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Vogt-Koyanagi-Harada Syndrome

Research suggests autoimmune disorder

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Updated April 08, 2006

Vogt-Koyanagi-Harada syndrome (VKH) syndrome has neurological, eye, ear, and skin symptoms. Research suggests it may be due to an autoimmune reaction in which the body attacks its own healthy cells that contain the pigment melanin. VKH syndrome does not shorten life span, but permanent eye and skin changes may result.

It is not known how often Vogt-Koyanagi-Harada syndrome occurs throughout the world. It occurs more frequently in people of Asian, Native American, Latin American, or Middle Eastern heritage. Published reports of VKH syndrome indicate that it occurs more frequently in women and than in men, and its symptoms may begin at any age.

Symptoms
Before Vogt-Koyanagi-Harada syndrome begins, individuals usually experience symptoms such as headache, vertigo, nausea, stiff neck, vomiting, and low-grade fever for several days. These symptoms are not specific to VKH syndrome and may be diagnosed as a viral infection or influenza. What distinguishes VKH syndrome from “the flu” is the start of eye symptoms such as sudden blurry vision, pain, and sensitivity to light. Typically, VKH syndrome consists of three phases: a meningoencephalitis phase, an ophthalmic-auditory phase, and a convalescent phase.

In the meningoencephalitis phase, symptoms such as generalized muscle weakness, headache, loss of muscle use on one side of the body (hemiparesis or hemiplegia), joint pain (dysarthria), and difficulty speaking or understanding language (aphasia) occur.

In the ophthalmic-auditory phase, symptoms such as blurry vision, pain, and eye irritation due to inflammation of the iris (iridocyclitis) and uvea (uveitis) occur. Auditory symptoms may include difficulty hearing, ringing in the ear (tinnitus), or dizziness.

In the convalescent phase, skins symptoms such as light or white patches of color in the hair, eyebrows, or eyelashes (poliosis), light or white patches of skin (vitiligo), and hair loss (alopecia) appear. The skin symptoms usually begin several weeks or months after the vision and hearing symptoms start.

Diagnosis
Because Vogt-Koyanagi-Harada syndrome is rare, a correct diagnosis usually requires consultation with specialists. There is no specific test for the syndrome, so diagnosis is based on the symptoms present plus test results. A neurologist will perform a lumbar puncture to examine the cerebrospinal fluid (CSF) for changes characteristic of VKH syndrome. An ophthalmologist will perform special testing of the eyes to look for uveitis. A dermatologist will take a skin sample (biopsy) about one month after the eye symptoms begin to check for changes characteristic of VKH syndrome, such as lack of pigment (melanin) in light or white patches of skin that are present.

The American Uveitis Society recommends that 3 of these 4 criteria be met to confirm the diagnosis of Vogt-Koyanagi-Harada syndrome:

  • iridocyclitis in both eyes, and uveitis
  • neurological symptoms or characteristic changes in the CSF
  • skin symptoms of poliosis, vitiligo, or alopecia

Treatment
To reduce the inflammation in the eyes, corticosteroids such as prednisone are given. If this does not work well, immunosuppressant drugs such as azathioprine (Imuran) or cyclophosphamide (Cytoxan, Neosar) may be used. Skin symptoms are treated the way vitiligo is treated, which may include phototherapy, corticosteroids, or medicated ointments.

Early diagnosis and treatment of Vogt-Koyanagi-Harada syndrome helps prevent permanent vision changes such as glaucoma and cataracts. Skin changes may be permanent, even with treatment, but hearing is usually restored in most individuals.

Last updated 4/8/06

Sources:
Choczaj-Kukula, A. (2005). Vogt-Koyanagi-Harada syndrome. eMedicine, accessed at http://www.emedicine.com/derm/topic739.htm
Read, R. W. (2003). American Uveitis Society: Vogt-Koyanagi-Harada Disease.

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