Vogt-Koyanagi-Harada syndrome

A form of uveomeningoencephalitis endemic in the Far East. A disease, usually occurring in adult life, in which severe bilateral inflammation of the iris (iridocyclitis), ciliary body and choroid of the eye is associated with relapsing meningoencephalitis, deafness, alopecia, depigmentation of the skin and eye, symmetrical vitilligo and poliosis (whitening of the ends of the hairs). There is a tendency towards recovery of sight, but it is not always complete. The syndrome usually occurs in young adults in Japan (most cases) and Italy. Mental and growth retardation may occur in some rare familial cases; most cases being sporadic.

Some authors distinguish between the Vogt-Koyanagi syndrome and the Harada disease. The symptoms of the Vogt-Koyanagi syndrome overlap those of Harada disease/syndrome, and they may at times be indistinguishable from Harada's disease. The conspicuous feature of the Vogt-Koyanagi syndrome is the severe, prolonged bilateral uveitis.

If an exudative choroiditis, causing retinal detachment, is present the condition is called Harada syndrome. It is thought to be of viral origin but this has not been proved. Features that reportedly differentiate Harada’s syndrome from the Vogt-Koyanagi syndrome include: 1) The characteristic development of bilateral retinal detachment and its usual spontaneous healing; 2) alopecia and poliosis, which may appear but are not an integral part of the syndrome; 3) changes in the spinal fluid. Vogt-Koyanagi-Harada has also been described as Vogt-Koyanagi syndrome plus retinal detachment due to exudative choroiditis.

Alfred Vogt first described this syndrome in 1906. In 1922, Einosuku Harada discovered a rare case of acute uveitis with bilateral retinal detachment. He compiled similar cases and established a new clinical entity, which is now named "Harada disease". Later it was found that this disease together with the diseases described by Vogt and Yoshizo Koyanagi is an expression of the same disease entity, i.e., Vogt-Koyanagi-Harada disease.

We thank Patrick Jucker-Kupper, Switzerland, for information submitted.

• A Vogt:
  Frühzeitiges Ergrauen der Zilien und Bemerkungen über den sogenannten plötzlichen Eintritt dieser Veränderung.
  Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1906, 44: 228-242.
• E. Harada:
  Beitrag zur klinischen Kenntnis von nichteitriger Choroiditis (Choroiditis diffusa acuta).
  Nippon Ganka Gakkai Zasshi (= Acta Societatis Ophthalmologica Japonica), 1926, 30: 356-361. Clinical study of nonsuppurative choroiditis. A report of acute diffuse choroiditis.
  Acta Societatis ophthalmologicae Japonicae, 1926, 30: 356.
• Y. Koyanagi:
  Dysakusis, Alopecie und Poliosis bei schwerer Uveitis nicht traumatischen Ursprungs.
  Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1929, 82: 194-211.
• A. Cowper:
  Harada’s disease and Vogt-Koyanagi syndrome.
  Archives of Ophthalmology, Chicago, 1951, 45: 367-376.
• T. Yugé:
  The relation between VK syndrome and sympathetic ophthalmia.
  American Journal of Ophthalmology, Chicago, 1957, 43: 735-744. After Cowper’s work in 1951 Vogt-Koyanagi and Harada’s sequenz is considered the same entity.