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Vogt-Koyanagi-Harada Disease Clinical Presentation

  • Author: R Christopher Walton, MD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Apr 15, 2016
 

History

Patients with VKH disease usually initially present to an ophthalmologist for ocular problems, including sudden loss of vision, ocular pain, and photophobia. Hearing disturbances and dizziness may be present. After weeks or months, most patients notice cutaneous signs (eg, hair loss, poliosis, vitiligo).

Four clinical stages have been described in VKH disease, consisting of the prodromal stage, uveitic stage, chronic stage, and recurrent stage.

Prodromal stage

The prodrome typically lasts for a few days and is characterized by the following signs:

  • Fever
  • Headache
  • Meningismus
  • Nausea
  • Vertigo
  • Orbital pain
  • Tinnitus

CSF pleocytosis occurs in more than 80% of patients during this stage. Photophobia and tearing may develop, and patients also may note that their skin and hair is sensitive to touch during this stage.

Uncommon manifestations during the prodrome include cranial nerve palsies and optic neuritis. Some patients may not develop or report the symptoms characteristic of the prodrome.

Uveitic stage

The acute uveitic stage follows the prodromal stage by several days in most patients and typically lasts for several weeks. During this stage, the most common symptom is acute bilateral blurring of vision. As many as 70% of patients present with bilateral blurring of vision; in most of the remaining patients, the fellow eye is involved within several days.

Clinically, this stage manifests as bilateral posterior uveitis with retinal edema, optic disc hyperemia or edema, and, eventually, serous retinal detachments. Often, an accompanying anterior uveitis characterized by mutton-fat keratic precipitates and iris nodules are present. The intraocular pressure may be elevated because of forward rotation of the lens-iris diaphragm.

Chronic stage

During the chronic stage, ocular and dermatologic manifestations are common. Depigmentation of the choroid begins within the first 3 months after the onset of the disease. Areas of hyperpigmentation also may develop in the fundus. Dalen-Fuchs nodules may be seen in the peripheral and midperipheral retina. These nodules are small, yellow lesions that typically are located in the midperiphery of the retina. Eventually, the lesions fade and become atrophic.

Dermatologic changes include vitiligo and poliosis of the lashes, eyebrows, and hair. The vitiligo tends to be distributed symmetrically over the head, eyelids, and trunk. The duration of the chronic stage is typically several months, but it may last for many years.

Recurrent stage

During the recurrent stage, patients may develop recurrent or chronic anterior uveitis. In some patients, low-grade choroidal inflammation may accompany the anterior uveitis, which may require indocyanine green angiography for visualization.[48] Recurrent posterior uveitis with serous retinal detachment is rare. Patients treated with corticosteroids and/or immunomodulator therapy for 6 months or less may be at higher risk for recurrent serous retinal detachment.[49]

Ocular complications are relatively common during this stage and include cataracts, glaucoma, choroidal neovascularization, and subretinal fibrosis.

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Physical Examination

Patients suspected of having VKH disease should undergo a thorough physical examination to determine whether cutaneous, neurologic, and ophthalmic manifestations of the disorder exist.

Cutaneous manifestations

Sensitivity to touch of the hair and skin may be noted during the prodromal stage, while vitiligo, poliosis, and alopecia typically develop during the chronic stage. Vitiligo often is distributed symmetrically over the head, face, and trunk. The sacral region is a common site for the development of vitiligo. Poliosis may involve the scalp hair, eyebrows, and eyelashes.

Neurologic manifestations

Meningeal signs develop during the prodromal stage; they include meningismus, headache, and occasional confusion. CSF pleocytosis is relatively common during the prodrome.

Focal neurologic signs include the following:

  • Cranial nerve palsies
  • Hemiparesis
  • Transverse myelitis
  • Ciliary ganglionitis

Inner ear disorders, including dysacusis, tinnitus, and vertigo, occur in as many as 75% of patients. Cochlear hearing loss occurs mainly in high-frequency ranges. Inner ear dysfunction improves several months after onset in most patients.

Ophthalmic manifestations

Visual acuity may be decreased markedly in both eyes at the onset of the uveitic stage. Patients may present with unilateral loss of vision, but most develop bilateral disease within the first 10 days following onset.

Ocular adnexa involvement includes poliosis of the scalp, eyebrows, or eyelashes, which may develop during the convalescent stage of VKH disease. Vitiligo also may occur on the eyelids and face during this stage.

Anterior segment

Anterior-segment manifestations include the following:

  • Perilimbal vitiligo (Sugiura sign) - One of the earliest manifestations of depigmentation; uncommon, except in Japanese patients
  • Granulomatous or nongranulomatous anterior uveitis - May occur; Busacca nodules, Koeppe nodules, and mutton-fat keratic precipitates are characteristic of granulomatous anterior uveitis
  • Posterior synechiae - May be noted, especially in chronic cases; pupillary membrane formation is relatively common

Some patients may present with a shallow anterior chamber due to edema and infiltration of the ciliary body, with forward rotation of the lens-iris diaphragm and possible angle-closure glaucoma. Glaucoma may occur secondary not only to angle closure but also to pupillary block, or it may arise in association with chronic uveitis.

Cataracts may develop as a result of chronic inflammation and/or chronic corticosteroid therapy.

Posterior segment

Anterior vitreous cells may be noted, especially in patients with severe anterior uveitis. Optic disc hyperemia or edema may be present.

A study by Nakao et al indicated that a patient’s age and optic disc morphology, not the severity of inflammation, are associated with optic disc swelling in VKH disease. The retrospective, observational study included 58 patients (116 eyes), with 16 patients (32 eyes) demonstrating disc edema. Patients with disc swelling had a mean age of 58.9 years, compared with 41.4 years for those without swelling. Other factors, such as intraocular pressure, refractive error, and the cup-to-disc ratio, as well as the ratio of the disc-macula distance to the disc diameter, also differed between patients with swelling and those without it.[50]

One of the earliest retinal manifestations of VKH disease is retinal edema, which is often located within the posterior pole. This typically is followed by the development of bilateral, multifocal serous retinal detachments. The detachments occur most commonly in the inferior retina. (See the image below.)

Bilateral, multifocal serous detachments in a pati Bilateral, multifocal serous detachments in a patient with Vogt-Koyanagi-Harada disease. Disc hyperemia is evident in the right eye.

During the chronic stage of the disease, the serous detachments resolve and retinal pigment epithelium (RPE) alterations are common, including depigmentation, demarcation lines, and areas of hyperpigmentation.

The fundus of Asian and Hispanic patients may develop the characteristic red-orange appearance of the sunset-glow fundus, although this is relatively uncommon in other groups of patients. Areas of hyperpigmentation are also common and reflect changes occurring at the level of the RPE. Subretinal fibrosis, RPE migration, and disciform scars also may occur.

Neovascularization of the disc and retina may develop and can result in vitreous hemorrhage. Choroidal neovascularization of the macula may occur in the chronic stage and can result in profound loss of visual acuity.

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Contributor Information and Disclosures
Author

R Christopher Walton, MD Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, University of Tennessee College of Medicine

R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, Retina Society, American College of Healthcare Executives, American Uveitis Society

Disclosure: Nothing to disclose.

Coauthor(s)

Camila K Janniger, MD Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, Rutgers New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Anna Choczaj-Kukula, MD, PhD Locum Consultant Dermatologist, Barnet and Chase Farm NHS Trust; Honorary Clinical Research Fellow, Royal Free Hospital, UK

Anna Choczaj-Kukula, MD, PhD is a member of the following medical societies: American Academy of Dermatology, Royal Society of Medicine, European Academy of Dermatology and Venereology, British Association of Dermatologists

Disclosure: Partner received salary from Johnson & Johnson for management position.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Acknowledgements

David F Butler, MD Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Steve Charles, MD Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians and Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society

Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Other; Topcon Medical Lasers Consulting fee Consulting

Anna Choczaj-Kukula, MD, PhD Locum Consultant Dermatologist, St John's Institute of Dermatology, St Thomas' Hospital, UK

Anna Choczaj-Kukula, MD, PhD is a member of the following medical societies: American Academy of Dermatology, British Association of Dermatologists, European Academy of Dermatology and Venereology, and Royal Society of Medicine

Disclosure: Johnson & Johnson Salary Management position

Dirk M Elston, MD Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Janet Fairley, MD Professor and Head, Department of Dermatology, University of Iowa, Roy J and Lucille A Carver College of Medicine

Janet Fairley, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Federation for Medical Research, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Camila K Janniger, MD Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

John D Sheppard Jr, MD, MMSc Professor of Ophthalmology, Microbiology and Molecular Biology, Clinical Director, Thomas R Lee Center for Ocular Pharmacology, Ophthalmology Residency Research Program Director, Eastern Virginia Medical School; President, Virginia Eye Consultants

John D Sheppard Jr, MD, MMSc is a member of the following medical societies: American Academy of Ophthalmology, American Society for Microbiology, American Society of Cataract and Refractive Surgery, American Uveitis Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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Bilateral, multifocal serous detachments in a patient with Vogt-Koyanagi-Harada disease. Disc hyperemia is evident in the right eye.
Fluorescein angiography of the left eye in a patient with Vogt-Koyanagi-Harada disease. Midphase is shown on the left, with multiple areas of hyperfluorescence at the level of the retinal pigment epithelium (RPE). Late phase on the same angiogram (right) reveals multiple placoid areas of hyperfluorescence at the level of the RPE and pooling of dye in the areas of serous detachment.
Patient with progressive dysacusis and recent onset of visual loss. Fundus photo shows a large, multifocal serous detachment of the right eye. B-scan ultrasonography reveals posterior choroidal thickening with an overlying retinal detachment.
Patient with progressive dysacusis and recent onset of visual loss is shown here following 6 weeks of systemic corticosteroid therapy. Diffuse depigmentation of the choroid with retinal pigment epithelium migration is seen. Residual retinal striae are present in the peripapillary region. B-scan ultrasonography shows resolution of retinal detachment and choroidal thickening.
 
 
 
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