Vogt-Koyanagi-Harada (VKH) disease is a rare autoimmune stromal choroiditis affecting melanocyte-rich tissues and typically presenting with bilateral ocular inflammation associated with neurological, auditory, or integumentary manifestations. Early diagnosis is essential because prompt initiation of intensive immunosuppressive therapy is associated with improved long-term outcomes. An 18-year-old Hispanic man presented in September 2022 with a one-week history of bilateral visual loss and ocular redness. He had experienced a first episode of anterior uveitis in June 2022 and subsequently developed marked depigmentation of the hair, eyebrows, beard, and eyelashes beginning in July 2022. Ophthalmic examination and multimodal imaging demonstrated bilateral granulomatous panuveitis with active stromal choroiditis. Extensive infectious and inflammatory investigations were negative, while HLA-DRB1*04 testing was positive. A diagnosis of incomplete VKH disease was established. Despite treatment with intensive topical corticosteroids, systemic corticosteroids, mycophenolate mofetil, adalimumab, methotrexate, and repeated intravitreal dexamethasone implants, the patient developed recurrent inflammatory relapses and evolved toward a chronic-recurrent disease course. This case highlights the diagnostic value of early poliosis as a clinical clue to VKH disease, even in the absence of neurological prodromal symptoms. It also illustrates the potential for rapid progression toward chronic-recurrent inflammation despite aggressive multimodal therapy, emphasizing the importance of early recognition, close monitoring, and sustained treatment adherence.
Wang et al. (2026) studied this question.