What is the definition of Vogt-Koyanagi-Harada Disease?

Vogt-Koyanagi-Harada disease (VKH disease) affects the eyes, ears, nervous system, and skin. The symptoms of VKH occur in different phases. The early symptoms include headaches, eye pain, and dizziness. These are followed by hearing loss and inflammation of the eye (panuveitis). Later symptoms include development of white patches of skin and hair (vitiligo), hair loss (alopecia), cataracts, and glaucoma. The exact cause of VKH disease is unknown, but the symptoms are thought to be due to an abnormal response of the immune system to a viral infection. Genetic factors may be involved. Diagnosis of VKH disease is based on the symptoms, clinical exam, and imaging studies. Other more common diseases may need to excluded before a diagnosis of VKH disease is made. VKH disease is treated with corticosteroids and other medications.

What are the alternative names for Vogt-Koyanagi-Harada Disease?

  • VKH syndrome
  • Vogt-Koyanagi-Harada syndrome
  • Uveomenigitic syndrome
  • VKH disease

What are the causes for Vogt-Koyanagi-Harada Disease?

The exact cause of Vogt-Koyanagi-Harada (VKH) disease is not known. It is thought to be due to an abnormal immune response in which the body attacks its own pigment cells (melanocytes). In addition, genetic factors may play a role.

What are the symptoms for Vogt-Koyanagi-Harada Disease?

The following list includes the most common signs and symptoms in people with Vogt-Koyanagi-Harada disease. These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition. The symptoms of Vogt-Koyanagi-Harada(VKH) disease occur in different phases. In the early phase, symptoms may include:
  • Headache
  • Dizziness (vertigo)
  • Neck stiffness
  • Nausea and vomiting
  • Hearing loss
  • Ringing in the ears (tinnitus)
The uveitis phase occurs a few weeks after the early phase. In this phase, symptoms may include:
  • Blurry vision in one or both eyes
  • Inflammation of the eyes (panuveitis)
  • Floating spots in the vision (floaters) that are signs of retinal detachment
The convalescent phase usually occurs a few weeks to months after the uveitis phase. In this phase, symptoms may include:
  • Patches of white skin (vitiligo)
  • Patches of white hair, eyelashes, and eyebrows (poliosis)
  • Hair loss (alopecia)
The recurrent phase occurs in about half of the people with VKH disease. Symptoms may include:
  • Clouding of the lens (cataracts)
  • Build-up of pressure in the eyes (glaucoma)
  • Abnormal blood vessels growth under the retina (choroidal neovascularization)
Most people with VKH disease develop symptoms in both eyes. Vision and hearing problems can improve with treatment. The skin changes and hair loss may be permanent. 

What are the current treatments for Vogt-Koyanagi-Harada Disease?

Treatment of Vogt-Koyanagi-Harada (VKH) disease is focused on managing the symptoms. Treatment usually involves early and aggressive treatment with systemic corticosteroids (steroids). Other medications may be used as well. Specialists involved in the care of someone with VKH disease may include: 
  • Dermatologist
  • Ophthalmologist
  • Neurologist

How is Vogt-Koyanagi-Harada Disease diagnosed?

Vogt-Koyanagi-Harada (VKH) disease is diagnosed based on the symptoms, clinical exam, eye exam, and imaging studies. In addition, other more common conditions may need to be excluded before VKH disease can be diagnosed. A set of standard signs and symptoms have been published to help guide the diagnosis of VKH disease.