Congenital ocular melanocytosis (COM) is an extremely rare melanocytic hyperplasia (0.038% in Caucasian population); it affects episclera and sclera. The conjunctival epithelium is not affected, as opposed to conjunctival melanosis. The pigmentation is grayish-blue and its consistency with the underlying tissues as the conjunctiva moves (deep pigmentation) is an essential diagnostic clue. COM may be isolated or associated with facial pigmentation, in the area innervated by the trigeminal nerve--known as oculodermal melanocytosis or nevus of Ota. COM may associate the following findings: iris hypercromia, iris mammillations, fundus hypercromia, uveal melanoma (most frequent choroidal melanoma) and glaucoma (10%). Melanocytes have been found in the brain, orbit, bucal mucosa in COM.
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