Ocular melanocytosis (melanosis oculi) is a congenital focal proliferation of subepithelial melanocytes characterized by unilateral patchy but extensive slate-gray or bluish discoloration of the episclera (Fig 20-14). Intraocular pigmentation is also increased, which is associated with a higher incidence of glaucoma and risk of malignant melanoma. Some patients, particularly persons of Asian ancestry, may have associated involvement of eyelid and adjacent skin with dermal hyperpigmentation that produces brown, bluish, or black discoloration without thickening (oculodermal melanocytosis, nevus of Ota). Small patches of slate-gray scleral pigmentation, typically bilateral and without clinical significance, are common in black and Asian children. Melanosis of skin and sclera is occasionally associated with Sturge-Weber syndrome and Klippel-Trénaunay-Weber syndrome.
Figure 20-13 Pigmented nevus of the bulbar conjunctiva, right eye.
Figure 20-14 Ocular melanocytosis.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.