Bilateral diffuse uveal melanocytic proliferation is a rare paraneoplastic syndrome that presents with bilateral progressive loss of vision. A 70-year-old woman presented with a 3-month history of progressive, bilateral vision loss. The patient had bilateral, diffuse, shallow, subretinal fluid with patchy, reddish-brown lesions at the level of the retinal pigment epithelium (RPE) that showed significant early hyperfluorescence on fluorescein angiography and a corresponding loss of autofluorescence. Optical coherence tomography of both eyes revealed complete RPE and inner segment/outer segment junction loss with adjacent areas of thickening at the level of the RPE. Bilateral diffuse uveal melanocytic proliferation was diagnosed based on these clinical findings, and a systemic evaluation for malignancy revealed metastatic endometrial adenocarcinoma. Both autofluorescence and optical coherence tomography demonstrated unique imaging characteristics that correlated with the reported histopathology of bilateral diffuse uveal melanocytic proliferation. These imaging modalities can contribute to the rapid and accurate diagnosis of bilateral diffuse uveal melanocytic proliferation.
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