Bilateral naevus of OTA

Asok Kumar Gangopadhyay

 Department of Dermatology, RKM Seva Pratisthan, Calcutta-700026, India

Correspondence Address:
Asok Kumar Gangopadhyay
Department of Dermatology, RKM Seva Pratisthan, Calcutta-700026
India

Introduction

Naevus of Ota is usually an unilateral macular or slightly raised discoloration of face in the areas of distribution of ophthalmic, maxillary and rarely along the mandibular division of trigeminal nerve, along with ipsilateral discolouration of various structures of eye. This condition is rare in India,[1],[2] very rare in bilateral distribution and very-very rare in male patients. Here is the report of such an unusual case of bilateral naevus of Ota in a male patients.

Case Report

A yound Hindu male aged 35, policemen by profession, presented with the complaint of pigmentation over both the cheeks and sides of the forehead since childhood. He had pigmentation over the lateral sides of both sclera alongwith. The pigmentation was first noticed by his parents when he was a child of around one year. As far as he could gather from his parents, the pigmentation started as a few scattered bluish macules over both the cheek, which spread very very incidiously towards the forehead. The pigmentation over the sclera appeared few months after the cutaneous pigmentation. Apart from the cosmetic problem the patients had no symptoms as such. As far as the patient could recollect there was not much variation in the intensity of the colour which remained bluish grey all these years. Examination revealed scattered nonhairy bluish grey macules over both the cheeks down below the angle of mouth and above upto the both sides of the forehead. The margin was irregular blending vaguely with adjacent normal skin. There was mottled, bluish pigmentation over the lateral aspect of the sclera of both eyes. The palpebral conjunctiva, iris and cornea were free from any lesion. Ophthalmologic examination was normal. There was no lesion over the oral or nasal mucosa. There was no significant family history, general examination did not reveal any abnormality.

The epidermis was unremarkable. Numerous dendritic melanocytes were scattered along the collagen bundles through out the dermis.

Discussion

Although reported earlier,[3] the syndrome owes its name to Ota who described an unusual syndrome of ′Nevus-fusco-caeruleus-ophthalmo-maxillaris′ in 1939.[4]Later on, the same condition was recorded under different titles e.g. congenital melanosis bulbi, melanosis bulborum, aberrant monoglian spot, progressive melanosis oculi, oculomucodermal melanosis etc. All these designations try to convey something of the colour and the location of the process. The term fusco-caeruleus connotes a ′dark-dusky-slate-tan-purple-sky blue colour.′ Location is suggested to be in and around eye and adjacent areas of face. Bilateral involvement is rare, only about 5%. Ota′s naevus is not a hereditary dissease, although it has been reported in close relatives. Naevus of Ota is prevalent among the Japanese, not uncommon in Orientals, Chinese. Quiroga et at[6] state Ota′s naevus is common in dark races, However Ota′s naevus is infrequent in Indians. About 80% of the patients with Ota′s naevus are female,[7] although it is not certain whether it is the actual sex predilection or it is the cosmetic concern of the women which brings more of that sex to attention. The lesion may be present at bith (60%) the rest appearing in the first decade of life, very very rarely at or after puberty and after pregnancy. Apart from the skin and sclera, the other areas rarely involved are the mucous membrane of the mouth, nose, tympanic membrane, external auditory canal. Leptomeninges have been found rarely to bear the appearance. Besides the scleral involvement; melanocytic involvement has been reported rarely in the conjunctiva, cornea, iris, fundus oculi, optic papilla, retrobulbar fat, periosteum of orbital bone and the optic nerve, although vision is not improved with time. Naevus of Ito, naevus flammeus and persistant mongolian spot may co-exist. Melanoma has been reported very very rarely from Ota′s naevus.[8]

Regarding pathogensis it is believed by most investigators that the melanocytes are of neuroectodermal origin, but whether they are directly from the neural crest or of Schwann cell origin is debatable.

So keeping all these facts in mind, this particular case reported herein is very interesting in the sense that it is from India - where it is less commonly found, it is bilateral which is not the usual type, and it is in a male patient when the females dominate in this domain (so far as the frequency of involvement is concerened).

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