Presumed Vogt-Koyanagi-Harada disease with unilateral ocular involvement: report of three cases

Graefes Arch Clin Exp Ophthalmol. 2009 Aug;247(8):1127-32. doi: 10.1007/s00417-009-1068-8. Epub 2009 Mar 24.

Abstract

Aim: To report three cases of presumed Vogt-Koyanagi-Harada (VKH) disease with unilateral ocular manifestations.

Methods: This retrospective study reviewed the long-term follow-up observations of three patients who attended the uveitis clinic at Tokyo Medical University Hospital. The patients were followed for 5-16 years with systemic clinical, ophthalmologic and laboratory examinations. Ophthalmoscopic findings, extraocular manifestations, visual acuity, and response to corticosteroid administration were evaluated.

Results: Three patients had characteristic clinical features of VKH involving only one eye, including diffuse choroiditis, serous retinal detachment, focal areas of delayed choroidal perfusion, multifocal areas of pinpoint leakage, macular oedema, and optic nerve staining. All patients received systemic corticosteroid therapy during the acute phase of the disease. During the follow-up period (5-16 years), all three patients developed sunset-glow fundus and nummular chorioretinal depigmented scars in the affected eye only, as well as systemic complications of deafness, vitiligo, and poliosis.

Conclusion: The clinical and laboratory features of all three patients were typical of VKH disease except for the unilateral involvement. It is important for ophthalmologists to recognize unilateral VKH disease, even though it is a rare clinical variant of the disease.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Choroid Diseases / diagnosis*
  • Choroid Diseases / drug therapy
  • Choroid Diseases / physiopathology
  • Deafness / diagnosis
  • Female
  • Fluorescein Angiography
  • Follow-Up Studies
  • Glucocorticoids / therapeutic use
  • Humans
  • Male
  • Middle Aged
  • Ophthalmoscopy
  • Prednisolone / therapeutic use
  • Retinal Diseases / diagnosis*
  • Retinal Diseases / drug therapy
  • Retinal Diseases / physiopathology
  • Retrospective Studies
  • Uveitis / diagnosis
  • Uveitis / drug therapy
  • Uveitis / physiopathology
  • Uveomeningoencephalitic Syndrome / diagnosis*
  • Uveomeningoencephalitic Syndrome / drug therapy
  • Uveomeningoencephalitic Syndrome / physiopathology
  • Visual Acuity / physiology
  • Vitiligo / diagnosis

Substances

  • Glucocorticoids
  • Prednisolone