World Library  
Flag as Inappropriate
Email this Article

Vogt–Koyanagi–Harada syndrome

Article Id: WHEBN0004312166
Reproduction Date:

Title: Vogt–Koyanagi–Harada syndrome  
Author: World Heritage Encyclopedia
Language: English
Subject: List of cutaneous conditions, Lentigo simplex, Depigmentation, Liver spot, Argyria
Publisher: World Heritage Encyclopedia

Vogt–Koyanagi–Harada syndrome

Dermatologic manifestation of VKH

Vogt–Koyanagi–Harada syndrome (VKH syndrome) is an uncommon multisystem disease of presumed autoimmune etiology that is characterized by chronic, bilateral, diffuse, granulomatous uveitis with accompanying dermatologic, neurologic, and auditory involvement. VKH syndrome is named for ophthalmologists Alfred Vogt from Switzerland and Yoshizo Koyanagi and Einosuke Harada from Japan.[1][2][3]


  • Pathophysiology 1
  • Clinical presentation 2
  • Diagnosis 3
  • Treatment 4
  • References 5
  • External links 6


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, uvea, central nervous system and inner ear. A new T-cell subset Th17 may play an important role in the initiation and maintenance of inflammatory disease when stimulated by the interleukin IL-23, thus producing IL-17.[4]

There is a higher rate of VKH syndrome in people of Asian, Latin, and Mediterranean descent

Clinical presentation

Uveitis with poliosis of the eyelashes.

There are 4 stages of VKH syndrome: prodromal, acute uveitic, convalescent, and chronic recurrent.

  • The prodromal stage is marked by flu-like symptoms. Patients present with headache, nausea, meningism, dysacusia (discomfort caused by loud noises or a distortion in the quality of the sounds being heard) and tinnitus.
  • The acute uveitic stage is heralded by the onset of blurring of vision in both eyes and is marked by bilateral granulomatous anterior uveitis, variable degree of vitritis, thickening of the posterior choroid with elevation of the peripapillary retinal choroidal layer, hyperemia and edema of the optic nerve, and multiple serous retinal detachments.
  • The convalescent stage occurs several weeks later and is marked by gradual depigmentation of the choroid, resulting in the classic orange-red discoloration, or sunset-glow fundus appearance. Skin changes including alopecia, vitiligo, and poliosis typically appear during this stage.
  • The chronic recurrent stage is marked by repeated bouts of uveitis.


The diagnosis of VKH syndrome is made by ophthalmologist or optometrist based on clinical presentation. Imaging techniques such as fluorescein angiography are used to confirm diagnosis.


The acute stage of VKH syndrome is exquisitely responsive to early and aggressive treatment with topical, periocular and systemic corticosteroids and cycloplegic agents.


  1. ^ A Vogt. Frühzeitiges Ergrauen der Zilien und Bemerkungen über den sogenannten plötzlichen Eintritt dieser Veränderung. Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1906, 44: 228-242.
  2. ^ Y. Koyanagi. Dysakusis, Alopecie und Poliosis bei schwerer Uveitis nicht traumatischen Ursprungs. Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1929, 82: 194-211.
  3. ^ E. Harada. Clinical study of nonsuppurative choroiditis. A report of acute diffuse choroiditis. Acta Societatis ophthalmologicae Japonicae, 1926, 30: 356.
  4. ^ Bordaberry, MF (Nov 2010). "Vogt-Koyanagi-Harada disease: diagnosis and treatments update.". Current opinion in ophthalmology 21 (6): 430–5.  

External links

  • VKH Story
This article was sourced from Creative Commons Attribution-ShareAlike License; additional terms may apply. World Heritage Encyclopedia content is assembled from numerous content providers, Open Access Publishing, and in compliance with The Fair Access to Science and Technology Research Act (FASTR), Wikimedia Foundation, Inc., Public Library of Science, The Encyclopedia of Life, Open Book Publishers (OBP), PubMed, U.S. National Library of Medicine, National Center for Biotechnology Information, U.S. National Library of Medicine, National Institutes of Health (NIH), U.S. Department of Health & Human Services, and, which sources content from all federal, state, local, tribal, and territorial government publication portals (.gov, .mil, .edu). Funding for and content contributors is made possible from the U.S. Congress, E-Government Act of 2002.
Crowd sourced content that is contributed to World Heritage Encyclopedia is peer reviewed and edited by our editorial staff to ensure quality scholarly research articles.
By using this site, you agree to the Terms of Use and Privacy Policy. World Heritage Encyclopedia™ is a registered trademark of the World Public Library Association, a non-profit organization.

Copyright © World Library Foundation. All rights reserved. eBooks from Project Gutenberg are sponsored by the World Library Foundation,
a 501c(4) Member's Support Non-Profit Organization, and is NOT affiliated with any governmental agency or department.