An Overview of Vogt-Koyanagi-Harada Syndrome

In This Article
Table of Contents

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. Some aspects of the disease (a flu-like presentation) suggest the possibility of a viral cause (etiology) although no virus has yet been identified. Most cases are sporadic, but there may also be a genetic component. VKH syndrome does not shorten lifespan, 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 than in men (in a two to one ratio), and its symptoms may begin at any age, but most commonly among people in their 40s.

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.

Meningoencephalitis phase

In this 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.

Ophthalmic-Auditory Phase

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.

Convalescent Phase

In the convalescent phase, 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) appear. 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, glaucoma, choroidal abnormality and sometimes retinal detachment.

This photo contains content that some people may find graphic or disturbing.

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

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 three of these four 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, cyclophosphamide, tacrolimus, mycophenolate mofetil, cyclosporin and rituximab may be used.

Neurological symptoms often resolve with steroids.

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 help prevent permanent vision changes such as glaucoma and cataracts. Some evidence suggests that patients may benefit from early use of immunomodulatory therapy (compared to only steroids). Skin changes may be permanent, even with treatment, but hearing is usually restored in most individuals.

Was this page helpful?
Article Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  • Choczaj-Kukula A. Vogt-Koyanagi-Harada syndrome. eMedicine. 2005.

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