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 Table of Contents  
Year : 2016  |  Volume : 5  |  Issue : 1  |  Page : 39-42

Unilateral nevus of ota: A case report of oculodermal melanocytosis!

1 Department of Oral Medicine & Radiology, Jaipur Dental College, Jaipur, Rajasthan, India
2 Department of Prosthodontics, Jaipur Dental College, Jaipur, Rajasthan, India

Date of Web Publication1-Jul-2016

Correspondence Address:
Vela Desai
B-406, Trimurty Appartments, Opposite BSNL Telecom Colony, Model Town, Malviya Nagar, Jaipur - 302 017, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2277-4696.185192

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Hulke first described nevus of Ota in 1860, but a detailed description was made first by Ota in 1939 wherein the typical pattern of the bluish black pigmentation along with the cutaneous distribution of the trigeminal nerve were described. This condition is most prevalent in Japanese population but comparatively rare among Indians. Nevus of Ota is a nonhereditary pigmentation disorder, more commonly seen in females. The authors present, yet another case of unilateral nevus of Ota in a young Indian girl.

Keywords: Congenital, nevus of Ota, ocular pigmentation, pigmentation, treatment

How to cite this article:
Maheshwari R, Desai V, Sunil Kumar M V, Gaurav I. Unilateral nevus of ota: A case report of oculodermal melanocytosis!. J Dent Allied Sci 2016;5:39-42

How to cite this URL:
Maheshwari R, Desai V, Sunil Kumar M V, Gaurav I. Unilateral nevus of ota: A case report of oculodermal melanocytosis!. J Dent Allied Sci [serial online] 2016 [cited 2023 Jan 30];5:39-42. Available from: https://www.jdas.in/text.asp?2016/5/1/39/185192

  Introduction Top

Nevus of Ota is benign melanocytic pigmentary disorder also known as “congenital melanosis bulbi,” “oculodermal melanocytosis,” and “oculomucodermal melanocytosis.” Nevus of Ota is characterized by abnormally blue hyperpigmentation of the facial skin and oral cavity that often follows the distribution of first and second divisions (ophthalmic and maxillary) of trigeminal nerve.[1],[2]

In 1916, Pusey described case of a Chinese student with pigmentation of both sclera and facial region, but as a definitive entity it was first reported by Dr. Ota of Japan in 1939. The onset of nevi typically is either at or after birth (60%), or soon after birth (between 1 and 10 years of age), at puberty, but few rare cases of acquired type have also been reported. The involvement of oral mucous membrane is extremely rare.[3],[4],[5] This condition is frequent in Japanese descent but is also seen in Chinese, Indian, African, and European descent. However, the exact prevalence rate in Indian population still remains unknown. It has a strong predilection for females with ratio of 5:1.1. It is usually unilateral, bilateral involvement is described in 5-10% of cases.[3],[4] In this paper, we are reporting a case of nevus of Ota in an Indian female patient with involvement of the face and sclera.

  Case Report Top

A 23-year-old female patient visited to the Department of Oral Medicine and Radiology with sensitivity in the lower left back tooth since the last 6 months. The patient's medical and family history was noncontributory. The patient had habits of chewing raw betel nut occasionally. Extraoral examination revealed the presence of an asymptomatic blue-grey lesion on the face [Figure 1] that had been present since birth. The discoloration gradually progressed to involve the forehead, temple, malar [Figure 2], periorbital area, sclera, lower palpebral, and ala of the nose [Figure 3] and [Figure 4] with interspersed pinpoint lentigo-like spots. No other pigmentations or abnormalities were noticed during the general physical examination. On eliciting specific history regarding sclera and facial discoloration, the patient revealed that it was present since birth, and family history was noncontributory. Her parents were nonconsanguineous. The patient denied taking any medication, and she gave no history of photosensitivity. The patient was asymptomatic with no effect on vision, sensory changes, and hearing. This was her first dental visit and she had not consulted any dermatologist previously for this. On intraoral examination, extrinsic stains on tooth were present. There was no evidence of pigmentation in the oral cavity. Oral hygiene was comparatively good with only decay in one tooth.
Figure 1: Cutaneous pigmentation on the left side of the face

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Figure 2: Pigmentation on the left forehead and malar region of the face

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Figure 3: Pigmentation of lower and lateral part of the sclera

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Figure 4: Pigmentation of left ala of nose

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Cutaneous examination revealed a unilateral, diffuse, homogenous, slate colored, macular pigmentation over the left upper and middle one-third of the face. The border of the lesion was irregular.

Opthalmological examination revealed similar pigmentation in the sclera. The patient was referred to dermatologist and ophthalmologist for consultation. Ophthalmic investigation revealed less visual acuity in the left eye then compared to the right. However, there was no evidence of cataract or glaucoma. Dermatologist confirmed the diagnosis of Ota's nevus. Based on the clinical findings and dermatologist consultation, a diagnosis of nevus of Ota was made.

The other possible differential diagnoses were  Sturge- Weber syndrome More Details More Details, café-au-lait spots of neurofibromatosis, blue nevus, melasma, and actinic lentigo. Sturge-Weber syndrome presents with unilateral port wine stains and intraocular changes appearing similar to nevus of Ota. Lesions have typical reddish-purple color and regress as age progresses in contrast to nevus of Ota, which has a blackish color and advances as age progresses. Majority of the cases of Sturge-Weber syndrome have a history of neurological disorder like epilepsy. A computed tomography scan of the head was performed to rule out cerebral angiomatosis which is also a characteristic feature of Sturge-Weber syndrome. Neurofibromatosis was ruled out because of the lack of cutaneous nodules and the freckling in the axillary region. Blue nevus could be differentiated because of its typically bluish color, papular, found on the skin, in which the dermal melanocytes being deeper and denser than in the nevus of Ota. Melasma can be ruled out as it occurs during pregnancy and is related to hormonal changes. Actinic lentigo presents with brownish papules or plaques. No ocular pigmentation is seen in any of the above-discussed entities.[1] A detailed history along with radiographic, laboratory, and histopathological investigations aid in the differential diagnosis and their by confirming the diagnosis. Habit counseling and diet counseling were done. As there were extrinsic stains a thorough oral prophylaxis with oral hygiene instructions was followed by the restoration of the decayed teeth. The need for regular follow-up has been impressed upon the patient owing to the lesions' malignant transformation potential. However, the patient has been on a regular follow-up. The patient was motivated for the correction of the Nevus with laser therapy, but she was not convinced.

  Discussion Top

Nevus of Ota appears as a bluish hyperpigmentation along the ophthalmic and maxillary divisions of the trigeminal nerve. It can be congenital or acquired.[1],[2] It has been hypothesized that nevus is caused by melanocytes that have not migrated completely from the neural crest to the epidermis during the embryonic phase leading to dermal nesting and melanin production. The melanocytes enter the ophthalmic and maxillary branches of the trigeminal nerve creating characteristic blue spots.

The pathogenesis of nevus of Ota is multifactorial. The predisposing factors are genetics, female sex hormones, infection, trauma, and ultraviolet light exposure.[6],[7] The clinical appearance of nevus of Ota mostly has been described as unilateral (as in our case) or some time bilateral, macular, rarely papular or nodular, patchy brown, slate-blue or blue/grey-black pigmentation, with deeper lesions appearing blue due to Tyndall effect, in which, all but the blue end of the light spectrum penetrates into the deep dermis and is absorbed by dermal melanin. Intermingling of lentigo-like spots may also be noted.[8],[9] Pigmentation may affect sclera, conjunctiva, cornea, iris, choroid and less commonly, the optic nerve, retrobulbar fat, orbit, periostium, and extraocular muscles. The dermal lesions are bluish, confluent, nonhairy, flat, and pigmented macules with poorly defined margins. Melanocytosis may also affect the oral cavity, nasal mucosa, external auditory canal, tympanic membrane, orbital fissures, meninges, and the brain.[1] Ear involvement is rare, and the pigmentation of oral cavity is extremely rare. Although the cases have been reported with palate and buccal mucosa involvement.[6],[7],[8] Most of the cases have occurred in females (64%). The appearance of palatal pigmentation usually blends with oral mucosa and is typically irregular, ill-defined, and often exhibit mottled patch. In nevus of Ota palatal pigmentation can be differentiated from blue nevus as the latter is slightly raised or papular that is usually less than 1cm in size. Biopsy is generally not indicated.

The sclera is commonly involved in patients with nevus of Ota. The ocular complications associated with nevus of Ota are increased intraocular pressure and glaucoma.

Based on the extent of the involvement, Tanino classified nevus of Ota into four types:[5]

Type I: IA - Mild orbital type: Distribution over the upper and lower eyelids, periauricular, and temple regions

Type I: IB - Mild zygomatic type: Pigmentation in the infrapalpebral fold, nasolabial fold, and the zygomatic region

Type I: IC - Mild forehead type: Involvement of the forehead alone

Type I: ID - Involvement of ala nasi alone

Type II: Moderate type: Distribution over the upper and lower eyelids, periocular, zygomatic, cheek, and temple regions

Type III: The lesion involves the scalp, forehead, eyebrow, and nose

Type IV: Bilateral type: Both sides are involved.

According to this classification, the case corresponds to Type III nevus of Ota.

Histopathology of affected skin shows the presence of benign dendritic melanocytosis containing melanin in the dermis. Other observation is that the melanosomes in cells cultured from Ota's nevus are in part abnormal, almost mimicking those found in dysplastic nevi and melanoma.[9],[10]

Although diagnosis of nevus of Ota can be made based on its striking clinical characteristics, other entities that need to be particularly considered are nevus of Ito, Mongolian spots, blue nevus, and acquired bilateral nevus of Ota-like macules (ABNOM), owing to their clinical resemblance.

Nevus of Ito has features similar to that of Ota's nevus, except that it occurs along the distribution of the posterior supraclavicular and lateral cutaneous branchial nerves, involving the shoulder, neck, supraclavicular area, and upper arm. The Mongolian spot is congenital and involves the lumbosacral area and frequently regresses at about the age of 5-7 years. The diagnosis of above entities is essentially clinically owing to their similar histopathological features, thus eliminating the need for diagnostic biopsy. ABNOM is clinically distinguished by speckled or coalescent brownish-blue or slate-gray pigmentation over the face with no oral mucosa and eye involvement and histologically by diffuse upper dermal melanocytes.[8] Our patient, however, had no pigmentation of nail, perioral pigmentation, lentigines, and deafness, thus, exclusion of above pathologies was made.

There is no definitive diagnosis for nevus of Ota. Diagnosis is mainly by clinical examination and history. Skin biopsies are required only if clinical changes are suspected of malignant transformation (e.g., ulceration, new papular lesions, and variegations in color) within the involved skin, ocular tissues, or mucosal tissues.[8],[9],[10]

Various therapies are suggested in the literature which provides a successful outcome. Cosmetic cover-up products can be used for camouflage only. Combined derma abrasion and carbon dioxid lasers provide good result. Therapies with photothermolysis combined with Q-switched lasers are safe and effective for lightening nevi of Ota. The Q-switched lasers can selectively destroy the melanosomes and melanocytes that are responsible for pigmentation. Mild side effects such as purpura, erythema, and edema are also known to occur, that usually resolve after a few days. However, changes such as hyperpigmentation or hypopigmentation occur more frequently. The response to laser treatment has been reported to be dependent on lesion color. Darker lesions which have a greater number of melanocytes require more treatments for eradication. Overall, laser therapy is very effective in the treatment and recurrence is rare. Others such as alexandrite and neodymium-doped: Yttrium aluminum garnet laser have also been used. Topical tretinon, hydroquinone, and corticosteroid creams can be used in the treatment of postinflammatory hyperpigmentation. Other options are epidermal peeling, dermabrasion with autologous cultured epithelium grafting, and the combination of sequential dry ice.[11],[12],[13]

  Conclusion Top

The psychological impact of the lesions is high, and early treatment would considerably reduce the stress later in life. Without treatment, the skin lesions are permanent. Dentists should be ever alert to diagnose this condition and refer the patient to a dermatologist, otolaryngologist, and ophthalmologist for consultation. The lesions are usually asymptomatic, but rare instances of malignant melanoma arising from the lesions have been reported, usually along with the ophthalmic division of the trigeminal nerve. Hence, they should be screened biannually for any signs of malignant transformation such as a sudden increase in the size of discoloration, ulceration, and paresthesia. Furthermore, literature reveals that nevus (unilateral or bilateral) can be associated with Turner syndrome, Sturge-Weber syndrome, Klippel-Trenaunay syndrome, neurofibromatosis, multiple hemangiomas, spinocerebellar degeneration, ipsilateral deafness, nevus of Ito, and congenital cataract, so a thorough knowledge of this entity is essential.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/ their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Fitzpatrick TB, Kitamura H, Kukita A, Zeller R. Ocular and dermal melanocytosis. AMA Arch Ophthalmol 1956;56:830-2.  Back to cited text no. 1
Patel BC, Egan CA, Lucius RW, Gerwels JW, Mamalis N, Anderson RL. Cutaneous malignant melanoma and oculodermal melanocytosis (Nevus of Ota): Report of a case and review of the literature. J Am Acad Dermatol 1998;38(5 Pt 2):862-5.  Back to cited text no. 2
Ota M. Nevus fuscoceruleus ophthalmomaxillaris. Tokyo Med J 1939; 63:1243-5.  Back to cited text no. 3
Wilcox JC. Melanamatosis of skin and central nervous system. Am J Dis Child 1939;57:391.  Back to cited text no. 4
Tanino H. Nevus fuscoceruleus ophthalmomaxillaris Ota. Jpn J Dermatol 1939;46:435-51.  Back to cited text no. 5
Shetty SR, Subhas BG, Rao KA, Castellino R. Nevus of ota with buccal mucosal pigmentation: A rare case. Dent Res J (Isfahan) 2011;8:52-5.  Back to cited text no. 6
Guledgud MV, Patil K, Srivathsa SH, Malleshi SN. Report of rare palatal expression of Nevus of Ota with amendment of Tanino's classification. Indian J Dent Res 2011;22:850-2.  Back to cited text no. 7
[PUBMED]  Medknow Journal  
Sharma G, Nagpal A. Nevus of ota with rare palatal involvement: A case report with emphasis on differential diagnosis. Case Rep Dent 2011;2011:670679.  Back to cited text no. 8
Cronemberger S, Calixto N, Leite HF, Oftalmol RB. Nevus of Ota: Clinical opthalmological findings. Rev Bras Oftalmol 2011;70:278-83.  Back to cited text no. 9
Mehta V, Balachandran C. Case report bilateral nevus of Ota. J Pak Assoc Dermatol 2007;17:59-61.  Back to cited text no. 10
Chan HH, Kano T. Nevus of Ota: Clinical aspect and management. Skinmed 2003;2:89-96.  Back to cited text no. 11
Sekar S, Kuruvila M, Pai HS. Nevus of Ota: a series of 15 cases. Indian J Dermatol Venereol Leprol 2008;74:125-7.  Back to cited text no. 12
[PUBMED]  Medknow Journal  
Alsaif F, Alshahwan H. Bilateral nevus of Ota associated with Turner syndrome. J Saudi Soc Dermatol Dermatol Surg 2010;15:33-6.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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