All About Nevus of Ota: Diagnosis, Treatment, Prevention

Nevus of Ota or oculodermal melanocytosis is a type of melanocytosis. A condition indicated by brown, blue-green, or grey patches in the eye and surrounding areas. In most cases, the nevus of Ota is developed from birth or during adolescence. 

Nevus of Ota characterized by hyperpigmented blue-gray patches on the face of a patient

Patients suffering from the nevus of Ota have increased amounts of melanin (pigment) and melanin-producing  pigment cells (melanocytes). The patches appear due to entrapped melanocytes in the tissues. 

Hyperpigmentation can appear in the whites of the eye (sclera), intraocular blood vessel area called the uvea (ciliary body, choroid, and iris). There are many forms of hyperpigmentation. It may also infect the eyelid, mouth, and other parts of the body.

While the patches are generally a cosmetic concern, they may transform into malignant melanoma. Nevus of Ota is mostly benign, however, the patients are more likely to develop intraocular and central nervous system malignant melanomas if left untreated. 

Dermatologists can diagnose the condition and suggest various treatment options. Patients suffering from the nevus of Ota must get their skin and surrounding regions examined by an ocular oncologist (eye-cancer specialist) and a neurologist periodically. A dermatologist or an ophthalmologist is useful in offering treatments for the condition. Treatment depends upon the severity and type of lesions developed on the face. 

What are the Symptoms of Nevus of Ota?

Unilateral presentation is commonly observed, meaning Nevus of Ota usually develops on one side of the face. If it is present bilaterally, it is called the nevus of Hori. 

Nevus of Ota is usually asymptomatic, although in few cases sensory loss is reported. It is mainly characterized by bluish hyperpigmentation along the trigeminal nerve. Patches are confluent, non-hairy, flat with poorly defined margins. In some cases, pigmentation can affect the oral cavity and nasal mucosa.  

The melanocytes are entrapped causing hyperpigmentation which usually appears to be patchy brown, slate blue, or grey-black appearance. It can affect the following regions in your body, 

  • Eyelids
  • Nose
  • Forehead
  • Cheeks
  • Temple
  • Side of the face
  • Whites of the eye
  • Iris
  • The area around the eye

Pigmentation develops in the area of your face controlled by the trigeminal nerve. The trigeminal nerve is located in the side of the face and is responsible for sending pain, touch, and temperature sensations from your face to the brain. 

Nevus of Ota causes thickening of the tissue in and around the eye.

Nevus of Ota develops during childhood, it can darken and grow with age. Nevus of Ota can also develop in adolescence. The color of the pigment can change depending on various factors such as hormones, weather, or illness. 

The condition is benign, it does not spread to other parts of the body. Nevus of Ota is not contagious, it does not spread by touch. These symptoms are noticed on a patient's face and can have a physiological impact on them. 

How is Nevus of Ota diagnosed?

If you notice any bluish-green or grey patches on your face, you must consult your doctor. Your dermatologist will examine the affected region and diagnose the condition.

Careful examination of your eye and the surrounding region, especially eyelids and episcleral layer is diagnostic of the nevus of Ota. Your doctor may recommend a biopsy to identify the presence of heavily pigmented melanocytes. 

Your doctor may use a biomicroscope to thoroughly examine the whites of your eye. Your dermatologist may even dilute your eyes and use ophthalmoscopy to look at the back of your eyes. 

They may even recommend an ultrasound of the eye to further investigate the condition. Ultrasound imaging will reveal hyperpigmentation in the uveal layer of the eye. It is important to examine the anterior and posterior of the eye in patients, ultrasound can enable anterior and posterior imaging. 

Differential Diagnosis

Differential diagnoses are the list of possible conditions or diseases that could be causing the symptoms of Nevus of Ota. It is important to correctly diagnose the condition to begin proper treatment. 

Nevus of Hori- The condition is very similar to nevus of Ota. While the nevus of Ota is unilateral (affects only one side of the face), the nevus of Hori affects bilaterally, both sides of the face. 

Nevus of Ito- Patients develop similar hyperpigmented patches on their arms, shoulders, neck, and underarms.

Hyperpigmentation is always caused by dermal melanocytosis. Some medications such as minocycline, amiodarone, and gold can cause heavy pigmentation as a side effect. 

In some cases, patients can discover that they have melasma, a skin condition that causes dark, discolored patches on the skin. Melasma is caused due to hormonal changes during pregnancy or due to sun exposure. 

Melasma can develop due to the following reasons:

  • UV radiations
  • Pregnancy
  • Hormonal changes
  • Hypothyroidism
  • Few medications

Erythema can also cause benign patches and lesions on the skin. A person who has sustained burns or physical trauma may also have hyperpigmentation of the tissues as they heal from the skin damage. 

Mongolian spots, in this condition lesions, are present in the lumbosacral region and rarely on the face. These lesions disappear on their own by the age of 3 to 6 years. 

Ecchymosis is a discoloration underneath the skin due to physical trauma. Black or blue patches are observed, which undergo color changes as they heal. Bleeding under the skin is commonly observed in Ecchymosis.

Vascular malformations may also be present in some patients. 

Dermal melanocytosis can occur anywhere on the body, including inside the mouth. Other forms of dermal melanocytosis may also be observed in other regions of the body. 

What are the causes of Nevus of Ota?

There is no definitive cause of nevus of Ota, various hypotheses are proposed by researchers who have tried to identify the cause of developing Nevus of Ota.

Few studies indicate a genetic mutation resulting in the melanoblast pool. Others argue that radiations, radiotherapy, or hormonal changes are the main cause of the condition. Another hypothesis states a disturbance in the pigment cell pathway since birth.

Nevus of Ota is more prevalent in females than males, a 5:1 ratio is reported. Asian or African women are more likely to suffer from the condition. 

Nevus of Ota is more common in people with dark-skinned ethnic backgrounds. Around 0.014%- 0.034% of the Asian population is affected by the nevus of Ota. Nevus of Ota can be present in people with different skin pigmentations. 

White people are less likely to develop nevus of Ota. Although they are more susceptible to developing a malignant melanoma which is associated with this condition. 

Sometimes the nevus of Ota is found associated with the nevus of Ito. 

Treatment of Nevus of Ota

There are a couple of ways to reduce hyperpigmentation. Let’s take a look at the ones specific to Nevus of Ota.

Laser Therapy

Before and after image of treatment using laser therapy

For Nevus of Ota, laser treatment has proven to be the most effective, however, multiple sessions and applications are required to correct the patches. The laser destroys the melanocytes that cause hyperpigmentation, to restore natural skin pigmentation. 

Laser therapy is more effective in people with lighter skin tones. Laser therapy may leave scars behind and multiple sessions are required as hyperpigmentation can reappear. In a few cases, nevus of Ota can return as hypo or hyper-pigmentation (light or darker patches as compared to the original hues) in patients.  

As there are few limitations of laser therapy, many patients opt for cosmetic products over laser treatment. Foundations, camouflaging creams, or concealers can hide the affected region well. Various color-correcting products and contouring products also help conceal nevus of Ota. 

Malignant melanomas require treatment, it depends on the size of the patch and the extent of invasion. Your doctor may perform a Mohs micrographic surgery to remove the lesions and reconstruct the surrounding tissue. For some cases, your doctor may even recommend radiotherapy, thermotherapy, or chemotherapy. 

Ophthalmic 

Ophthalmological screening and recording of vision are performed to rule out uveal melanomas. Uveal melanoma will require treatment based on its size and location. Doctors may perform radiotherapy, surgical resection, enucleation, or thermotherapy. 

Studies reveal that less than 4% of people can develop choroidal melanoma in the affected eye. Patients are recommended to have their pigmentations examined by their ophthalmologist periodically to rule out complications. 

Alternative treatment

There are many minimally invasive treatment options available to treat nevus of Ota. Dermatologic treatments involve the following alternatives,

  • Chemical peels- In this method, a chemical is applied to your skin, which makes it blister and eventually peel off.
  • Dermabrasion- It is also called surgical skin planing. In this technique, your dermatologist uses a specialized instrument to ‘sand’ the skin. The topmost layer of the skin is scraped away to unveil smooth new skin. 
  • Electrocautery- In this procedure heat from electricity is used to destroy abnormal tissue. The electric current passes through an electrode which is placed near the affected tissue. This procedure is used to treat lesions and tumor cells.
  • Cryotherapy- This procedure involves using very low temperatures to remove abnormal tissues. Mostly liquid nitrogen or argon gas is used for this procedure. 

Can Nevus of Ota cause other complications?

The main social impact of Nevus of Ota is a cosmetic concern. Malignancies are rare. 1 in 400 patients develops malignancies. In rare cases, it may affect the optic disc. 

There is a 10.3% risk of developing glaucoma. In some patients, other complications such as increased intraocular pressures are also observed. For these complications, periodic examination by your doctor is highly recommended for patients suffering from the condition. 

The follow-up care prescribed by doctors should be religiously followed by patients with increased intraocular pressure. 

Overall, in the majority of patients, the lesions are benign with an excellent prognosis without treatment. Yearly screening by your dermatologist and ophthalmologist will rule out the chances of developing glaucoma or melanomas. 

Should I be worried if I am suffering from Nevus of Ota?

Patients should be taught that Nevus of Ota is benign lesions that appear to be seen as hyperpigmented blue-green or grey patches on the face. Patients should bear in mind that malignancies are very rare. Patients should seek medical consultation if any changes in the size of the lesion or any discolorations are seen. Periodic follow-up visits with your doctor are recommended to measure intraocular pressures. 

What is the cost of treating Nevus of Ota?

The cost of treatment depends on the type of treatment chosen. Laser treatments are relatively costly as compared to removal of nevus of Ota. The cost of treatment of nevus of Ota also depends upon the type of lesion, size of the lesion, and its invasion on the skin surface.

There are chances of recurrence, hence follow-ups are recommended to rule out any symptoms or further complications.  

What is the outlook for Nevus of Ota?

Nevus of Ota is a cosmetic concern and can sometimes be treated. Without treatment, the lesion may remain unchanged throughout one's life. The lesions may darken over time or due to sun exposure. So, being sun protective should be one of the basic measures.

Medical or surgical intervention is needed for the following reasons,

  • To rule of malignancies
  • To reduce symptoms of Nevus of Ota
  • To prevent bleeding, ulceration, infection, or enlargement of the patches
  • To correct disfigurement
  • To prevent progression to malignancies
  • To rule out other reactive processes

Although the nevus of Ota is benign, patients are advised to see their dermatologist and ophthalmologists check for signs of developing glaucoma or melanoma. 

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