eMedicine Specialties > Dermatology > Pediatric Diseases

Vogt-Koyanagi-Harada Syndrome

Author: Anna Choczaj-Kukula, MD, PhD, Locum Consultant Dermatologist, St John's Institute of Dermatology, St Thomas' Hospital, UK
Coauthor(s): Camila K Janniger, MD, Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School
Contributor Information and Disclosures

Updated: Jul 15, 2009

Introduction

Background

Vogt-Koyanagi-Harada (VKH) syndrome is a rare systemic disease involving various melanocyte-containing organs. Bilateral panuveitis associated with cutaneous, neurologic, and auditory abnormalities are manifestations of this inflammatory granulomatous disorder. As first described by Vogt in 1906 and Koyanagi in 1929, predominantly anterior uveitis associated with poliosis, vitiligo, and auditory disturbances characterizes Vogt-Koyanagi syndrome. In 1926, Harada reported a patient with idiopathic uveitis affecting the posterior segment with retinal detachment and meningeal irritation. At present, these 2 disorders are considered variations of a single entity referred to as Vogt-Koyanagi-Harada syndrome or uveoencephalitis.

Pathophysiology

The etiologic and pathogenic factors in VKH syndrome remain unclear. The clinical course of VKH syndrome with an influenzalike episode suggests a viral or postinfectious origin. Some studies invoke a possible role of Epstein-Barr virus reactivation in this disease.1 Although a viral cause has been proposed, no virus has been isolated or cultured from patients with VKH syndrome. Morris and Schlaegel found viruslike inclusion bodies in the subretinal fluid of a patient with VKH syndrome.

Clinical and experimental data continue to support an immunologic etiology. An autoimmune reaction seems to be directed against an antigenic component shared by uveal, dermal, and meningeal melanocytes, possibly tyrosinase or a tyrosinase-related protein. It has been suggested that Th17 subset of T cells plays a role in the initiation and maintenance of this disease. VKH syndrome can be associated with other autoimmune disorders such as autoimmune polyglandular syndrome,2 hypothyroidism, Hashimoto thyroiditis, diabetes mellitus,3,4 and IgA nephropathy.5

Single reports of patients developing VKH syndrome after cutaneous injury have been noted, as well as 2 cases of this condition occurring after BCG therapy for melanoma and 1 case following surgery of metastatic malignant melanoma.6  Case reports indicate that even an indirect trauma in melanocyte-containing tissue may induce an inflammatory response within the eye, with Vogt-Koyanagi-Harada disease following a closed head trauma.7  Cases of this syndrome were reported to be linked to malignant lymphoma.8,9

Immunologic analysis of cerebrospinal fluid (CSF) lymphocytes in VKH syndrome and studies of human uveal melanocytes show that uveal pigment can stimulate lymphocyte cultures from patients with VKH syndrome. Lymphocytes of peripheral blood and CSF from these patients may reveal in vitro cytotoxicity against allogenic melanoma cells.

Circulating antibodies against a retinal photoreceptor region have been detected in patients with this disorder.

The possibility that VKH syndrome has an autoimmune pathogenesis is supported by the statistically significant frequency of HLA-DR4, an antigen commonly associated with other autoimmune diseases. VKH syndrome has been closely associated with HLA-B54, HLA-DR4, and HLA-DR53 in Japanese patients10 ; with HLA-DR4, HLA-DRw53, and HLA-DQw3 in subjects of Native American ancestry; with HLA-DR1 and HLA-DR4 in Hispanic patients living in southern California; and with HLA-DR4 and HLA-DQw7 in Chinese patients.11 HLA-DR4 also was found to be significantly related to VKH syndrome in white Europeans, specifically in Italian patients.12 These findings confirm the possibility of immunogenic predisposition and the decisive role of HLA-DR4 antigen in the development of the disease.

Data indicate that patients with VKH syndrome are sensitized to melanocyte epitopes and display a peptide-specific Th1 cytokine response. Patients bearing HLA-DRB1*0405 recognize a broader melanocyte-derived peptide repertoire, so the presence of this allele increases susceptibility to the development of VKH disease. In a group of French VKH syndrome DRB1*04-positive patients, the HLA-DRB1*0405 subtype was found in 71%.13

Frequency

United States

VKH syndrome is rare. No precise data are available regarding frequency of the disease.

International

VKH syndrome is rare but widely distributed.14,15

Mortality/Morbidity

VKH syndrome is not associated with mortality. Acute disturbances in hearing and vision may occur, and the cutaneous changes may be permanent.

Race

VKH syndrome occurs more frequently in individuals with darker pigmentation (eg, persons of Asian, Native American, Latin American, or black heritage). VKH syndrome is one of the most common forms of uveitis among pigmented races. The manifestations of VKH syndrome in whites resemble those in the Japanese population. However, cutaneous signs are much more rarer.

Sex

Women appear to be affected with VKH syndrome more frequently than men.

Age

The onset of VKH syndrome has been reported to range from 3-89 years, with a maximum frequency in the thirties. Although often unrecognized, VKH syndrome may affect children.16

Clinical

History

VKH syndrome is usually preceded by a prodromal stage of nonspecific symptoms including headache, vertigo, nausea, nuchal rigidity, vomiting, and low-grade fever that may last a few days. Patients 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).

Physical

  • The American Uveitis Society has recommended that in addition to an absence of prior trauma or surgery, at least 3 of the following 4 criteria be met to confirm the diagnosis of VKH syndrome:
    • Bilateral iridocyclitis
    • Posterior uveitis, which may include exudative retinal detachment, optic nerve swelling, or atrophy of the retinal pigment epithelium
    • CSF pleocytosis or evidence of tinnitus, dysacusis, headache or meningismus, or cranial nerve involvement
    • Cutaneous findings of vitiligo, alopecia, or poliosis
  • The classic course of VKH syndrome consists of the following 3 phases:
    • In the meningoencephalitis phase, the degree of neurologic symptoms may vary. Generalized muscle weakness, hemiparesis, hemiplegia, dysarthria, and aphasia have been reported. Most of the neurologic symptoms have been directly attributed to changes in CSF (eg, pleocytosis, increased pressure, protein levels), inflammatory arachnoiditis, or resulting subarachnoidal adhesions. Mental changes ranging from mild confusion to psychosis may occur.
    • The ophthalmic-auditory phase is characterized by common features such as decreased visual acuity, eye pain, eye irritation, and loss of vision. Dysacusis (usually bilateral) and tinnitus develop in 50% of patients.
    • The convalescent phase is characterized by cutaneous signs developing after uveitis begins to subside, usually within 3 months from the onset of the disease. Although cutaneous signs typically occur several weeks to months after the onset of ocular inflammation, skin changes have sometimes been observed many years before uveitis appeared. Pigmentary changes tend to be permanent.
      • Poliosis, which occurs in 90% of patients, involves the eyebrows and eyelashes and, occasionally, the scalp and body hair. Poliosis affects 50% of patients and usually appears after the onset of alopecia, which may be patchy or diffuse.
      • Vitiligo manifests in 63% of patients and is often symmetric. Most patients have perilimbal vitiligo (Sugiura sign). Atypical variants of vitiligo with inflammatory raised borders and plaque-type inflammatory erythema have also been reported.
      • Halo nevi may be present.17

Causes

The cause of VKH syndrome is unknown, but a viral factor has been suggested in the pathogenesis. An autoimmune reaction to melanocytes with the involvement of T-cell–mediated cytotoxicity and apoptosis is postulated. Although almost all instances of VKH syndrome are sporadic, and familial cases are rare, some authors suggest that the condition may be inherited, probably as an autosomal recessive trait. Numerous data demonstrate the association of HLA-DR4 antigen and VKH syndrome in different racial groups. According to some studies, the major factor contributing to susceptibility for the disease is presence of the DRB*0405 allele.18

More on Vogt-Koyanagi-Harada Syndrome

Overview: Vogt-Koyanagi-Harada Syndrome
Differential Diagnoses & Workup: Vogt-Koyanagi-Harada Syndrome
Treatment & Medication: Vogt-Koyanagi-Harada Syndrome
Follow-up: Vogt-Koyanagi-Harada Syndrome
References
[ CLOSE WINDOW ]

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Further Reading

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Keywords

Vogt-Koyanagi-Harada syndrome, VKH syndrome, VKHS, oculocutaneous syndrome, uveocutaneous syndrome, uveoencephalitis, posterior uveitis, bilateral iridocyclitis, iritis, glaucoma, alopecia, vitiligo, dysacusia, CSF pleocytosis, Harada syndrome, poliosis, vitiligo, halo nevi, alopecia, HLA-DR4 antigen

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Contributor Information and Disclosures

Author

Anna Choczaj-Kukula, MD, PhD, Locum Consultant Dermatologist, St John's Institute of Dermatology, St Thomas' Hospital, UK
Disclosure: Johnson & Johnson Salary Management position

Coauthor(s)

Camila K Janniger, MD, Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School
Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Janet Fairley, MD, Professor and Head, Department of Dermatology, University of Iowa
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.

Pharmacy Editor

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.

Managing Editor

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-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.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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