An Overview of Vogt-Koyanagi-Harada Syndrome

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Vogt-Koyanagi-Harada (VKH) syndrome is a rare disorder that affects many parts of the body, but specifically the brain, eyes, ears, and skin. The most common symptom is loss of sight that comes on quickly.

Although the cause of VKH syndrome is unknown, research suggests it may be due to an autoimmune reaction in which the body attacks its own healthy cells that contain the pigment melanin.

Because some symptoms are similar to those of viral diseases, such as the flu, VKH may be caused by a virus, although no virus has yet been identified. Genetics may also play a role in VKH syndrome, which does not shorten your lifespan but may cause permanent damage to the eyes and skin.

It is not known how often Vogt-Koyanagi-Harada syndrome occurs throughout the world, but it's believed to exist in about 1.5–6 people out of 1 million in the United States. It is most common 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 than in men (a 2-to-1 ratio). Its symptoms may begin at any age but most commonly appear in people in their 40s.

Symptoms

Before the onset of VKH syndrome, you may experience symptoms such as headache, vertigo (a dizzy sensation), 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 (the flu).

What distinguishes VKH syndrome from viruses like the flu is the sudden onset of eye symptoms such as 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.

Meningoencephalitis phase

This phase affects the meninges (the three layers of membranes that protect the brain and spinal cord) and causes inflammation of the brain tissues (encephalitis). In this phase, symptoms such as generalized muscle weakness, headache, loss of muscle use on one side of the body, joint pain, and difficulty speaking or understanding language occur.

Ophthalmic-Auditory Phase

In the ophthalmic-auditory phase, which affects the eyes and ears, 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 ears (tinnitus), or dizziness.

Convalescent Phase

In the convalescent phase, you may notice skin 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). The skin symptoms usually begin several weeks or months after the vision and hearing symptoms start.

Eye symptoms are also still common during this stage, including:

  • Recurrent uveitis (returning inflammation of the middle layer of the eye, causing eye redness, pain, and blurred vision)
  • Glaucoma (a group of eye conditions that damage the optic nerve, which connects the eye to the brain)
  • Choroiditis (inflammation of the choroid, the layer of blood vessels and connective tissue between the sclera and the retina)
  • Retinal detachment (the retina, a thin layer of tissue at the back of the eye, pulls away from its normal position)

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Vitiligo in vogt-koyanagi-harada syndrome
Vitiligo in vogt-koyanagi-harada syndrome. Waikato District Health Board / DermNet / CC BY-NC-ND 

Diagnosis

Because VKH syndrome is rare, a correct diagnosis usually requires consultations with specialists. There is no specific test for the syndrome, so diagnosis is based on the symptoms present plus test results.

A neurologist, a doctor who specializes in disorders of the brain and nervous system, will perform a lumbar puncture, or spinal tap. This procedure involves the insertion of a hollow needle in the space within the spinal column of the lower back to examine the cerebrospinal fluid (CSF) for changes characteristic of VKH syndrome. An ophthalmologist, a doctor specializing in disorders and diseases of the eye, will perform special testing of the eyes to look for uveitis.

A physician specializing in conditions of the skin, hair, and nails, a dermatologist, will take a skin sample (biopsy) about one month after the eye symptoms begin to check for changes that may point to VKH syndrome. One change a dermatologist will look for is a lack of melanin in any light or white patches of skin that are present.

The American Uveitis Society recommends that in order for a diagnosis of VKH syndrome to be made, there should be no history of trauma to the eyes and that three of the following four signs should be present:

  • Iridocyclitis (inflammation of the iris and ciliary bodies, which help focus the eyes) in both eyes
  • Choroiditis, or posterior uveitis
  • Neurological symptoms or characteristic changes in the CSF
  • Skin symptoms of poliosis, vitiligo, or alopecia

Treatment

To reduce inflammation in the eyes, corticosteroids such as prednisone are given. If this does not work well, immunosuppressant drugs may be tried, including the following:

  • Azathioprine
  • Cyclophosphamide
  • Tacrolimus
  • Mycophenolate mofetil
  • Cyclosporin
  • Rituximab

Neurological symptoms often resolve with steroids.

Skin symptoms are treated the way vitiligo is treated, which may include phototherapy (treatment with a special type of light), corticosteroids, or medicated ointments.

Early diagnosis and treatment of VKH syndrome help prevent permanent vision changes such as glaucoma and cataracts (the clouding of the lens of the eye). Some evidence suggests patients may benefit from the early use of immunomodulatory therapy (immunotherapy drugs that can change your body's immune response) instead of only using steroids. Skin changes may be permanent, even with treatment, but hearing is usually restored in most individuals.

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  • Choczaj-Kukula A. Vogt-Koyanagi-Harada syndrome. eMedicine. 2005.

  • Read RW. American Uveitis Society. Vogt-Koyanagi-Harada Disease. Updated 2014.