Introduction: Newborns can present with a host of varying birth marks. Nevi can represent a benign condition or be a harbinger of a more concerning underlying condition or genetic syndrome. Hyperpigmentation in the newborn is very common and has a wide spectrum of presentation from large areas of dark discoloration to spotty segmental patches to solitary light brown macules. Some nevi may have ...

A 16-month-old female presented with an extensive epidermal nevus demonstrating epidermolytic hyperkeratosis on histologic evaluation. Individuals with this disorder are at increased risk of bearing children with epidermolytic ichthyosis. This occurs because the same mutations causing cutaneous somatic mosaicism may also affect the gonads. Genetic counseling is advised for individuals with exte...