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Melanocytic lesions are extremely common in pediatric patients. At least 1 melanocytic nevus develops by early childhood in more than 95% of fair-skinned individuals.1 Dermal melanocytosis and other pigmented lesions such as freckles, lentigines, café au lait macules, and Becker nevi are also frequently observed in children and adolescents. In addition, a variety of disorders characterized by increased or decreased cutaneous pigmentation can present in childhood, ranging from postinflammatory hyperpigmentation and hypopigmentation to vitiligo to patterned pigmentation reflecting cutaneous mosaicism. Genetic diseases with pigmentary manifestations (eg, oculocutaneous albinism, piebaldism, Waardenburg syndrome, tuberous sclerosis, neurofibromatosis) are reviewed in Chapter 360. It is important for pediatricians to be aware of the clinical spectrum and natural history of benign melanocytic lesions and self-limited disorders of pigmentation in children as well as of findings that should raise concern.

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Melanocytic nevi (moles) are benign proliferations of a type of melanocyte. The 2 major differences between ordinary melanocytes that reside in the basal layer of the epidermis and nevus cells are that (1) nevus cells cluster as nests within the lower epidermis and/or dermis, whereas epidermal melanocytes are evenly dispersed as single cells; and (2) nevus cells do not have dendritic processes (with the exception of those within blue nevi). Like ordinary melanocytes, nevus cells are capable of producing the pigment melanin. Melanocytic nevi can be acquired or congenital, banal or atypical (dysplastic). There are several variants, such as halo, blue, and Spitz nevi, that have specific clinical and histologic characteristics.2

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Acquired Melanocytic Nevi

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Acquired melanocytic nevi begin to appear after the first 6 months of life and increase in number during childhood and adolescence, typically reaching a peak count during the third decade and then slowly regressing with age.3-5 Both environmental and genetic factors play a role in the development of acquired melanocytic nevi. Sun exposure is the primary environmental influence (eTable 361.1); hereditary components include pigmentary phenotype as well as a particular predisposition to “moliness.”

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eTable 361.1. Environmental Triggers for the Development and Growth of Melanocytic Nevi in Children and Adolescents

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