Nevus of Ota: What It Is and How It Affects the Eye

Nevus of Ota

Nevus of Ota is a benign pigmentary condition, but it is not trivial when the eye is involved. The skin patch is not a skin cancer. The important medical issues are inside the eye: a higher-than-usual risk of uveal melanoma, and a risk of open-angle glaucoma. Most people with this condition never develop either problem. The reason for eye care is that both can be silent, and both are easier to manage when found early.

What it is

Nevus of Ota, also called oculodermal melanocytosis, is a dermal melanocytosis. Pigment cells sit deep in the skin instead of in the surface layer, where melanocytes normally live. Ota described it in 1939. The older name is nevus fusco-caeruleus ophthalmo-maxillaris.

It appears as a flat blue, slate-gray, or brown patch on one side of the face, in the area supplied by the first and second branches of the trigeminal nerve. That area includes the forehead, eyelid, temple, cheek, and side of the nose. The lower jaw is usually spared.

The same kind of deep pigment may involve the eye on the same side. When the eye is involved and the skin is not, the condition is called ocular melanocytosis, or melanosis oculi. When both are involved, it is oculodermal melanocytosis. These terms are often used loosely. For risk, the distinction matters: the estimates below apply to people who have melanocytosis of the eye, not to a facial patch with a normal eye examination.

The color is blue or gray because the pigment is deep. Short wavelengths of light scatter on the way back out of the skin or sclera. This is the Tyndall effect. The pigment does not blanch with pressure. It is not a blood-vessel birthmark.

Who develops it, and when

Nevus of Ota is uncommon. It is seen most often in people of Asian ancestry and is more common in females. It is usually on one side. Both sides can be affected, but that is uncommon.

There are two typical times of onset. The patch may be present at birth, or it may appear around puberty. A patch that is already there can darken during puberty or pregnancy. It does not spread across the face like an infection, and it is not caused by trauma or poor hygiene.

It is almost always sporadic. It is not inherited in a simple family pattern, and genetic testing is not part of routine care. The pigment cells are neural-crest cells that remained in deeper tissue. In tissue that has been studied, many of these lesions carry non-inherited changes in GNAQ, a cell-signaling gene also commonly altered in uveal melanoma. That overlap helps explain the eye-cancer association. It does not mean the skin patch is a melanoma.

What is seen on the skin

The patch is flat, not a raised mole. Color can be uneven, and the border can be irregular. Extent varies from a small periocular patch to a large area of the forehead, eyelid, and cheek. Sun exposure may darken it. The pigment can also involve nearby mucosa, including the palate, or the eardrum on the same side. Those findings are uncommon and are part of the same condition, not a separate disease.

The skin lesion itself is benign. A new nodule, sore, or rapidly changing area within the patch is uncommon and should be examined. That is a different question from the usual flat discoloration.

What is seen in the eye

Eye involvement is frequent when it is looked for, and the sclera is the most recognizable site. The white of the eye shows slate-blue or gray patches. The pigment sits deep to the conjunctiva, in the episclera and sclera. A useful examination finding is that it does not move when the conjunctiva is slid with a cotton tip. A conjunctival nevus or another surface lesion moves with the conjunctiva. That distinction matters, because a new surface growth is not the same thing as stable deep pigment.

Other eye structures on the same side may be involved:

• The iris, which can make the two eyes different in color.
• The drainage angle, where fluid leaves the eye.
• The choroid, which often looks diffusely darker rather than like a single discrete freckle.
• Less often, other intraocular tissues.

Stable scleral pigment does not itself reduce vision. Most people with nevus of Ota see normally. Blurred vision, flashes, a new shower of floaters, or loss of side vision are not expected features of the birthmark. They are reasons to be examined promptly.

Conditions that look similar

Condition	Usual appearance	Eye
Nevus of Ota	One-sided blue-gray patch of the forehead, eyelid, or cheek	Often involved
Ocular melanocytosis	No facial patch	Involved by definition
Nevus of Ito	Same deep pigment on the shoulder, neck, or upper arm	Not an eye condition
Hori nevus	Small blue-brown spots on both cheeks, usually in adult women	Typically spared
Mongolian spot	Blue-gray patch on the lower back or buttocks in infancy; often fades	Not involved
Port-wine stain	Flat red or purple patch that blanches	A different disease; glaucoma can occur if it surrounds the eye

Hori nevus is also called acquired bilateral nevus of Ota-like macules. It is not nevus of Ota, and it does not carry the same eye associations. A port-wine stain can share the problem of glaucoma when it involves the eyelid, but it is a vascular birthmark, not melanocytosis.

Uveal melanoma

Uveal melanoma is the main serious association. It is a cancer of the pigment layer inside the eye, most often the choroid. When it occurs in this setting, it is almost always in the eye on the same side as the melanocytosis.

The risk is higher than in the general population, but the absolute chance remains low. A widely cited 1998 estimate by Singh and colleagues put the lifetime risk at about 1 in 400 for a white person with ocular or oculodermal melanocytosis, compared with about 1 in 13,000 for white people overall. That is roughly a thirtyfold increase. It is an estimate from prevalence data, not a count from a lifelong study of one clinic cohort.

Two limits of that figure should be stated plainly. First, it was calculated for white patients. Nevus of Ota is more often seen in people of Asian ancestry, in whom uveal melanoma is less common, so that number should not be used as a personal risk. Second, it applies to melanocytosis involving the eye. It should not be quoted to someone who has only a cutaneous patch and a normal, careful eye examination.

Even among white patients, a risk of about 1 in 400 means that the great majority never develop an eye melanoma. Surveillance is recommended because the increase over baseline is real and the cancer is serious, not because melanoma is the expected outcome.

If a uveal melanoma does arise in an eye with melanocytosis, large ocular-oncology series, including work from Shields and colleagues published in 2013, have found a higher risk of metastasis than in uveal melanoma without melanocytosis. This is another reason an ocular oncologist, not only a general eye clinic, should manage a suspicious lesion.

Glaucoma

Melanocytosis can clog the eye’s drainage angle with pigment cells and cause secondary open-angle glaucoma. Pressure can rise with no pain, no redness, and no early change in vision. Waiting for pain is not a safe plan. Angle-closure glaucoma is not a typical feature of this condition.

Glaucoma risk, like melanoma risk, depends on eye involvement. A skin patch alone does not raise eye pressure. Treatment, when needed, follows ordinary glaucoma care: pressure-lowering drops, laser, or surgery, chosen by the ophthalmologist. Treating the facial patch does not treat the angle.

Uncommon risks outside the eye

Melanoma arising in the skin portion of nevus of Ota is reported but rare. Melanocytosis can, rarely, involve the meninges on the same side and give rise to a meningeal melanocytoma or melanoma. These are not expected findings. They are reasons to take new neurologic symptoms seriously, not reasons to screen every patient with scans.

How the diagnosis is made

The diagnosis is clinical. A classic facial patch does not need a biopsy. The eye evaluation is the part that changes management.

A baseline examination should include vision, slit-lamp examination of the sclera and iris, intraocular pressure, gonioscopy of the drainage angle, and a dilated look at the choroid. Photographs are useful so later exams have something to compare.

Ultrasound, optical coherence tomography, autofluorescence, and angiography are for a suspicious spot or for measuring a lesion. They are not required to “confirm” uncomplicated nevus of Ota. A flat area of choroidal melanocytosis is not the same thing as a raised tumor. A new or growing elevated lesion, or one with orange pigment or fluid, needs an ocular oncologist.

Treatment of the skin

No treatment is medically required for a stable patch. The eye risk is not removed by lightening the skin.

Options, if the appearance bothers the patient, are:

• Camouflage makeup, which does not change the condition.
• Laser treatment of the skin. Q-switched ruby, alexandrite, and Nd:YAG lasers, and picosecond lasers, can lighten dermal pigment. An early demonstration of this approach was Q-switched ruby laser treatment reported by Watanabe and Takahashi in 1994. Several sessions are usually needed. Clearing is often incomplete, the patch can darken again, and lighter or darker spots can remain, especially in darker skin. The eye must be shielded. Laser settings belong to the treating clinician.
• Excision is rarely a good choice for a large facial area because of scarring.

Creams meant to bleach surface pigment do not reach these deep cells and are not effective treatment. Laser must not be aimed at the sclera or into the eye. Skin treatment does not lower the risk of glaucoma or uveal melanoma, because the intraocular pigment cells remain.

Eye care over time

Anyone with nevus of Ota should have at least one complete eye examination when the diagnosis is recognized, including children. Infants and young children can be examined; how that is done depends on age.

If the eye is involved, follow-up continues even when vision is perfect. Many specialists examine a stable eye about once a year, and sooner if pressure is high, the choroid is heavily involved, or a lesion is being watched. There is no single schedule that fits every patient. The ophthalmologist who has seen the eye should set the interval. Expert practice is not the same thing as a universal mandated protocol.

A person with a facial patch and a truly normal eye examination still needs that baseline exam, so “normal” is a finding rather than an assumption. After that, the clinician can decide how often to repeat it. The 1-in-400 estimate should not be applied to that person.

Seek an examination promptly for any of the following:

• New blur, flashes, many new floaters, a curtain over the vision, or loss of side vision.
• A new dark spot on the colored part of the eye, a raised spot on the surface of the eye, or a change in pupil size or shape.
• Eye pain, halos, or redness. These can occur with high pressure, but their absence does not mean pressure is normal.
• A nodule, ulcer, or rapidly changing area in the skin patch.
• New neurologic symptoms such as persistent headache, vomiting, or seizures, which are rarely related but should not be ignored.

What to remember

• The facial patch is benign deep pigment, not a melanoma.
• The medical issue is the eye on the same side, when the eye is involved.
• Uveal melanoma is more common than in the general population and still uncommon in absolute terms.
• The often-quoted lifetime figure of about 1 in 400 is for white patients with eye involvement. It is not a personal number for every reader.
• Open-angle glaucoma can occur and is often silent. Skin laser does not prevent it.
• A baseline dilated examination, pressure check, and gonioscopy are the starting point. Imaging and oncology referral are for suspicious findings, not for every diagnosis.
• Most people with nevus of Ota keep normal vision. The goal of follow-up is to keep it that way.

This is general information, not a diagnosis or a treatment plan. Care belongs with a dermatologist for the skin and an ophthalmologist for the eye, and with an ocular oncologist if an intraocular lesion looks suspicious.

Sources

The risk figure and the laser landmark cited above are:

Singh AD, De Potter P, Fijal BA, Shields CL, Shields JA, Elston RC. Lifetime prevalence of uveal melanoma in white patients with oculo(dermal) melanocytosis. Ophthalmology. 1998;105(1):195-198.

Watanabe S, Takahashi H. Treatment of nevus of Ota with the Q-switched ruby laser. New England Journal of Medicine. 1994;331(26):1745-1750.

The higher chance of metastasis when uveal melanoma arises in an eye with melanocytosis is reported in large ocular-oncology series, including Shields CL, et al., JAMA Ophthalmology, 2013;131(8):993-1003. The association of oculodermal melanocytosis with uveal melanoma was established earlier, including by Gonder JR, Shields JA, Albert DM, Augsburger JJ, and Lavin PT, Ophthalmology, 1982. Other statements in this article are standard clinical descriptions of the condition, not new numerical claims.