Case Report: Vogt-Koyanagi-Harada Disease in an African-American Female

August 2012 | Volume 11 | Issue 8 | Features | 1004 | Copyright © August 2012



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


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.


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