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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
Copyright © 2023 VisualDx®. All rights reserved.
Patient Information for Nevus of Ota in Child
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Contributors: Jessica R. Williams, Rawn Bosley MD


Nevus of Ota occurs when pigment-producing cells (melanocytes) occur in the deeper layers of the skin (dermis). The resultant patch may appear slate gray, blue gray, brownish gray, or blackish. It typically occurs on one side (unilateral) of the face and can be seen on the forehead and/or on the cheek between the eye and the upper lip.

Nevus of Ota is mostly asymptomatic but can rarely be associated with a loss of sensation in the areas of the face with skin darkening (hyperpigmentation). Nevus of Ota is typically noncancerous (benign).

The exact cause is unknown, but ultraviolet (UV) radiation and/or hormones may darken lesions.

It occurs during infancy or adolescence and can become darker and larger with age after puberty.

Potential complications of nevus of Ota include increased eye (ocular) pressure, glaucoma (occurs in 10%), and melanoma (cancer) of skin or eye (occurs in 1 in 400 individuals with nevus of Ota).

Precautions include yearly glaucoma screening and melanoma screening.

Who’s At Risk

Nevus of Ota most frequently affects people of Asian or African descent and is most common in women.

Signs & Symptoms

It presents as a grayish blue patch on the forehead, around the eye, and/or on the cheek.

Self-Care Guidelines

  • Makeup can be applied over the patch(es) cosmetically.
  • Chemical bleaching products (chemical peels, topical hydroquinone cream) can be used to lighten the areas of skin affected but may have a risk of permanent skin lightening (hypopigmentation).
  • If you have a nevus of Ota, it is important to schedule visits with your ophthalmologist and dermatologist biannually to avoid potential complications. It is also important to visit your dentist to screen for mucosal involvement of the lesion.
  • Examine your skin to look for new lesions or any lesions that have changed in size, shape, or color, and visit a dermatologist if any are seen.

    When to Seek Medical Care

    • Enlargement of the area of skin darkening.
    • Change in color of the pigmentation of the skin.
    • An open sore (ulceration) of the affected area.
    • Loss of sensation (paresthesia) of the affected area.


    Your dermatologist may use laser therapy for cosmetic treatment to destroy the melanocytes that cause the skin darkening.

    Your physician may request regular skin checks.
    Copyright © 2023 VisualDx®. All rights reserved.
    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.