Bilateral Diffuse Uveal Melanocytic Proliferation With a Positive Ophthalmoscopic and Visual Response to Plasmapheresis

Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a paraneoplastic syndrome resulting in profound bilateral vision loss, with approximately 30 cases reported in the world’s literature. In 1990, Gass et al1 described 5 characteristic signs of the disease: multiple subretinal round red patches in the retinal pigment epithelium (RPE), early fluorescence of these lesions on fluorescein angiography, multiple elevated pigmented and nonpigmented uveal melanocytic tumors with diffuse uveal tract thickening, exudative retinal detachments, and rapid cataract development. The histopathologic findings include diffuse uveal infiltration by benign hypopigmented spindle cells and occasional epithelioid cells. There is focal infiltration of the choroid by heavily pigmented melanocytes with sparing of the choriocapillaris.1–4 Destruction of the RPE occurs in areas overlying the infiltrate.1 Treatment for BDUMP has been largely unsuccessful. Modalities have included corticosteroids, ocular surgery, ocular radiation, and treatment of the underlying malignant neoplasm.1–3,5 While some have shown transient vision improvement or stabilization, we describe a new treatment for this visually devastating condition that resulted in vision improvement and stability with continued treatment until the patient’s death.