Case 126 Teaching Points

Severe, bilateral granulomatous panuveitis associated with poliosis, vitiligo, alopecia and central nervous system and auditory signs
Mechanism thought to be an autoimmune process directed against melanocytes
Epidemiology: 20-50-year-olds, women greater than men
Predilection for pigmented races: Asians, Hispanics, American Indians, Asian Indians, and African Americans
Positive HLA-Dr4 in 88%
Ocular findings include bilateral iridocyclitis, exudative retinal detachments, choroidal nodules and sunset-glow fundus
Treatment includes topical, sub-Tenon’s, and systemic steroids
For refractory cases, use cyclosporine, azathioprine, cyclophosphamide, or chlorambucil

References

Moorthy RS, Inomata H, Rao NA. Vogt–Koyanagi–Harada syndrome. Surv Ophthalmol 1995;39:265–292.

Last Modified: Wednesday, 28-May-2008 21:17:53 EDT