Criteria:
(1) no history of penetrating ocular trauma or surgery preceding the initial onset of the uveitis
(2) no clinical or laboratory evidence suggestive of other ocular disease entities
(3) bilateral ocular involvement, with findings dependent on stage of disease when the patient is examined (see below)
(4) history or presence of neurological and/or auditory findings (see below)
(5) integumentary findings occurring concurrent or after (not preceding) the onset of CNS or ocular disease (see below)
Ocular involvement, early:
(3E1) diffuse chorioditis (with or without anterior uveitis, vitreous inflammatory reaction or optic disc hyperemia), which may be manifested by one or both of the following:
(3E1a) focal areas of subretinal fluid, or
(3E1b) bullous serous retinal detachments
(3E2) with equivocal fundus findings,
both
of the following must be present as well
(3E2a) fluorescent angiography shows focal areas of delay in choroidal perfusion, multifocal areas of pinpoint leakage, large placoid areas of hyperfluorescence pooling within subretinal fluid, and optic nerve staining (in order of sequential appearance)
(3E2b) diffuse choroidal thickening, without evidence of posterior scleritis by ultrasonography
Ocular involvement, late:
(3L1) history suggestive of prior presence of early ocular involvement, both 3L2 and 3L3, or multiple signs from 3L3
(3L2) ocular depigmentation, one or both of the following
(3L2a) sunset glow fundus (pale choroidal pigmentation)
(3L2b) Sugiura sign (perilimbal vitiligo)
(3L3) other ocular signs
(3L3a) nummular chorioretinal depigmented scars
(3L3b) retinal pigment epithelium clumping and/or migration
(3L3c) recurrent or chronic anterior uveitis
Neurological and/or auditory findings - one or more of the following:
(4a) meningismus (combination of malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back; headache alone is insufficient)
(4b) tinnitus
(4c) pleocytosis in the cerebrospinal fluid
Integumentary findings - one or more of the following:
(5a) alopecia
(5b) poliosis (premature graying of the hair)
(5c) vitiligo
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Criteria Present
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Diagnosis
|
|
1, 2, 3, 4, and 5
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complete VKH disease
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|
1, 2, 3 and (4 or 5)
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incomplete VKH disease
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1, 2, and 3
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probable VKH disease
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else
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not VKH disease
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