Purpose: To assess in patients followed in a French referral center the clinical spectrum of Vogt-Koyanagi-Harada (VKH) disease and the HLA-DRB1*04 genotype.
Methods: Patients previously diagnosed as having VKH disease were re-evaluated in a cross-sectional study using the VKH Committee's revised criteria. High-resolution HLA-DRB1 genotyping was performed.
Results: Eleven white patients satisfied ophthalmologic diagnostic criteria. All originated from Mediterranean countries. Nine and 3 patients had neurologic and/or cutaneous abnormalities, respectively. Among DRB1*04-positive patients, the HLA-DRB1*0405 subtype was 71%.
Conclusion: These VKH patients predominantly had an incomplete form. The HLA-DRB1*0405 subtype allele was enriched in a group of Mediterranean stock.