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Dermal Melanocytoses

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Pathology of Pigmented Skin Lesions
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Abstract

Nevus of Ota: Also known as oculodermal melanocytosis or nevus fuscoceruleus opththalmomaxillaris, it presents as a diffuse, slightly speckled, unilateral macular lesion of bluish to dark-brown pigmentation of the skin in the area of the ophthalmic and maxillary divisions of the trigeminal nerve. The conjunctiva and sclera can be involved in more than half of the cases and occasionally the mucous membranes of the nose, ear, and oral cavity. Nevus of Ota can be bilateral in up to 10 % of cases [1, 2]. Other affected areas include the forehead, the temple, the cheeks, and the nose. The cutaneous and mucosal discolorations in nevus of Ota are present throughout life and do not usually fade away with time; however, color can vary in intensity. These lesions are most commonly seen in Asians and in darker-skinned individuals and are uncommon in white patients. Most nevi of Ota present at birth (60 % of cases) and the second peak is seen during adolescence with a female predominance (4:1 ratio). Rare acquired cases with adult onset are sometimes considered as a separate disease, such as Hori nevus (acquired bilateral nevus of Ota-like facial macules). Malignant degeneration in a nevus of Ota is extremely rare [3–6]. The choroid is the most common site affected, but the uvea, iris, and optical tract can also be involved. Although nevus of Ota is much more prevalent in Asians, malignant change appears to be much more common in white patients [5, 6]. Nevus of Ota can be associated with meningeal melanocytosis, Sturge-Weber disease, Klippel-Trenaunay syndrome, and spinocerebellar degeneration.

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Plaza, J.A., Prieto, V.G. (2017). Dermal Melanocytoses. In: Pathology of Pigmented Skin Lesions. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-52721-4_2

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