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Patient Story
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A 15-year-old boy is brought in by his mother with a concern about growth of his birthmark. It has become somewhat more raised and bumpy in the past year (Figure 145-1). The adolescent reports no symptoms and is not worried about the appearance. He is otherwise healthy with no neurologic symptoms. The joint decision of the family and the doctor was to not excise the epidermal nevus at this time. He may choose to have this removed by a plastic surgeon in the future.

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FIGURE 145-1

Epidermal nevus on the face of a teenager. This nevus has been present since birth, and the patient is otherwise healthy. (Used with permission from Richard P. Usatine, MD.)

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Introduction
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  • Epidermal nevi (EN) are congenital hamartomas of ectodermal origin classified on the basis of their main component: sebaceous, apocrine, eccrine, follicular, or keratinocytic.

  • Nevus sebaceous (NS) is a hamartoma of the epidermis, hair follicles, and sebaceous and apocrine glands. A hamartoma is the disordered overgrowth of benign tissue in its area of origin.

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Synonyms
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  • EN syndrome is also called Solomon syndrome and is a neurocutaneous disorder characterized by EN and an assortment of neurologic and visceral manifestations.

  • NS is also called sebaceous nevus and nevus sebaceous of Jadassohn (Figure 145-2).

  • An inflammatory linear verrucous epidermal nevus (ILVEN; Figure 145-3) can be part of an epidermal nevus syndrome but some affected persons only have the cutaneous EN.

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FIGURE 145-2

Nevus sebaceous on the scalp of a 14-year-old boy. (Used with permission from Richard P. Usatine, MD.)

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FIGURE 145-3

Inflammatory linear verrucous epidermal nevus (ILVEN) on the trunk. Topical steroids were not helpful in diminishing his pruritus. (Used with permission from Robert T. Gilson, MD.)

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Epidemiology
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  • EN are uncommon (approximately 1 to 3 percent of newborns and children), sporadic, and usually present at birth, although they can appear in early childhood.

  • EN are associated with disorders of the eye, nervous, and musculoskeletal systems in 10 to 30 percent of patients; in one study, 7.9 percent of patients with EN had one of the nine syndromes—an estimated one per 11,928 pediatric patients.1

  • In another review of 131 cases of EN, most (60%) had noninflammatory EN, 1/3 had NS, and 6 percent had inflammatory linear verrucous EN.2

  • NS are usually present at birth or noted in early childhood.3 Most cases are sporadic but familial cases have been reported.

  • Linear NS is estimated to occur in 1 per 1000 live births.4

  • Linear NS syndrome includes a range of abnormalities ...

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