Vogt-koyanagi-harada diseaseH30.8

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 22.06.2022

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Synonym(s)

Harada disease; Harada Syndrome; Oculocutaneous syndrome; Uveo-encephalitis; Vogt Koyanagi Harada Syndrome; Vogt-Koyanagi Syndrome

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HistoryThis section has been translated automatically.

Vogt, 1906; Harada, 1926; Koyanagi, 1929

DefinitionThis section has been translated automatically.

Phased disease with inflammation of the uvea (prodromal fever reactions often combined with an infection of the respiratory tract), the retinal pigment layer, corneal precipitates, Koeppe nodules at the edge of the pupil and meninges, sometimes combined with encephalitis, cranial nerve deficits (N.vestibularis in 40-50%), later skin changes (symmetrical vitiligo in 60%; poliosis in 80% - mostly eyebrows and eyelashes) and hair changes (in >50% of cases: alopecia areata).

Occurrence/EpidemiologyThis section has been translated automatically.

Rare. The disease occurs mainly in Asians, dark-skinned Eurasians and dark-skinned Africans. In Saudi Arabia, the syndrome is the most common cause of uveitis.

EtiopathogenesisThis section has been translated automatically.

Unknown. Discussed are autoimmune reactions after viral infections, where a T-cell-mediated reaction against melanocytes in the skin, eye and inner ear occurs. Tyrosinase and TRP1 are regarded as possible allergens.

ManifestationThis section has been translated automatically.

Occurs mainly with blacks, orientals or Asians. 3rd - 4th decade of life.

Clinical featuresThis section has been translated automatically.

Skin manifestations (over 80% of cases; appearing weeks to months after onset of disease): Canities or poliosis, especially on eyebrows and eyelashes, also scalp, axillary and pubic hair. Vitiligo, bizarre or round hair thinning ( alopecia areata).

Extracutaneous manifestations: double-sided uveitis, secondary glaucoma, meningoencephalitis.

Progression/forecastThis section has been translated automatically.

Course over 1-4 years with relapses and remissions as well as defect healing.

LiteratureThis section has been translated automatically.

  1. Harada E (1926) Contributions to clinical coreitis of non-purulent choroiditis. Nippon Ganka Gakkai Zasshi 30: 356-361
  2. Koyanagi Y (1929) Dysacusis, alopecia and poliosis in severe uveitis of non-traumatic origin Clin Monatsbl Ophthalmology (Stuttgart) 82: 194-211
  3. Read RW (2002) Vogt-Koyanagi-Harada disease. Ophthalmol Clin North Am 15: 333-341
  4. Vogt A (1906) Early greying of the cilia and remarks on the so-called sudden occurrence of this change. Klin Monatsbl Ophthalmology (Stuttgart) 44: 228-242
  5. Okamoto Y et al (2004) Delayed regeneration of foveal cone photopigments in vogt-koyanagi-harada disease at the convalescent stage. Invest Ophthalmol Vis Sci 45: 318-322

Authors

Last updated on: 22.06.2022

Author: Prof. Dr. med. Peter Altmeyer