Vogt-Koyanagi-Harada syndrome

Vogt-Koyanagi-Harada syndrome

Infobox_Disease
Name = PAGENAME


Caption =
DiseasesDB = 13983
ICD10 = ICD10|H|20|8|h|15
ICD9 = ICD9|364.24
ICDO =
OMIM =
MedlinePlus =
eMedicineSubj = derm
eMedicineTopic = 739
MeshID = D014607

Vogt-Koyanagi-Harada syndrome (VKH syndrome) is a condition seen in humans and dogs involving various melanocyte-containing organs, characterized by uveitis (inflammation of the inside of the eye), poliosis (whitening of hair), vitiligo (loss of pigment in the skin), and meningitis, although dogs with this syndrome rarely develop meningitis. It is also known as uveodermatologic syndrome.

Pathophysiology

VKH syndrome is an immune-mediated disease. The mechanism of the disease is thought to be T helper cell mediated autoimmune attack of melanocytes in the skin and uvea of dogs and humans, and in the central nervous system and inner ear of humans. [cite journal |author=Sigle K, McLellan G, Haynes J, Myers R, Betts D |title=Unilateral uveitis in a dog with uveodermatologic syndrome |journal=J Am Vet Med Assoc |volume=228 |issue=4 |pages=543–8 |year=2006 |pmid=16478427 |doi=10.2460/javma.228.4.543]

In humans there is a higher rate of VKH syndrome in people of Asian, Latin, and Mediterranean descent.cite book|author=Gelatt, Kirk N. (ed.)|title=Veterinary Ophthalmology|edition=3rd ed.|publisher=Lippincott, Williams & Wilkins|year=1999|id=ISBN 0-683-30076-8]

VKH syndrome in dogs

In dogs VKH syndrome (often called VKH-like syndrome) most commonly affects young animals and is seen most commonly in the Akita Inu, but also in the Siberian Husky and Samoyed. Uveitis usually occurs first and is often severe enough to cause blindness. VKH syndrome can also cause retinal detachment, cataracts, and glaucoma. Pigment partly disappears from the retinal pigment epithelium and iris. Skin lesions include loss of pigment and hair on the eyelids, nose, and lips. Symptoms and biopsy will confirm the diagnosis. Treatment is with immunosuppressive drugs such as prednisone and azathioprine. The prognosis is guarded.

Eponym

It is named for Alfred Vogt, Yoshizo Koyanagi, and Einosuke Harada. [WhoNamedIt|synd|1072] [A Vogt. Frühzeitiges Ergrauen der Zilien und Bemerkungen über den sogenannten plötzlichen Eintritt dieser Veränderung. Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1906, 44: 228-242.] [Y. Koyanagi. Dysakusis, Alopecie und Poliosis bei schwerer Uveitis nicht traumatischen Ursprungs. Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1929, 82: 194-211.] [E. Harada. Clinical study of nonsuppurative choroiditis. A report of acute diffuse choroiditis. Acta Societatis ophthalmologicae Japonicae, 1926, 30: 356.]

References


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  • Vogt-Koyanagi-Harada syndrome — bilateral uveitis with exudative iridocyclitis, choroiditis, meningism, and temporary or permanent retinal detachment, occurring in association with alopecia, vitiligo, poliosis, loss of visual acuity, headache, vomiting, deafness, and sometimes… …   Medical dictionary

  • síndrome de Vogt-Koyanagi-Harada — Eng. Vogt Koyanagi Harada syndrome Síndrome uveomeníngeo que cursa con iridociclocoroiditis, meningitis linfocitaria, desprendimiento retinocoroideo, sordera y alopecia. Enfermedad de Vogt Koyanagi Harada …   Diccionario de oftalmología

  • Harada syndrome — Ha·ra·da syndrome (hah rahґdah) [Einosuke Harada, Japanese ophthalmologist, 1892–1947] Vogt Koyanagi Harada syndrome; see under syndrome …   Medical dictionary

  • Harada syndrome — Vogt Koyanagi Harada s …   Medical dictionary

  • Vogt-Koyanagi syndrome — uveomeningitis characterized by exudative iridocyclitis and choroiditis associated with patchy depigmentation of the skin and hair; the lashes and eyebrows also become whitened, and there may also be retinal detachment and associated deafness and …   Medical dictionary

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