An HLA-B27 positive, 18-year-old male patient was diagnosed with X-linked chronic granulomatous disease (CGD) two years after developing recurrent bronchitis and skin abscesses. He had multiple infections including fungal pneumonia, hepatic abscesses and CGD-associated colitis treated with intensive immunosuppression.

His ophthalmic history included bilateral, recurrent anterior uveitis controlled with topical corticosteroids, and bilateral chorioretinal scarring. His last ophthalmic examination revealed bilateral keratoconjunctivitis and anterior uveitis with fibrin deposition on the anterior lens capsule (fig 1A). There were multiple oval regions of choroidal and retinal …