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Vogt-Koyanagi-Harada syndrome
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Vogt-Koyanagi-Harada syndrome

Contributors: Mehdi Rashighi MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Vogt-Koyanagi-Harada (VKH) syndrome is a rare multisystem inflammatory condition characterized by acute onset of headache, tinnitus, and hearing loss, which may be followed within a few days by acute bilateral uveitis. In its complete presentation, vitiligo, poliosis, or alopecia will develop within several weeks. It is important to remember that the skin features of the syndrome never precede the neurological, auditory, or ocular manifestations.

VKH syndrome results from a T-cell-mediated autoimmune response to melanocytes of central nervous system and skin in genetically susceptible individuals. The event that triggers this response remains unidentified. The syndrome is reported to occur more frequently among people of Asian, Native American, Hispanic, and Indian descent. The disease more commonly affects women during the third or fourth decade of life. Individuals carrying the HLA-DRB1*0405 allele are most at risk.

VKH syndrome presents in 4 clinically distinct phases:
  • Prodromal – Acute onset of fever, headache, nuchal rigidity, tinnitus, vertigo, and hearing loss, which typically last 3-5 days before complete resolution.
  • Uveitic – Within a few days, patient develops acute bilateral uveitis, exudative retinal detachment, and optic disc swelling, which present as visual loss, blurring, photophobia, and ocular pain.
  • Convalescent – Within several weeks, patient develops diffuse symmetric depigmentation of the choroids and poliosis, vitiligo, or alopecia that typically affects scalp, face, bilateral eyebrows, and eyelashes, which may last months to years.
  • Chronic recurrent – Within months to years, about two-thirds of patients progress to the chronic phase, which is characterized by recurrent episodes of anterior uveitis.
Pediatric Patient Considerations:
VKH syndrome more frequently affects those in their third or fourth decade; however, there have been reports of VKH syndrome in children.

Codes

ICD10CM:
H20.829 – Vogt-Koyanagi syndrome, unspecified eye

SNOMEDCT:
193497004 – Vogt-Koyanagi-Harada disease

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Vitiligo – Neurological or auditory deficits, if present, never precede the skin depigmentation.
  • Alezzandrini syndrome – Skin findings are typically unilateral (vitiligo does not cross the midline and may be associated with poliosis of ipsilateral eyelashes, eyebrow, or scalp hairs). Patients may complain of ipsilateral vision and/or hearing loss.
  • Piebaldism – Poliosis of forelock is typically associated with widespread, symmetrical depigmentation of central face, trunk, and extremities. No associated ocular or auditory abnormalities.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:05/18/2017
Last Updated:05/18/2017
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Vogt-Koyanagi-Harada syndrome
Vogt-Koyanagi-Harada syndrome : Blurred vision, Bilateral distribution, Eyelash color change, Optic disc edema, Patchy non-scarring alopecia, Hypopigmented patches, Hypopigmented macules
Clinical image of Vogt-Koyanagi-Harada syndrome
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