Progressive confluent circumpapillary multiple evanescent white-dot syndrome

To describe a morphologic variant of the multiple evanescent white-dot syndrome that can mimic other conditions.

We examined three patients with severe cases of unilateral multiple evanescent white-dot syndrome characterized by an atypical progressive circumpapillary discoloration of the fundus.

The confluent circumpapillary lesion progressed toward or beyond the equator of the fundus, raising initial concern of a viral retinitis. However, pinpoint dots at the leading edge evolved into the typical wreath-like spots of multiple evanescent white-dot syndrome, which then coalesced into the advancing edge of a geographic retinitis or retinal pigment epitheliitis, before spontaneous resolution.

Progressive geographic circumpapillary discoloration, appearing as a giant white spot, occurs rarely in severe cases of multiple evanescent white-dot syndrome. The distinctive appearance may suggest a disorder other than multiple evanescent white-dot syndrome, which can make initial diagnosis more difficult and lead to unnecessary or inappropriate testing and treatment.