A congenital pigmentary lesion, ocular melanocytosis (melanosis oculi) is characterized by unilateral patchy but extensive slate-gray or bluish discoloration of the sclera (not conjunctiva). Intraocular pigmentation is also increased, which contributes to a higher incidence of glaucoma and increases the 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 or other abnormality (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 (Fig 26-18).
, GreenbergMF, PollardZF, GrossniklausHE. Conjunctival melanoma in a child.2004;41(1):56–58.