Giant congenital facial melanocytic nevus.

Giant Congenital Melanocytic Nevus (GCMNs) is mostly reported in area of trunk followed by limbs and head. Their incidence is <1:20,000 newborns It derives attention due to its association with malignant melanoma.The risk of developing malignant melanoma is between 5 to 10%.We report a case of twelve year old boy with hyperpigmented lesion on face.

Introduction

GCMNs is a visible melanocytic proliferation in the skin. They are usually smaller at birth and grow up to ≥20 cm in size by adulthood.[1] It occurs very rarely in the periorbital region and presents as brownish lesion with well defined borders and hypertrichiosis.

Case Report

A 12-year-old boy presented to us with complaints of hyperpigmented lesion on the left side of the faceand eyelids since birth. The mass was gradually increasing in size and pigmentation since then. On examination, there was a raised pigmented lesion measuring 15 cm × 20 cm and extending from the left sub-brow region to the angle of mouth, philtrum, and preauricular region. The edges of lesion were well defined and its surface was leathery to touch and hairy. The pigmentation was uniform throughout the lesion. On ocular examination, ocular surface and anterior and posterior segment of both the eyes were within normal limits [Figure 1]. It was not associated with any other similar hyperpigmented lesion on the body elsewhere and there was no family history. A clinical diagnosis of giant congenital melanocytic nevus (GCMN) was made. The patient underwent excision with skin grafting of the eyelid lesion and was referred to plastic surgery department for further management.

Figure 1

Giant hairy congenital melanocytic nevus involving the nose and the left upper and lower lids. Giant hairy congenital melanocytic lesion involving the nose, left side of cheek, and left upper and lower lids

Discussion

GCMN is mostly reported in area of trunk followed by limbs and head with the incidence of <1:20,000 newborns.[1] To the best of our knowledge, there are only three cases of periorbital GCMN involving both the upper and lower eyelid reported in literature.[123] Besides presenting as brownish lesion with hypertrichiosis the surface of lesion may be popular, warty, roughed or cerebriform. It is known to be associated with systemic disease like malignant melanoma and ocular findings such as iris melanosis, anterior staphyloma, ectopic lacrimal gland, and ocular choristoma.[34] The risk of developing melanoma is between 5% and 10%.[1] Besides this, facial GCMN is an important cause of psychosocial trauma due to its unsightly appearance.

Conclusion

All the children presenting with this entity should be referred to ophthalmologists in view of its association with malignant melanoma.

Declaration of patient consent

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Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.