Presumed atypical peripapillary Vogt-Koyanagi-Harada disease

To describe a case of bilateral presumed atypical Harada disease with sequential, not simultaneous, involvement of the peripapillary retina (subretinal fluid) in a healthy patient with no systemic complaints.

A 35-year-old healthy white man presented with sudden paracentral visual loss in the left eye. His medical history was unremarkable. However, he reported a similar episode 20 months earlier in the right eye that was associated with macular serous retinal detachment. The right eye showed evidence of reactive peripapillary atrophy and pigmentary alteration in the macula. Optical coherence tomography scans of the posterior left eye segment revealed a diffuse thickened choroid, papillomacular subretinal exudate and discontinuity of the ellipsoid layer with suggestion of vitreous cellularity. Autofluorescence imaging of the left eye showed peripapillary hyperautofluorescence. A fluorescein angiogram revealed progressive staining and pooling of the peripapillary retina with corresponding retinal vasculitis. Indocyanine green angiography revealed multiple hypocyanescent lesions with an area of hypercyanescence temporal to the disc. Rheumatologic evaluation and laboratory tests were all negative. Chest tomography was normal. Considering the apparent absence of infectious diseases, the patient was started on 60 mg/day prednisone. After 8 days, visual acuity improved to 20/250, improving to 20/20 vision six months after a slow steroid wean.

We believe our case represented a variant of the Vogt-Koyanagi-Harada syndrome in an atypical situation, because the patient fulfilled the presumed criteria. Furthermore, the findings of clinical and complementary examinations led to this nosological entity to the exclusion of others.

The point of this case is to alert ophthalmologists to the existence of this atypical presentation of the disease so that it should be included among the differential diagnoses of pathologies that present with these findings.