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 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 1  |  Page : 83-84

Nevus of Ota with palatal involvement and Helicobacter pylori gastritis: A rare case report

1 Department of Ocular Pathology, Uveitis and Neuroophthalmology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
2 Department of Ophthalmology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
3 Apollo Hospital, Guwahati, Assam, India

Date of Submission17-Mar-2020
Date of Acceptance14-Jul-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Dr. Dipankar Das
Department of Ocular Pathology, Uveitis and Neuro-Ophthalmology Services, Sri Sankaradeva Nethralaya, 96 Basistha Road, Beltola, Guwahati - 781 028, Assam
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_585_20

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Congenital oculodermal melanocytosis is also known as Nevus of Ota, condition where blue nevus of skin and orbit are seen with ipsilateral nevus in conjunctiva and iris. They are often associated with glaucoma which can occur at any age. They can have melanocytosis and develop choroidal melanoma. We report a case of a middle-aged female with facial pigmentation and bilateral scleral, conjunctival, and iris nevus with an advanced glaucomatous change in both eyes and pigmentation of the palatal mucosa. Trabeculectomy with mitomycin-C application was done in the left eye. Gastrointestinal endoscopy by gastroenterologist showed gastritis and Helicobacter pylori infection.

Keywords: Endoscopy, face, glaucoma, pigmentation, urease

How to cite this article:
Das D, Sarma P, Chetri K, Bhattacharjee H, Deshmukh S, Kumari N, Shinde K. Nevus of Ota with palatal involvement and Helicobacter pylori gastritis: A rare case report. Indian J Ophthalmol Case Rep 2021;1:83-4

How to cite this URL:
Das D, Sarma P, Chetri K, Bhattacharjee H, Deshmukh S, Kumari N, Shinde K. Nevus of Ota with palatal involvement and Helicobacter pylori gastritis: A rare case report. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2022 Oct 6];1:83-4. Available from: https://www.ijoreports.in/text.asp?2021/1/1/83/305531

Nevus of Ota usually has unilateral facial pigmentation with unilateral conjunctival, scleral, and iris pigment change.[1] It is associated with a high risk of development of choroidal melanoma (1:400).[2] The patient can have trabecular meshwork (TM) pigmentation and sometimes, the pigments can clog the TM causing glaucoma.[1],[2] We present a case of a middle-aged female with facial pigmentations and bilateral conjunctival, scleral, and iris pigmentations. She had advanced glaucomatous cupping in the right eye (OD) and was blind. Diffuse pigmentation of the palatal mucosa was noted and the gastroenterologist was consulted. On endoscopy, pigmentations had deeper involvement and the urease test for Helicobacter pylori was positive. As Nevus of Ota has a premalignant association for choroidal melanoma, simultaneous pigmentation of the palatal mucosa and gastritis increased the risk for future malignancy in the gastrointestinal (GI) tract.

  Case Report Top

A 36-year-old female presented to a tertiary care ophthalmic Institute of North-East India with a chief complaint of diminution of vision in OD for 8 years, which was gradual and worsening. She also complained of eye pain in both eyes (OU) for 1 year and it was intermittent. There was a history of pigmentation of the face and white part of the eye since childhood. There was no significant past medical and allergy history. On examination, the vision was found to be perception of light negative in OD and 20/80 in the left eye (OS) improving to 20/32 with pinhole. There was facial pigmentation with pigmentations on the skin of both orbital areas and forehead [Figure 1]. Conjunctival and scleral pigmentation was seen in OU. On slit-lamp examination, OU showed diffuse iris pigmentary change and nuclear sclerosis grade 1 cataract in OD [Figure 2]a. The patient's hard palate also showed diffuse pigmentation [Figure 2]b. Intraocular pressure (IOP) measured by applanation tonometry was 38 mm Hg in OD and 30 mm Hg in OS. Gonioscopy done by Posner 4-mirror goniolens revealed closed angle in OU. On fundus examination, retina and choroid in OU were normal. Optic disc showed advanced glaucomatous cupping (OD > OS) [Figure 3]a and [Figure 3]b. Pigmentation of the optic disc was also observed in OS [[Figure 3]b, marked with an arrow]. The patient was reviewed in the glaucoma clinic and was started on three topical anti-glaucoma medications in OS but IOP could not be controlled even after three medications. Trabeculectomy with the mitomycin-C application was carried out under local anesthesia in the OS. An eye-patch and shield was applied after the surgery and releasable suture was adjusted in third postoperative day. Postoperatively, IOP got lowered. The patient was referred to a gastroenterologist with the diagnosis of Nevus of Ota and pigmentation of the palatal mucosa. The gastroenterologist did upper GI endoscopy which revealed the deeper palatal pigmentation and urease test was positive for H. pylori [Figure 4]. The patient was prescribed necessary oral antibiotics and Pantoprazole for the prophylaxis of H. pylori gastritis.
Figure 1: Facial pigmentation. Some of the part of scleral pigmentations was also seen

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Figure 2: (a) Diffuse iris blue nevus in the left eye. Similar pigmentations were also seen in the right eye. (b) Diffuse palatal pigmentation

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Figure 3: (a and b) Advance glaucomatous optic atrophy (right > left). Media in OD was hazy due to associated cataract. (b) Optic disc of OS with surface pigmentation (marked with an arrow)

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Figure 4: (a and b) Upper gastrointestinal endoscopy with pigmentary change. Urease test for Helicobacter pylori was positive

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  Discussion Top

Nevus of Ota can have diffuse bluish-gray pigmented discoloration of face including the periorbital skin and forehead, conjunctiva, sclera and iris which usually have unilateral distribution.[3] If they are diagnosed early, patients are followed up for the development of choroidal melanoma in that eye.[3],[4] Pigmentation elsewhere should be looked for such as the oral cavity including the hard palate.[5] Histopathologically, skin of these patients can have heavy pigmentation with bipolar melanocytic cells which are fusiform in distribution. They are concentrated in both the reticular and papillary dermis of the skin. In finer details, melanocytes are usually present parallel to the skin surface except at the site of adnexal structures where they are seen to be oriented vertically.[6]

Our patient had diffuse light pigmentations all over the face with pigment change on conjunctiva; sclera and iris bilaterally. IOP was raised in OU with angle closure. The patient was blind with total glaucomatous cup in OD. OS showed advanced cupping and the Humphrey visual field analysis 30-2 showed inferior arcuate defect. Patient was finally managed with glaucoma filtering surgery in OS. Gastroenterologist also found the extension of the palatal pigmentations into the upper GI tract with evidence of gastritis in GI endoscopy. In urease test, H. pylori infection was detected and the patient was started with systemic medications.

Helicobacter pylori can cause chronic gastritis which sometimes, can lead to cellular infiltrations of GI mucosa and can cause injury to the epithelial cells resulting in gastric metaplasia.[7] Helicobacter pylori infection, if unchecked, can cause adenocarcinoma of the stomach.[8],[9],[10] We know that Nevus of Ota is a premalignant condition and malignant transformation of the pigmented site is higher, for example, the development of choroidal melanoma in the eye.[3],[4] Pigmentation on the palate and subsequent gastritis in this group of patient can induce a cancer in GI tract in the future. This group of patients need lifelong follow-up by an ophthalmologist and dermatologist. In special situation, gastroenterologist need to examine them and follow-up if associated pigmentation in oral cavity is detected as in our case. H. pylori infection is one of the major public health problems in developing country.[9],[10] Overall improvement of proper hygiene in the patient is also advisable in this condition.

  Conclusion Top

It is imperative to know the systemic associations of Nevus of Ota and screen the patient for the same. It may be ideal to explore for co-morbidities that may increase the risk of developing malignant tumors, and initiate appropriate management for the same.

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

This study was supported by Kanchi Sankara Health and Educational Foundation, Guwahati, Assam, India.

Conflicts of interest

There are no conflicts of interest.

  References Top

Nam JH, Kim HS, Choi YJ, Jung HJ, Kim WS. Treatment and classification of nevus of Ota: A seven-year review of a single institution's experience. Ann Dermatol 2017;29:446-53.  Back to cited text no. 1
Pal BP, Garge S, Khetan V. Choroidal melanoma: A short review with an Indian perspective. Oman J Ophthalmol 2017;10:135-44.  Back to cited text no. 2
[PUBMED]  [Full text]  
Mohan RP, Verma S, Singh AK, Singh U. 'Nevi of Ota: The unusual birthmarks': A case review. BMJ Case Rep 2013;2013:bcr2013008648.  Back to cited text no. 3
Radhadevi CV, Charles KS, Lathika VK. Orbital malignant melanoma associated with nevus of Ota. Indian J Ophthalmol 2013;61:306-9.  Back to cited text no. 4
[PUBMED]  [Full text]  
Adil M, Amin SS, Raj D, Alam M. Bilateral nevus of ota with involvement of palate: A rare case report. Indian J Paediatr Dermatol 2018;19:290-2.  Back to cited text no. 5
  [Full text]  
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. 6
de Vries AC, Haringsma J, Kuipers EJ. The detection, surveillance and treatment of premalignant gastric lesions related to Helicobacter pylori infection. Helicobacter 2007;12:1-15.  Back to cited text no. 7
Sheu BS, Wu MS, Chiu CT, Lo JC, Wu DC, Liou JM, et al. Consensus on the clinical management, screening-to-treat, and surveillance of Helicobacter pylori infection to improve gastric cancer control on a nationwide scale. Helicobacter 2017;22:e12368.  Back to cited text no. 8
Ankouane F, Noah DN, Enyime FN, Ndjollé CM, Djapa RN, Nonga BN, et al. Helicobacter pylori and precancerous conditions of the stomach: The frequency of infection in a cross-sectional study of 79 consecutive patients with chronic antral gastritis in Yaoundé, Cameroon. Pan Afr Med J 2015;20:52.  Back to cited text no. 9
Burkitt MD, Duckworth CA, Williams JM, Pritchard DM. Helicobacter pylori-induced gastric pathology: Insights from in vivo and ex vivo models. Dis Model Mech 2017;10:89-104.  Back to cited text no. 10


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


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