A systemic inflammatory disease with probable
immunogenetic predisposition that affects the eye (panuveitis) leading to
rapid loss of vision. It is also associated to a characteristic spectrum of
nonophthalmic features involving particularly the skin, hair (alopecia), and
ear. Meningitis and raised intracranial pressure have also been reported.
Harada Syndrome; Harada Disease; Vogt-Koyanagi Syndrome; Yugé Syndrome;
Oculocutaneous Syndrome; Uveocutaneous Syndrome;
Uveitis-Vitiligo-Alopecia-Poliosis Syndrome; Uveomeningeal Syndrome;
Uveomeningitis Syndrome; Uveomeningoencephalitis; Uveoencephalitis.
First described in 1906 by Alfred Vogt, Swiss
Unknown but more common in darker-pigmented races,
particularly Orientals and American Indians.
Unknown but strong association with HLA-DR
Immunocytologic findings are compatible with a
T-cell-mediated autoimmune reaction to uveal and dermal melanocytes.
Requires three of the following features: bilateral
iridocyclitis, posterior uveitis (including serous retinal detachment or
sunset glow fundus), central nervous system manifestations, and cutaneous
Patients are often of short stature. In addition to
uveitis, ophthalmic features can include visual loss, retinal detachment, cataract,
and glaucoma. Other features include the central nervous system and cutaneous
origin, including deafness (sensorineural), headache, tinnitus, dysacousis, and
meningism associated with cerebrospinal fluid pleocytosis. Alopecia,
premature graying of hair, and vitiligo are also observed. Onset is generally
in postadolescent life, but two case reports exist in children, the youngest being
4 years old. The condition is treated with high-dose steroids.
Complete medical history and physical
to central nervous system involvement and the use of steroids are important.
Evaluate adrenal function (clinical, electrolytes, glycemia).
Strict asepsis is needed because of
immunodeficiency induced by repeated steroid treatment.
Preoperative stress dose of steroids
might be required. Atropine and all drugs that can induce glaucoma should be
avoided. Succinylcholine should probably be avoided in case of retinal
detachment and because of its action on intraocular pressure. Consider benefit of
using aminoglycosides in cases of incomplete deafness.
Read RW: Vogt-Koyanagi-Harada disease. Ophthalmol Clin North Am 15(3):333, 2002.
Vogt A: Frühzeitiges Ergrauen der Zilien und Bemerkungen über den
sogenannten plötzlichen Eintritt dieser Veränderung. Klin Monatsbl Augenheilkd 44:228,