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Therapeutic Approach

Epidermal nevi (EN) comprise a heterogenous group of benign, hamartomas of the skin. Clinical appearance varies largely based on cell or structure of origin, which includes keratinocytes, the pilosebaceous unit, apocrine or eccrine glands, or smooth muscle cells. While some EN may occur as solely cutaneous lesions, they can be associated with a variety of extracutaneous manifestations involving numerous organ systems, particularly the central nervous system, skeletal system, and ocular system. The occurrence of EN in conjunction with extracutaneous findings is best umbrellaed under the term “epidermal nevi syndrome” (ENS). Management must be individually tailored and may be aimed at improving cosmesis, improving quality of life, reduction of tumor potential, reducing functional disability and potential life-threatening sequalae. See Table 98-1.

Table 98-1Epidermal Nevus Treatment Table

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