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Genetic and Rare Diseases Information Center (GARD)

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Vogt-Koyanagi-Harada syndrome

Other Names for this Disease
  • VKH syndrome
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What is the cause of Vogt-Koyanagi-Harada syndrome?

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What is Vogt-Koyanagi-Harada syndrome?

Vogt-Koyanagi-Harada syndrome is a condition that involves chronic inflammation of melanocytes, which are specialized cells that produce a pigment called melanin.  Melanin is the substance that gives skin, hair, and eyes their color.  Melanin is also found in the retina, where it plays a role in normal vision.  Individuals with Vogt-Koyanagi-Harada syndrome usually develop vision and hearing disturbances first, followed by signs of skin problems.  Research suggests this condition is an autoimmune disease.  Vogt-Koyanagi-Harada syndrome is more common in individuals with darker skin pigmentation including Asian, Middle Eastern, Hispanic, and Native American populations.[1][2]
Last updated: 3/14/2014

What are the signs and symptoms of Vogt-Koyanagi-Harada syndrome?

Vogt-Koyanagi-Harada syndrome affects the eyes, ears, skin (cutaneous), and nervous system. Early symptoms include headache, dizziness, neck stiffness, vomiting, and low-grade fever. The symptoms progress to sudden visual loss in one or both eyes, eye pain, eye swelling and irritation (uveitis, choroiditis) and hearing disturbances.  Changes in the coloring of the skin and hair are usually observed three months after symptoms first appear and they are often permanent.[1] Vision and hearing generally improve with medical treatment. [2]
Last updated: 3/14/2014

What causes Vogt-Koyanagi-Harada syndrome?

Research has indicated that Vogt-Koyanagi-Harada syndrome is an autoimmune disease in which the body attacks its own pigment cells (melanocytes), possibly in response to an infection (ie: a virus).[2] The strong association between Vogt- Koyanagi-Harada syndrome and certain racial and ethnic groups suggests that genetic factors may be involved.  Genes related to the immune system, namely those genes in the HLA gene family, may play a role in the development of Vogt-Koyanagi-Harada syndrome.  [3]
Last updated: 3/14/2014