Vogt-Koyanagi-Harada disease (VKHD), initially described as an uveomeningoencephalitic syndrome is characterized by panuveitis, often associated with neurologic and cutaneous manifestations, including headache, hearing loss, vitiligo and poliosis.
- HLA-DR4 strongly associated
History:
In 1906, Alfred Vogt in Switzerland first described a patient with premature whitening of eyelashes of sudden onset and bilateral subacute iridocyclitis. Twenty years later, Harada (1926) reported a case series with bilateral serous retinal detachment in association with cerebrospinal fluid (CSF) pleocytosis. Shortly thereafter (1929), Koyanagi published a review article associating unequivocally the posterior eye involvement with auditory and integumentary manifestations. In 1932, Babel suggested that these cases represented a single entity, which was then named Vogt-Koyanagi-Harada Disease.
It is speculated that the renowned painter Francisco José Goya y Lucientes (1746–1828) may have presented the disease in his mid career (1792). Its main features were loss of vision and hearing, ringing in the ears, vertigo, weakness on one side of the body, confusion, abdominal pain and malaise. Goya recovered most of his eyesight, but remained permanently deaf.
Pathophysiology
Multisystemic, granulomatous inflammation related to T-cell-mediated autoimmune dysregulation, targeting melanocytic self-antigens (eye, inner ear, meninges, skin and hair)
Clinical features
Acute onset of bilateral blurred vision with hyperemia preceded by flu-like symptoms.
Prodromal/meningeal stage:
Lasts a few days and mimics a viral infection
- Fever, headache, nausea, vertigo, orbital pain, photophobia, tearing, tinnitus, vertigo and neurologic symptoms.
Acute uveitic stage:
Typically occurs within 3 to 5 days of the prodromal stage and lasts for several weeks.
- Acute bilateral blurring of vision (due to diffuse choroiditis)
- Hyperemia & edema of optic disk
Convalescent stage:
Follows the acute uveitic stage, usually a few months later.
- Characterized by depigmentation of the integument and choroid.
- Vitiligo, alopecia and poliosis
- Perilimbal depigmentation
- “Sunset glow fundus”
Recurrent or chronic stage:
May interrupt the convalescent stage
- Mainly involves anterior segment, without clinically detectable posterior involvement
Extraocular manifestations:
- CNS involvement (prodromal/meningeal stage)
- Inner ear involvement
- Skin and appendages involvement:
- Vitiligo, alopecia, poliosis
- Sugiura’s sign (perilimbal vitiligo): Earliest depigmentation to occur
Diagnosis
Fundus examination:
- Dalen Fuchs nodules (local granulomas due to proliferation of lymphocytes and epitheloid cells) located b/t RPE & Bruch’s membrane
Differential diagnosis:
- Sympathetic ophthalmia (preceded by ocular penetrating trauma and/or previous intraocular surgery)
Management
