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Nevus of Ota in Child
See also in: External and Internal Eye
Other Resources UpToDate PubMed

Nevus of Ota in Child

See also in: External and Internal Eye
Contributors: Nnenna Agim MD, William M. Lin MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed


Nevus of Ota (also known as oculodermal melanocytosis) presents with facial dermal melanocytosis representing mosaic mutations in GNAQ or GNA11, BRAF, or NRAS. Clinically, dark blue / slate gray to dark brown / black macules with a speckled or homogenous pattern coalesce into a segmental patch involving the upper facial segment(s) unilaterally or bilaterally. Extension into the oral or nasal mucosal cavity and onto the sclerae may also be present.

The visible pigment arises from an increased number of dermal dendritic melanocytes, and the blue and gray color seen is secondary to the Tyndall effect. Nevus of Ota occurs with greater frequency in patients with Asian or African ancestry. The mutations driving nevi of Ota may result in clinical manifestations as soon as early infancy. Darkening and extension may occur with ultraviolet (UV) radiation exposure and with hormonal influences around puberty.

Malignant transformation is very rare, but melanoma has been reported, possibly due to a second-hit mutation in the same molecular pathway. Importantly, at least 2 pediatric patients (aged 16 years and 18 years) and a few more patients aged younger than 30 years with nevi of Ota have been reported as having developed facial melanoma. At least 18 cases of central nervous system melanoma have also been reported in patients with nevi of Ota; the youngest patient was 21 years old. Ipsilateral glaucoma is a contiguous potential association in 10% of patients with nevi of Ota. The mutations associated with nevi of Ota have also been found in uveal and remote cutaneous melanomas, which may occur in 1:400 cases. Two 24-year-old patients with nevi of Ota developed cutaneous melanomas with local and distant metastases. A 29-year-old patient with a nevus of Ota developed a retro-orbital ocular melanoma that metastasized to the brain. Meningeal melanocytomas have also been associated with nevi of Ota; they are usually located ipsilateral to the nevus.

Nevus of Ota may be a feature of phakomatosis pigmentovascularis, which has also been determined to feature GNAQ / GNA11 mutations in some cases.


D22.39 – Melanocytic nevi of other parts of face

254817005 – Nevus of Ota

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Differential Diagnosis & Pitfalls

  • Dyspigmentation from alkaptonuria (congenital ochronosis) or acquired ochronosis (occurring after high concentrations of hydroquinone) may have a similar hue but lack a trigeminal distribution.
  • Blue nevus / blue nevus of the conjunctiva may have similar blue-gray pigmentation but is a smaller, well-demarcated macule or papule.
  • Lentigines or giant café au lait macules impart a tan to brown hue; the latter is usually lighter in color than a nevus of Ota.
  • Drug-induced pigmentation can either be diffuse or photodistributed.
  • Ecchymoses (see traumatic purpura) will have a history of trauma.
  • Arteriovenous malformations are typically deep red; venous malformations may be segmental and have a bluish cast.

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Last Reviewed:08/11/2021
Last Updated:08/15/2021
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Patient Information for Nevus of Ota in Child
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Contributors: Jessica R. Williams, Rawn Bosley MD
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Nevus of Ota in Child
See also in: External and Internal Eye
A medical illustration showing key findings of Nevus of Ota : Bulbar conjunctiva pigmented macule, Hyperpigmented patch, Unilateral distribution
Clinical image of Nevus of Ota - imageId=1469618. Click to open in gallery.  caption: 'A large dark gray patch on the forehead and temple, extending into the scalp.'
A large dark gray patch on the forehead and temple, extending into the scalp.
Copyright © 2023 VisualDx®. All rights reserved.