This article is a brief overview of disorders of pigmentation of the skin that have associated ocular abnormalities. The clinical entities are categorized as either congenital, hereditary (genodermatoses), or acquired. In some disorders many of the eye abnormalities can be explained by a common pathogenic mechanism that involves the melanocytes in the skin as well as the melanocytes in the uveal tract and retinal pigment epithelium. The eye findings in the neurocutaneous syndromes are a reflection of the neural crest origin of cutaneous melanocytes.
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