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

Abstract

• Blue nevi arising in the sclera are uncommon, with only three prior cases documented in the literature, none of which arose within an area of ocular melanocytosis. • Oculodermal melanocytosis, a subtype of congenital dermal dendritic melanocytic proliferation, is well known to carry a risk (1/400) of uveal melanoma. Novel Insights • To our knowledge, this case is the first report of a scleral blue nevus arising within an area of oculodermal melanocytosis, suggesting a potential association between the two subtypes of congenital dermal dendritic melanocytic proliferations. • Although generally considered a benign condition, the cellular atypia and scleral expansion noted in our case may represent an intermediate stage in the potential progression from ocular melanocytosis towards melanoma.