INTRODUCTION
Vogt-Koyanagi-Harada (VKH) disease is a multisystem
inflammatory syndrome primarily encountered
by ophthalmologists presenting as bilateral, chronic
granulomatous panuveitis. Autoimmunity against melanocytes
in the choroid, meninges, inner ear, and integumentary
system is widely hypothesized.1,2 Named after 3 physicians
who separately reported the diverse diagnostic findings,3 VKH
often begins with a neurologic prodrome including meningismus
and auditory changes prior to visual changes in the acute
uveitic phase. In the chronic phase, vitiligo, poliosis, and/or
alopecia are common integumentary manifestations.1,3,4,7
CASE REPORT
A 64-year-old female with a past medical history significant for
cataracts and chronic uveitis presented to the clinic in March,
2011 for evaluation and treatment of asymptomatic, progressive
depigmented patches on her forehead, scalp, and trunk
that began abruptly one year earlier. She reported whitening
of her eyebrows, eyelashes, followed by scalp hair, but denied
alopecic patches. Her history of uveitis was complex: first diagnosed
in 1989 by an ophthalmologist after the sudden onset
of red, painful eyes and blurred vision, a previous diagnosis of
sarcoidosis had been given. Treatments included multiple immunomodulators
accompanied by chronic oral prednisone as
well as intravitreal fluocinolone. Other misdiagnoses included
multiple sclerosis in 1995 due to the sudden onset of left leg
motor weakness and a "confirmatory" lumbar puncture, which
revealed pleocytosis. Her visual problems culminated with left
sided retinal detachment of in 2010. Successful treatment for
her vitiligo was achieved with topical steroids followed by approximately
five months of weekly topical PUVA treatments.
DISCUSSION
Vogt-Koyanagi-Harada disease typically presents in the fourth
decade of life and affects females disproportionately. 1 Most
cases are found in Latin America and Asia, and are associated
with class II human leukocyte antigens DR4 and DRB1 * 0405. 2
Blacks of sub-Saharan descent, such as in this case, are uncommonly
affected. 4 CD4+ T-cells of the Th1 and Th17 subsets have
been implicated in the disease process as both IFN-γ and IL-17
are elevated in the peripheral blood and cerebrospinal fluid of
VKH patients. 4,5 Visual prognosis is good if treatment begins
early and continues long term including high dose glucocorticosteroids
and cyclosporine. 4,6 A recent review reported vitiligo
1