Sir,

Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a paraneoplastic syndrome described by Machemer1 in 1966, in which an underlying tumor causes diffuse bilateral proliferation of melanocytes in the uvea. Gass et al.2 later described five cardinal signs related to BDUMP: multiple red patches at the level of the RPE, corresponding early hyperfluorescence of these patches on fluorescein angiography, development of multiple pigmented and non-pigmented uveal melanocytic tumors with diffuse thickening of the uveal track, exudative retinal detachment, and rapidly progressive cataracts. Patients affected by BDUMP have a short life expectancy.2 We, however, report a case of BDUMP, which illustrates that long-term survival can occur despite multiple metastases.

Case report

A 62-year-old man was referred for cataract surgery to both eyes. At presentation, his right visual acuity was 0.40 logMAR and left visual acuity was 0.80 logMAR. He had a past medical history of transitional cell carcinoma of the bladder, with multiple metastases treated with surgical excision and chemotherapy. At preoperative review, his vision decreased to hand movements OD and counting fingers OS due to rapid progression of the cataracts. Postoperative examination revealed BDUMP (Figure 1). Further investigation discovered new metastases. Four years later, his visual acuity is 0.12 logMAR OD and 0.0 logMAR OS and some of the choroidal lesions have reduced in size.

Figure 1
figure 1

(a) Right fundus colour photograph at the time of the diagnosis showing myelinated fibers at the optic disc, two flat uveal pigmented lesions and one not pigmented in the superior nasal quadrant. (b) same quadrant, 4 years from the diagnosis with the same uveal lesions reduced in size. (c) left fundus colour photograph of the supero-temporal quadrant showing four flat pigmented uveal lesions, which decreased in size (d) 4 years after the diagnosis of bilateral diffuse uveal melanocytic proliferation.

Full size image

Comments

In our case, with hindsight, it was clear that the rapidly progressing cataracts were part of this paraneoplastic syndrome. Though regression has been reported after removal of the underlying cause,3 in our case, only some of the lesions showed a decrease in size. Duong et al.4 reported the longest survivor of this disorder (>102 months), but their patient underwent bilateral enucleation; our report shows a case of BDUMP with survival of over 4 years and a good visual acuity.

Ophthalmologists should be aware of the importance of the recognition of BDUMP ocular signs. Monitoring these patients with regular follow-ups will help to detect new lesions and the reduction in size of existing ones. BDUMP can therefore act as a biomarker in the management of cancer, especially when a primary tumor is not detected.