Congenital giant pigmented nevus Giant pigmented nevus is an uncommon birthmark appearing sporadically in one in 20,000 live births. Its features are different from those of an ordinary acquired nevus. Lesions are often darkly pigmented, hairy, and slightly infiltrated, eventually becoming verrucous or nodular. Satellite lesions may be present. Histologic features of an ordinary compound nevus, an intradermal nevus, a neural nevus, or a blue nevus may be present.
Treatment consists of multiple operations to excise as much of the lesion as possible.
Lesions on the scalp and neck may be associated with neurocutaneous melanocytosis of the leptomeninges that can be complicated by epilepsy, mental retardation, or central nervous system melanoma. Posterior axial LCMN, especially in association with satellite nevi, is a risk factor for CNS melanosis. Magnetic resonance imaging should be considered in the evaluation of newborns with these findings. All the patients with CNS involvement had nevi in the posterior axial location. Satellite nevi were present in 31 of the 33 patients.