Infected Giant Congenital Nodular Melanocytic Naevus in a 5-week-old Infant

Abstract

Giant congenital melanocytic naevus (GCMN) is present at birth and will measure ≥ 20 cm in diameter in
adulthood. It is rare, with an incidence of fewer than 1 in 20,000 births. Giant congenital melanocytic naevus
has an increased risk of extracutaneous abnormalities and transformation into malignant melanoma, but
sepsis may increase the risk for mortality in some children. Prompt identification, investigation, aggressive treatment of identified infections, and follow-up care for affected children, are essential.

A 5-week-old male infant presented with a widespread birthmark, progressively increasing swellings since
birth, high-grade fever and excessive crying of 3 days' duration. He was febrile and irritable. He had a bath suit
distribution of hyperpigmented lesions and multiple nodules with areas of ulceration and necrosis. He had
numerous satellite hyperpigmented patches. The complete Blood Count (CBC) showed leucocytosis with
granulocytosis. He had antibiotics. The histopathology of the tissue biopsy was consistent with a congenital
melanocytic nevus.

Ulcerated Giant congenital melanocytic naevus has a high risk of being complicated by sepsis and malignant
transformation and has a substantial psychological impact on the family of the affected child. Management of
this condition poses a considerable challenge, especially in developing countries where out-of-pocket health
financing often precludes access to quality healthcare for the low socioeconomic class without health
insurance.