Recent Advances in Dental Sciences

Nevus of Ota: A Rare Case with Review of Literature.

Renita Lorina Castelino1*, Subhas G Babu1, Fazil K A2, Anusha Rangare Laxmana3, and
Preethi Balan2.
1Department of Oral Medicine and Radiology, AB Shetty Memorial Institute of Dental Sciences, Nitte University, Mangalore- 575008,
Karnataka, India.
2Department of Oral Medicine and Radiology, Sri Anjaneya institute of dental sciences, Calicut, Kerala, India.
3Department of Oral Medicine and Radiology, Century International Institute of Dental Sciences and Research Centre, Poinachy,

Kasargod, Kerala, India.

ABSTRACT

Nevus of Ota is characterised by bluish black pigmentation along the cutaneous distribution of the trigeminal
nerve. It is described as hamartoma of dermal melanocytes, clinically presented as a blue or grey patch on the face and
eyes. It is more prevalent in the in Japanese population and affects around 0.5% of the population. The incidence is higher
in females compared to males. In this case report we report a case of nevus of Ota in a female patient with pigmentation
noticed on the skin of face and eyes.
Keywords: Ota, pigmentation, nevus, intra oral

*Corresponding Author

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Nevus of Ota is a congenital hyperpigmentation of the facial skin in the distribution of the first and second divisions
of the trigeminal nerve which is associated with increased pigmentation of the various ocular structures. The pigmentation
may affect the sclera, conjunctiva, cornea, iris, choroid and less commonly, the optic nerve, retro bulbar fat, orbit periosteum
and the extra ocular muscles [1]. Most of the cases of nevus of Ota are unilateral (90%) and 5 to 10% of cases occur
bilaterally. Nevus of Ota is most commonly found in Asian population and affects 0.5% of the population but highest in the
Japanese population [1]. The incidence is higher in females with male to female ratio being 1:4.8 [2]. The case presented
here highlights a rare case of nevus of Ota with clinical features and review of literature

Case report

A 30 year old female patient reported to the Department of Oral Medicine and Radiology with the complaint of
stains and deposits in all teeth since 6 months. There were no associated symptoms reported. The patient’s medical and
drug history was non-contributory. On extra oral examination, diffuse hyper pigmented areas were noted on the right
periorbital and the zygomatic area. The hyper pigmented areas extended superiorly upto the right eyebrow, inferiorly 3 cm
below the inferior border of right eye, medially upto the corner of the eye and laterally 1 cm beyond the right lateral border of
the eye. Bluish black pigmentation of the right sclera of eye was also noticed. No other pigmented areas were noted on the
general physical examination. On further questioning of the patient, the patient stated that the pigmentation was present
since birth and had gradually increased in size. The patient had not visited any doctor for this pigmentation before. Based on
the chief complaint a provisional diagnosis of chronic generalised marginal gingivitis was made. The patient was advised
oral prophylaxis. Based on the clinical features of the hyper pigmented areas a provisional diagnosis of Nevus of Ota was
made. The differential diagnoses considered were blue nevus, melasma, café-au-lait spots of neurofibromatosis were
considered. A thorough examination of the patient was done by a dermatologist, otolaryngologist and ophthalmologist. The
visual acuity and intraocular pressure were normal as said by the ophthalmologist .The CT scan of the patient was done to
rule out cerebral angiomatosis. The CT report was found to be normal. After the investigations performed on the patient all
the differential diagnoses considered were ruled out. The patient was then suggested laser treatment for the hyper
pigmented areas, but he patient refused the treatment due to financial constraints. The patient is kept on a regular recall for
check-up.

DISCUSSION

Nevus of Ota was first described by Hulke in 1860 but a detailed description was made by by Ota in 1939 wherein
the typical pattern of the bluish black pigmentation along with the cutaneous distribution of the trigeminal nerve was
described [1]. Nevus of Ota or ocular dermic melanocytosis is a clinical condition characterized by blue-dark pigmentation,
green-blue, violet, or tan spots in the eyes and/or on the face’s skin and eyelids [3]. Nevus of Ota is usually present at birth
but it can also occur in puberty or during pregnancy. It can also appear during the first year of life in 50-60% of cases, with a
second peak occurring during adolescence [3,4]. In our case the pigmentation was present at birth. This condition is most
prevalent in Japanese population but comparatively rare among Indians [5]. The exact aetiology of this condition is not
known but some researchers believe that the melanocytes move from the neural crest to the skin during the early embryonic
life. The failure of complete migration into the epidermis before birth with dermal nesting and melanin production produces
characteristic blue patches [6]. The recent studies have revealed that hormonal alteration and exposure to ultra-violet
radiation may have an important role to play in the pathogenesis of this condition [7]. Tanino in 1939 has proposed the most
useful clinical classification and in this classification nevus of Ota has been classified into four major subtypes [8]

Type-I

IA: Mild orbital type - Distribution over the upper and lower eyelids, periocular and temple region.
IB: Mild zygomatic type-Infrapalpebral fold, nasolabial fold and zygomatic regions are affected.
IC: Mild forehead type-Only forehead is affected.
ID: Ala nasi alone is affected.

Type-II

Moderate type-The lesions affect upper and lower eyelids, periocular, zygomatic, cheek and temple regions.

Type-III

The condition is distributed over the scalp, forehead, eyebrows and nose.

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Bilateral type.

Our case falls under Type IB

The colour of the lesions depends upon the depth of involvement of the tissues and the race. The deeper lesions
appear blue in color due to the Tyndall effect whereas the more superficial lesions are slate grey in colour [3]. Our case had
bluish black pigmentation on face, eyelids and sclera. The various treatment modalities available for Nevus of Ota involving
the skin includes dermabrasion, epidermal peeling and argon laser. It has also been successfully treated by Q-switched
ruby, alexandrite and Nd: YAG laser [9,10]. Laser treatment was suggested to our patient but the patient refused the
treatment due to financial constraints. Malignant alterations may occur in nevus of Ota with the appearance of melanoma
affecting the skin, orbit, iris, ciliary body, choroid and brain [11, 12]. The lesions are usually asymptomatic but have to be
kept on a long term follow up to check any abnormalities arising from the lesion. Our patient was kept on a long term follow
up.

Figure 1: Extra oral picture showing hyper pigmented areas on the right side of the face on upper and lower eyelids
and the zygomatic area

Figure 2: Pigmentation seen in the right sclera

CONCLUSION

The case reported here is a rare dermatological melanocytic hamartoma involving eyes and face area. The case
should be diagnosed and frequent monitoring is required. The treatment usually recommended is laser therapy and follow
up is required as the lesions can reoccur.

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[1] Shetty SR, Babu S, Rao K, Castelino R. Dental Res J 2011; 8(1): 52-55
[2] Hidano A, Kajima H, Ikeda S, Mizutani H, Miyasato H, et al. Arch Dermatol 1967;95:187–195.
[3] Cronemberger S, Calixto n and Freitas HL. Rev Bras Oftalmol 2011; 70 (5): 278-8.
[4] Wang HW, Liu YH, Zhang GK, Jin HZ, Zuo YG, Jiang GT, Wang JB. Dermatol Surg 2007;33(4):455-60.
[5] Mukhopadhyay AK. Indian J Dermatol Venereol Leprol 2004; 70(2): 112-3.
[6] Sekar S, Kuruvila M, Pai HS. Indian J Dermatol Venereol Leprol 2008; 74(2): 125-7.
[7] Wang BQ, Shen ZY, Fei Y, Li H, Liu JH, Xu H, et al. J Invest Dermatol 2011; 131(2): 358-62.
[8] Tanino H. Jpn J Dermatol 1939; 46: 107-11.
[9] Page DG, Svirsky JA, Kaugars GE. Oral Surg Oral Med Oral Pathol 1985; 59(3): 282-4
[10] Geronemus RG. Arch Dermatol 1992; 128(12): 1618-22.
[11] Sharan S, Grigg JR, Billson FA. Br J Ophthalmol. 2005;89(11):1529.
[12] Singh AD, Shields CL, Shields JA, Sato T. Arch Ophthalmol 2001;119(6):925-6.

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