Skip to Main Content

++
Patient Story
++

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.

++
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.)

Graphic Jump Location
++
Introduction
++

  • 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.

++
Synonyms
++

  • 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.

++
FIGURE 145-2

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

Graphic Jump Location
++
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.)

Graphic Jump Location
++
Epidemiology
++

  • 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 ...

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessPediatrics Full Site: One-Year Subscription

Connect to the full suite of AccessPediatrics content and resources including 20+ textbooks such as Rudolph’s Pediatrics and The Pediatric Practice series, high-quality procedural videos, images, and animations, interactive board review, an integrated pediatric drug database, and more.

$595 USD
Buy Now

Pay Per View: Timed Access to all of AccessPediatrics

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.