Introduction
Melanocytic nevi are benign proliferations of melanocytic cells. Congenital divided melanocytic nevus is a rare form of melanocytic nevus, which presents in the upper and lower eyelids unilaterally. As this nevus extends up to the lid margins, its edges touch or “kiss” when the eyelids are closed. Although commonly presenting at birth, there have been cases where it appeared in later life. This occurs due to a rare embryological phenomenon and thus only 30 cases have been reported worldwide.[1]
Case Report
A 66-year-old female presented to the skin OPD with history of having a painless, black colored, non-progressive swelling over right upper and lower eyelids since 40 years. The lesion caused difficulty in approximation of eyelids. On examination, there were brown-black colored uneven plaques with verrucous edges and hypertrichosis, covering approximately 80% of both right upper and lower eyelid margins on right side. [Figure 1] With the eyelid closed, both nevi acquired contiguity with their contact surfaces and resembled a single entity. [Figure 2] There was lower lid ectropion and slight ptosis on the right due to the nevus, but there were no other eye complaints. Conjunctiva, sclera, and cornea were spared from pigmentation and ophthalmic examination was normal.
Figure 1.
Kissing nevus of the right eyelid
Figure 2.
With the eyelid closed, both nevi acquire contiguity with their contact surfaces and resemble a single entity
Discussion
Nevus, a Latin word for “maternal impression” or “birth mark,” indicates a circumscribed, nonneoplastic skin or mucosal lesion usually present at or soon after birth. Most congenital nevi are products of genetic alteration of cells, which arises from mosaicism. Melanocytic nevus, a type of nonvascular epidermal nevus, is a benign proliferation of melanocytic cells, which are arranged in nests in epidermis, dermis, or other tissue.[2] Although congenital melanocytic nevus develops during intrauterine life, the incidence of late congenital nevi can be explained by the inadequate production of melanin initially, or by small size of the nevus early on, which delays its detection.[3] Congenital divided nevus of the eyelids is a rare form, usually involving contiguous portions of the unilateral upper and lower eyelid margins, rendering the name “kissing nevus”. Normally, during intrauterine 6th week, the lids appear as ectodermal protrusions and grow toward each other and fuse at 24th week, after which they gradually separate. Melanoblasts originate from the neural crest and migrate during the 12–14 weeks of gestation to colonize the epidermis. During nevus formation, the defect in melanocyte migration or Schwann cell of neuroectodermal origin produces a split nevus.[4] This theory explains that it might be a single congenital nevus which has been divided due to developmental separation of eyelids. During developmental separation of eyelids, the single formed nevus undergoes a division and appears as two different entities. The nevi are usually asymptomatic and spare other eye structures, such as conjunctiva, sclera, and cornea.[5] In course of time there is a risk of malignant change (2% to 30%) in the nevus giving rise to malignant melanoma. Treatment options include dermabrasion, cryotherapy, excision, and skin grafting.[6] Multiple stage mosaic punching excision of the congenital divided nevus using a 10,600-nm CO2 pulsed laser has been performed in 15 cases in one of the study with good results.[6] Excision and blepharoplasty approach can also be used to treat such cases. Reconstruction can be done by various methods including the entire reconstructive ladder with both one- and two-staged approaches.[7]
Conclusion
Congenital divided melanocytic nevus is a very rare clinical finding. We have presented a case of the so-called “kissing nevus” on right upper and lower eyelids, which appeared at the age of 20 years in a 66 year old female, without any functional deformity which was unusual.
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
Nil.
Conflicts of interest
There are no conflicts of interest.
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