Vogt-Koyanagi-Harada (VKH) Syndrome

This is a rare syndrome most commonly affecting Orientals (esp. Japanese), Hispanics, Indians, and Native Americans. It is rare in white races. Originally, it was described as two separate diseases, Vogt-Koyanagi syndrome and Harada syndrome, but they overlap in so many respects that they are now combined as Vogt-Koyanagi-Harada syndrome.

Ocular symptoms

Four phases:

Prodromal. May start with flu-like symptoms (fever, headache, nausea). Within 1-2 days, blurring of vision, sensitivity to light, red painful eyes, visual distortion of shapes. There may also be sensitivity of the skin and hair to touch
Uveitis. This may only be detected by an ophthalmologist, who may see signs of iritis (cells) and choroiditis (bilateral exudative retinal detachments)
Chronic. After treatment with oral steroids, inflammation subsides
Recurrent. The uveitis in VKH often recurs or becomes chronic

Symptoms outside the eye

Hearing deficiency. This is due to inner ear dysfunction. Some patients complain of vertigo and tinnitus. The hearing loss is usually in the high frequency ranges. The hearing loss usually returns to normal after several weeks
Neurological symptoms. Fever, headache, nausea, vomiting.
Skin symptoms. Vitiligo (white patches on the skin) are common on the face (esp near eyelashes), hands, shoulder, breast, and the low back.
Hair symptoms. Whitened hair (poliosis) and loss of hair (alopecia) often occurs

In practice, certain sets of symptoms tend to go together:

Vogt-Koyanagi syndrome: iritis, alopecia, vitiligo, dysacousia
Harada syndrome: bilateral exudative uveitis, pleocytosis of cerebospinal fluid


Fundus angiography in the early exudative phase shows slow leakage of dye through the retinal pigment epithelium
Lumbar puncture (spinal tap) and cerebospinal fluid analysis shows an increase in the number of lymphocytes (pleocytosis)


Intravenous steroids in the early active stage is effective in improving the vision.
Oral steroids (eg. Prednisolone) after a few days of intravenous steroids. They may be continued in a reducing dose for 2-3 months until the uveitis has been controlled
Steroid eye drops (eg. Prednisolone {PredForte] or Dexamethasone [Maxidex])


The long-term use of oral and topial steroids may cause cataracts, which can be removed.
Glaucoma may develop and occasional pressure checks may be necessary

Cause and Pathology

The cause is unknown.  The disease is associated with the inherited blood marker HLA-B22. Under the microscope, the disease is basically the same as sympathetic uveitis, with granulomatous inflammation throughout the uveal tissues. Granulomatous inflammation is a type of inflammation in which there are large numbers of infiltrating immune cells called lymphocytes, macrophages, and epithelioid cells.

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