Uveomeningoencephalitic syndrome

Topic Completed: 1 February 2014

Minor changes: 20 February 2020

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PubMed Search: Uveomeningoencephalitic syndrome [title]

Nat Pernick, M.D.
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Cite this page: Pernick N. Uvea: uveomeningoencephalitic syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/eyeuveauveomeningoencephalicsyndrome.html. Accessed May 29th, 2020.
Definition / general
  • Also called Vogt-Koyanagi-Harada syndrome
  • Rare multisystemic autoimmune disease affecting eyes, meninges, ears and skin
  • Sudden onset of blindness, pleocytosis and temporary hearing loss; no history of eye trauma or surgery (by definition)
  • Blindness is due to severe bilateral panuveitis with serous retinal detachments
  • HLA-DRB1*0405 is present in chronic phase
Case reports
Microscopic (histologic) description
  • Chronic inflammation of choroid and iris, loss of choroidal melanocytes and dense chorioretinal adhesions
Microscopic (histologic) images

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Fig 1: marked lymphocytes in choroid (CH) adjacent to retinal pigment epithelium (arrowheads),
with vitreous body (VB) inside the neuronal retina (NR)
Fig 2: chorioretinal dense adhesions (DA); choroid separated from sclera (SC) by processing artifact
Fig 3: loss of choroidal melanocytes; retinal pigment epithelium (arrowheads)
Fig 4: moderate lymphocytes around sphincter muscle (SM)

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