed by biopsy of an orbital tumour.


A 54-year-old man presenting with a 6-week history of painless proptosis and ptosis of the right eye was referred to our outpatient clinic. Eye examination revealed marginal blepharitis on the right eye (fig 1A), and the right eye movement was restricted in all directions. Funduscopy showed severe disc swelling in the right eye (fig 1A). His best-corrected visual acuity (BCVA) …

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  • Funding: None.

  • Competing interests: None.

  • Ethics approval: Ethics approval was provided by the Institutional Review Board of Tokyo University Hospital.

  • Patient consent: Obtained.

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