An Update on Oculodermal Melanocytosis and Rare Associated Conditions.

Oculodermal melanocytosis (ODM) is a rare disease, which is characterized by hyperpigmentation of facial skin and several parts of the eye, such as the sclera, conjunctiva, cornea, iris, ciliary body, and choroid. The condition usually affects the Asian female population. The most typical presenting ocular sign is iris heterocromia. Iris hyperpigmentation may be associated with iris mammillations, which are dome-shaped protuberations of the iris surface. They are linked to a higher risk of malignant transformation when present in patients with ODM. Glaucoma is a complication of ODM and is caused by angle abnormalities or mechanical occlusion by melanocytes in an open irido-corneal angle. Choroidal and ciliary body melanoma have a higher incidence in this condition characterized by melanocytosis. Patients presenting ODM should undergo routine ophthalmological examination in order to carefully monitor for glaucoma and melanoma.