Melanocytic nevus (1,2,3,4,5,6,7,8) consists of melanocytes derived from the neural crest that migrate to the skin during embryonic development (1,2). An eyelid nevus can be acquired or congenital. The acquired type begins in childhood in the basal epithelium (junctional nevus), gradually migrates into the dermis in young adults (compound nevus), and later in life resides entirely in the dermis (dermal nevus). The average young adult has about 15 cutaneous nevi and the eyelid is occasionally affected (2). Multiple nevi should raise suspicion of the dysplastic nevus syndrome, in which there is a familial tendency toward development of cutaneous melanoma (2).

The clinical features vary with patient age and stage of the disease. It usually appears between ages 5 and 15 years as a small macule that gradually evolves through the stages listed above. Eyelid nevus can range from deeply pigmented to amelanotic. An eyelid margin nevus can extend onto the palpebral conjunctiva. It can surround the lacrimal punctum (peripunctal nevus; 3). The surface can be smooth or verrucous (3,4). It does not generally produce loss of cilia. A variant of congenital nevus is deeply pigmented and larger than an acquired nevus (2). It is slightly elevated and frequently has excess hairs. As opposed to the junctional nevus, this lesion has a greater chance of malignant transformation, perhaps as high as 5% (2,8). A variant of congenital nevus is the divided nevus of the upper and lower eyelids (“kissing nevus”) that develops before embryologic separation of the eyelid and divides when the eyelids separate.

Melanocytic nevi are divided into junctional, compound, and intradermal types (1,2). These are not entirely separate categories, but rather represent stages in the “life cycle” of nevus, as discussed above.

The diagnosis of nevus is often made by the arrangement of its cells in nests rather than by cellular characteristics (2). A junctional nevus has well-circumscribed nests of cells at the basal level of the epidermis. A compound nevus possesses features of both a junctional and an intradermal nevus. It can have considerable fibrous tissue and may resemble a neurofibroma or other neural tumors.

There are other variants of melanocytic nevus such as balloon cell nevus and epithelioid cell nevus that rarely involve the eyelid (1,2). Epithelioid cell nevus (Spitz nevus), a lesion that closely resembles melanoma, has been seen on the eyelid of young children.

Cutaneous nevi on the trunk and extremities are frequently excised because of their malignant potential. There is a greater tendency to observe those on the eyelid until growth is documented, partly because of cosmetic considerations. Management is generally periodic observation, with excision of more suspicious lesions. If the lesion is superficial, an elliptical resection is recommended. If it is present on the eyelid margin only, it can be shaved parallel to the eyelid margin. More extensive tumors require anterior lamellar or full-thickness eyelid resection (7).

Some congenital nevi can occasionally involve most of the eyelid area and their management is complex. The clinician must weigh the chances of malignant transformation with the consequences of radical removal and cosmetic surgery.

Many acquired nevi become clinically apparent in childhood and remain relatively dormant for the remainder of the patient’s life. Examples are shown of flat and minimally elevated lesions on eyelid margin.

Although it is classically found in Caucasians in the first decade of life, eyelid nevus can be initially diagnosed at any age and in patients of any race.

An eyelid nevus can be nonpigmented, thus resembling a papilloma, basal cell carcinoma, or other amelanotic lesion.

In some instances, typical eyelid margin nevus can be associated with loss of cilia. It can sometimes be first recognized in middle-aged or older patients.

Suspicious or growing lesions near the eyelid margin can be removed by an elliptical or shaving technique. These methods are illustrated in Chapter 15.

Divided nevus of the eyelid can assume any of several configurations.

Margo CE. Pigmented lesions of the eyelid. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. Philadelphia: W B Saunders; 1994:1797-1812.

In some instances, congenital periocular nevus can be very extensive, raising difficult management problems.

Margo CE. Pigmented lesions of the eyelid. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. Philadelphia: W B Saunders; 1994:1797-1812.

Oculodermal melanocytosis is a congenital pigmentation of periocular skin, uveal tract, and sometimes the orbit, ipsilateral meninges, and ipsilateral hard palate (1,2,3,4,5,6,7,8,9,10,11,12,13). Its incidence has been estimated to be about 0.04% in Caucasians (4). The excess melanocytes can spawn malignant melanoma of the uvea, orbit, and brain (1). Malignant transformation of the eyelid component into cutaneous melanoma is rare (2,3,4). Although oculodermal melanocytosis is generally diagnosed in Caucasians, it occurs in African Americans and Asians, in whom it is also associated with a higher incidence of uveal melanoma (9). This section on eyelid lesions considers mainly the periocular cutaneous lesion as part of the spectrum of oculodermal melanocytosis. The associated uveal melanoma is discussed in the atlas of intraocular tumors.

Clinically, the cutaneous lesion is a flat, tan to gray pigmentation that affects the facial and periocular skin, including the eyelids. Although it may be somewhat irregular, it tends to follow the distribution of the first and second divisions of the trigeminal nerves. It is bilateral in about 10% of cases. Interesting variations include involvement of the temporal skin and hard palate. Another association is iris mammillations, which are irregular, confluent, dome-shaped elevations on the iris surface. This can be the predominant feature when the episcleral and posterior uveal pigmentation is subtle or absent (11). Oculodermal melanocytosis can rarely occur in association with other phakomatoses, such as Sturge-Weber syndrome and phakomatosis pigmentovascularis IIa (12).

Histopathologically, nevus of Ota is characterized by excess scattered dendritic melanocytes in the dermis. It is not as cellular as a true blue nevus.

A patient with these clinical findings should be evaluated for evidence of ocular melanocytosis and undergo periodic fundus examination to detect early malignant melanoma of the uveal tract. Uveal melanoma usually occurs in adult Caucasians, but has been recognized in children (6) and African American patients (7) with congenital melanocytosis, underscoring the need to follow affected patients who would otherwise be at low risk to develop uveal melanoma. The incidence of uveal melanoma in patients with ocular or oculodermal melanocytosis is estimated to be 1:400 cases (5).