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MEDICAL UNIVERSITY Plovdiv, BULGARIA FACULTY OF DENTAL MEDICINE 1 Oral Pathology Department www.meduniversity-plovdiv.bg CONTACT S Faculty of Dental Medicine Oral Pathology Department Plovdiv 4000, Bulgaria 3 Hristo Botev Bldv. [email protected] ORAL MANIFESTATION OF OTA'S NEVUS. A CASE REPORT WITH EMPHASIS ON DIFFERENTIAL DIAGNOSIS 1 1 1 Tomov G , Mutafchieva M , Nikolov N Nevus of Ota represents benign melanocytic pigmentation disorder that affects predominantly the Asian population. It occurs in the area innervated by the first and second division of the trigeminal nerve. Oral manifestations are rarely noted with only 15 cases reported. This report documents a rare oral manifestation of nevus of Ota with emphasis on the differential diagnosis. CASE REPORT A 52-year-old Bulgarian male from Caucasian race is referred to the Oral Pathology Department. There was no significant medical history reported. Extraorally, the patient presented with asymptomatic hyperpigmented macular bluish-black lesion involving the right periorbital area. (Fig.1a) Bluish pigmentation of sclera was also observed. (Fig.1b) It was present since birth, and the family history was noncontributory. There was no relevant drug history. On intraoral examination, blackish discoloration approximately 2 cm in diameter with diffuse margins was evident on the right side of the hard palate, which was not extending through the midline. Other parts of the oral mucosa were normal. The pigmented lesion was diagnosed as Ota's nevus and was classified as Type I subclass IE according to modified Tanino's classification. Patient was referred to dermatologist for consultation and discoloration of the right tympanic membrane was also revealed. The patient was not willing for any kind of treatment regarding the pigmentation but he was strongly advised for regular follow up. DISCUSSION The general concern regarding the Ota's nevus manifestations in the oral cavity is the correct differential diagnosis with other possible pigmented lesions. Ota's nevus has 2 peak ages of onset: in early infancy and in early adolescence that suggest hormones as a factor in the development of this condition. Isolated cases of delayed-onset nevi of Ota that first appear in adults, including in older patients, have been reported. The color or perception of the color of nevus of Ota may fluctuate according to personal and environmental conditions. It is usually unilateral and is located in the areas innervated by the first and second branches of the trigeminal nerve. Involvement of palatal mucosa occurs infrequently in nevus of Ota (only 12 cases have been reported). Most of cases have occurred in females. Appearance of palatal pigmentation usually blends with oral mucosa and is typically irregular, ill-defined and often mottled patch. Clinical differential diagnosis for oral lesions of nevus of Ota includes different clinical conditions (Chart 1) Usually oral melanotic macules, oral melanoma, postinflammatory pigmentation and drug induced hyperpigmentation can be misdiagnosed as nevus of Ota. Oral melanotic macules can also exist on palate but can be distinguished from nevus of Ota as it is commonly smaller in size. Drug induced hyperpigmentation is usually acquired after ingestion of drugs like minocycline, amiodarone and gold. Oral mucosal melanoma is rare, accounting for less than 1% of all oral malignancies. The most common site is the palate, which accounts for about 40% of cases, but other oral mucosal sites may also be affected. Oral melanoma is generally encountered between the fourth and seventh decades of life, with a greater incidence in men than in women. There is no definitive diagnosis for nevus of Ota (the histological findings are similar to some pigmented nevi). Diagnosis is developed mainly by clinical examination and history. The risk of malignant melanoma in oral cavity cannot be ignored and all patients with nevus of Ota should be screened regularly for any signs of malignant transformation. CONCLUSION Nevus of Ota with palatal involvement is a rare entity. Dentists should have a thorough knowledge regarding the pigmented lesions as it can indicate varying medical conditions and can lead to future complications if not diagnosed early. Figure 1. Bluish black pigmentation of right periocular area (a) and discoloration of sclera (b) Figure 2. Bluish black pigmentation of right palatal area PIGMENTED LESIONS DIFFUSE AND BILATERAL FOCAL Early onset Physiologic pigmentation Peutz-Jeghers syndrome Blanching Hemangioma Varix Nonblanching Thrombus Hematoma Blue-grey Amalgam tattoo Other foreignbody tattoos Blue nevus Brown Melanotic macule Pigmented nevus Melano- acanthoma Melanoma Predominantly adult onset Red-blue-purple Addison’s disease Heavy metal pigmentation Kaposi’s sarcoma With systemic signs and symptoms Drug-induced pigmentation Postinflammatory pigmentation Smoker’s melanosis Non systemic signs and symptoms NEVUS OF OTA Focal Unilateral (areas innervated by the first and second branches of the trigemial nerve) Early infancy onset Color: vary, nonblanching No systemic signs and symptoms

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MEDICAL UNIVERSITYPlovdiv, BULGARIA

FACULTY OF DENTALMEDICINE

1Oral Pathology

Department

www.meduniversity-plovdiv.bg

CONTACTS

Faculty of Dental MedicineOral Pathology Department

Plovdiv 4000, Bulgaria3 Hristo Botev Bldv.

[email protected]

ORAL MANIFESTATION OF OTA'S NEVUS.A CASE REPORT WITH EMPHASIS

ON DIFFERENTIAL DIAGNOSIS1 1 1Tomov G , Mutafchieva M , Nikolov N

Nevus of Ota represents benign melanocytic pigmentation disorder that affects predominantly the Asian population. It occurs in the area innervated by the first and second division of the trigeminal nerve. Oral manifestations are rarely noted with only 15 cases reported. This report documents a rare oral manifestation of nevus of Ota with emphasis on the differential diagnosis.

CASE REPORT

A 52-year-old Bulgarian male from Caucasian race is referred to the Oral Pathology Department. There was no significant medical history reported. Extraorally, the patient presented with asymptomatic hyperpigmented macular bluish-black lesion involving the right periorbital area. (Fig.1a) Bluish pigmentation of sclera was also observed. (Fig.1b) It was present since birth, and the family history was noncontributory. There was no relevant drug history.

On intraoral examination, blackish discoloration approximately 2 cm in diameter with diffuse margins was

evident on the right side of the hard palate, which was not extending through the midline. Other parts of the oral mucosa were normal. The pigmented lesion was diagnosed as Ota's nevus and was classified as Type I subclass IE according to modified Tanino's classification.

Patient was referred to dermatologist for consultation and discoloration of the right tympanic membrane was also revealed. The patient was not willing for any kind of treatment regarding the pigmentation but he was strongly advised for regular follow up.

DISCUSSION

The general concern regarding the Ota's nevus manifestations in the oral cavity is the correct differential diagnosis with other possible pigmented lesions. Ota's nevus has 2 peak ages of onset: in early infancy and in early adolescence that suggest hormones as a factor in the development of this condition. Isolated cases of delayed-onset nevi of Ota that first appear in adults, including in older patients, have been

reported. The color or perception of the color of nevus of Ota may fluctuate according to personal and environmental conditions. It is usually unilateral and is located in the areas innervated by the first and second branches of the trigeminal nerve.

Involvement of palatal mucosa occurs infrequently in nevus of Ota (only 12 cases have been reported). Most of cases have occurred in females. Appearance of palatal pigmentation usually blends with oral mucosa and is typically irregular, ill-defined and often mottled patch. Clinical differential diagnosis for oral lesions of nevus of Ota includes different clinical conditions (Chart 1) Usually oral melanotic macules, oral melanoma, postinflammatory pigmentation and drug induced hyperpigmentation can be misdiagnosed as nevus of Ota. Oral melanotic macules can also exist on palate but can be distinguished from nevus of Ota as it is commonly smaller in size. Drug induced hyperpigmentation is usually acquired after ingestion of drugs like minocycline, amiodarone and gold. Oral mucosal

melanoma is rare, accounting for less than 1% of all oral malignancies. The most common site is the palate, which accounts for about 40% of cases, but other oral mucosal sites may also be affected. Oral melanoma is generally encountered between the fourth and seventh decades of life, with a greater incidence in men than in women.

There is no definitive diagnosis for nevus of Ota (the histological findings are similar to some pigmented nevi). Diagnosis is developed mainly by clinical examination and history. The risk of malignant melanoma in oral cavity cannot be ignored and all patients with nevus of Ota should be screened regularly for any signs of malignant transformation.

CONCLUSION

Nevus of Ota with palatal involvement is a rare entity. Dentists should have a thorough knowledge regarding the pigmented lesions as it can indicate varying medical conditions and can lead to future complications if not diagnosed early.

Figure 1. Bluish black pigmentation of right periocular area (a)and discoloration of sclera (b)

Figure 2. Bluish black pigmentation of right palatal area

PIGMENTED LESIONSDIFFUSE AND BILATERAL FOCAL

Early onset

Physiologic pigmentation

Peutz-Jeghers syndrome Blanching

Hemangioma Varix

Nonblanching

Thrombus Hematoma

Blue-grey

Amalgam tattoo

Other foreignbody tattoos

Blue nevus

Brown

Melanotic macule

Pigmented nevus

Melano-acanthoma

Melanoma

Predominantly adult onset Red-blue-purple

Addison’s disease Heavy metal

pigmentation Kaposi’s sarcoma

With systemic signs and symptoms

Drug-induced pigmentation

Postinflammatory pigmentation

Smoker’s melanosis

Non systemic signs and symptoms

NEVUS OF OTAFocal Unilateral (areas innervated by the first and second branches of the trigemial nerve) Early infancy onset Color: vary, nonblanching

No systemic signs and symptoms