Indian Journal of Dermatology
CASE REPORT
Year
: 2006  |  Volume : 51  |  Issue : 3  |  Page : 198--199

Familial nevus of ota


Rashmi Kumari, Devinder Mohan Thappa 
 Department of Dermatology and STD, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry - 605 006, India

Correspondence Address:
Rashmi Kumari
Department of Dermatology and STD, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry - 605 006
India

Abstract

A 23-year-old pregnant woman with an asymptomatic unilateral, bluish, pigmented lesion on her left periocular skin was referred from the obstetrics department for dermatological evaluation. Dermatologic examination revealed unilateral, blue-gray, poorly defined macule on the periorbital skin of the left side of the face mainly over the malar prominence and left temple not crossing the midline, conforming to the maxillary division of trigeminal nerve distribution. The pigmentation was speckled with interspersed dark-brownish elements. Her father, a 47 - year- old man had a similar pigmentation at the same site over the malar prominence of face not extending onto the forehead. Here we report two cases of nevus of Ota occurring in 2 generations of the same family for its rarity.



How to cite this article:
Kumari R, Thappa DM. Familial nevus of ota.Indian J Dermatol 2006;51:198-199


How to cite this URL:
Kumari R, Thappa DM. Familial nevus of ota. Indian J Dermatol [serial online] 2006 [cited 2022 May 21 ];51:198-199
Available from: https://www.e-ijd.org/text.asp?2006/51/3/198/27986


Full Text

 Introduction



Nevus of Ota or "naevus fuscocaeruleus ophthalmomaxillaris," is an uncommon disorder of periocular hyperpigmentation affecting the first and second divisions of trigeminal nerve and is associated with scleral melanosis. It is commonly seen in orientals and is considered a congenital rather than a hereditary disease.[1] Herewith we report two cases of nevus of Ota occurring in 2 generations of the same family for its rarity.

 Case Reports



Case 1

A 23-year-old pregnant woman with an asymptomatic unilateral, bluish, pigmented lesion on her left periocular skin was referred from the obstetrics department for dermatological evaluation. The lesion was present from birth and had been gradually increasing and extending onto the surrounding skin of the same side of the face and the forehead. She had never sought any treatment previously for this. Dermatologic examination revealed unilateral, blue-gray, poorly defined macule on the periorbital skin of the left side of the face mainly over the malar prominence and left temple not crossing the midline, conforming to the maxillary division of trigeminal nerve distribution. The pigmentation was speckled with interspersed dark-brownish elements [Figure 1]. Ocular examination disclosed a bluish discoloration of the sclera more on the superior quadrant than inferior and brownish discoloration of the conjunctiva in the left eye [Figure 2]. Visual acuity and intraocular pressure were normal and there was no evidence of ocular melanoma. Otoscopy showed no pigmentation of the tympanic membrane. Nasal, buccal, pharynx and palatine mucosa were normal. Central nervous system examination was found to be normal.

Case 2

Her father, a 47 - year - old man had a similar pigmentation at the same site over the malar prominence of face not extending onto the forehead. The pigmentation was less obvious due to his darker complexion than his daughter and was more homogenous [Figure 3]. Also, ipsilateral eye showed bluish black pigmentation over the sclera. No associated defects were detected on further investigations.

Both the patients were advised regular ophthalmological follow up annually and referred for laser therapy.

 Discussion



Nevus of Ota, which was probably first reported by Hulke in 1860 before Ota's description in 1939[2] is characterized by unilateral, irregularly patchy, bluish gray discolouration of the skin of the face supplied by the first and second divisions of the trigeminal nerve, particularly the periorbital region, the temple, the forehead, the malar area, and the nose. It is said to be most prevalent in Japan, where the incidence among dermatology outpatients is between 0.2 and 0.8%.[3] This condition is comparatively rare in Indians and uncommon in males with female to male ratio of 5:1.[4]

Exogenous factors may alter the intensity of pigmentation in a nevus of Ota. Color tone may be increased by fatigue, emotional excitement, insomnia, and unusually cold or warm weather. Hormonal fluctuations, including those associated with menstruation and menopause, can cause similar darkening. Spontaneous regression has not been observed.[3]

Based on the extent and distribution, Tanino classified nevus of Ota as mild, moderate, intensive, or bilateral.[5] According to this classification our cases would correspond to type II. Both cases were associated with scleral melanosis. Ocular pigmentation, (22 to 77% of cases) is almost always ipsilateral. The pigment is deep in the conjunctiva and does not move with it, in contrast to benign epithelial melanosis. Pigmentation may affect the sclera, conjunctiva, cornea, iris, choroid, and less commonly, the optic nerve, retrobulbar fat, orbit periosteum, and extraocular muscles.[6] The pigmentation of mucosal membranes of the head and neck is variable; the tympanic membrane is the most frequently affected although nasal, buccal, pharynx, and more rarely, palatine mucosa may also be involved.[2] No mucosal pigmentation was observed in both our cases. Histologic features in nevus of Ota include darkly pigmented, spindle-shaped dendritic melanocytes in the upper and mid-dermis.[3]

The main problem is usually cosmetic but different complications may appear. Glaucoma appears in approximately 10% of patients, and this is due to the proliferation of melanocytes in the anterior chamber angle.[6] The most serious but unusual complication is the development of malignant melanoma on pigmented areas.[7] Different disorders such as Sturge-Weber syndrome, Klippel-Trenaunay syndrome, neurofibromatosis, multiple hemangiomas, spinocerebellar degeneration, ipsilateral deafness and congenital cataract have been associated with nevus of Ota.[2] Selective photothermolysis with the Q-switched ruby laser is considered to be a safe and effective treatment for this pigmentation.[3]

This is probably the sixth report of familial nevus of Ota in the world and the first in Indian literature reported to date.[8] The presence of nevus of Ota in two consecutive generations raises the possibility of a genetic defect resulting in the defective migration of melanocytes from the neural crest to the epidermal-dermal junction. The increasing number of familial case reports of nevus of Ota lend added evidence for its hereditary basis.

References

1Rebat M, Halder RM, Nandedkar MA, Neal KW. Pigmentary disorders in ethnic skin. Dermatol Clin 2003;2:617-28.
2Alvarez-Cuesta CC, Raya-Aguado C, Vazquez-Lopez F, Garcia PB, Perez-Oliva N. Nevus of Ota associated with ipsilateral deafness. J Am Acad Dermatol 2002;47:S257-9.
3Alam M, Arndt KA, Dover JS. Laser treatment of nevus of Ota. Dermatol Ther 2001;14:55-9.
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5Tanino H. Naevus fusco-caeruleus ophthalmomaxillaris (Ota). Jpn J Dermatol 1939;46:107-11.
6Patel 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;38:862-5.
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