Intra-Scleral Blue Nevus Arising within Oculodermal Melanocytosis: Melanoma or Not?

We report a 61-year-old female who presented to our service with recent growth of a thickened, pigmented, sub-conjunctival lesion within an area of ocular melanocytosis in her left eye. Lamellar sclerectomy was performed. Histopathological assessment revealed a localized melanocytic proliferation with features of blue nevus arising within the area of ocular melanocytosis. There was a small zone of cells showing cytological atypia and expansion of the scleral stroma. We believe this case to be the first report of intrascleral blue nevus arising within an area of ocular melanocytosis. Given the cytological atypia, the blue nevus may perhaps represent an intermediate stage within a progression from ocular melanocytosis towards melanoma. Patients with oculodermal melanocytosis merit regular long-term surveillance for early detection of melanoma, not only in the uvea, but also in the orbit and potentially the ocular surface.