Vogt-koyanagi-harada disease H30.8

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).

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.

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.

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.

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