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Epidermal nevus and epidermal nevus syndrome

Teresa S Wright, MD, FAAD, FAAP
Section Editor
Moise L Levy, MD
Deputy Editor
Rosamaria Corona, MD, DSc


Epidermal nevi are benign, hamartomatous growths of the skin that are present at birth or develop in early childhood. They may be composed of a variety of epidermal cells and structures, including keratinocytes, sebaceous glands, hair follicles, apocrine and eccrine glands, and smooth muscle cells, and are thought to represent a form of cutaneous mosaicism [1,2]. Lesions with prominent adnexal components (eg, sebaceous, follicular, and/or apocrine) are sometimes referred to as "organoid," while lesions with primarily epidermal differentiation are known as "non-organoid" or "keratinocytic" nevi.

Keratinocytic epidermal nevi, also called linear epidermal nevi or linear verrucous epidermal nevi, are the most common form. They present as linear or whorled, skin-colored to brown plaques that tend to follow linear patterns on the skin known as "lines of Blaschko" (figure 1 and picture 1A-C).

This topic will review the pathogenesis, clinical presentation, and management of linear epidermal nevus. Nevus sebaceous is discussed separately. (See "Nevus sebaceus and nevus sebaceus syndrome".)


Epidermal nevi occur in approximately 1 to 3 per 1000 live births; males and females tend to be equally affected [2]. Most epidermal nevi occur sporadically as an isolated finding, but they also may occur in association with a variety of developmental abnormalities. Familial cases have been reported [3,4].


Epidermal nevi originate from pluripotential germinative cells in the basal layer of the embryonic epidermis. They are thought to represent forms of mosaicism, resulting from postzygotic mutations (or other genetic alteration) in embryonic cells destined to populate a particular area of the epidermis. Theoretically, mutations occurring very early in embryonic development give rise to more extensive epidermal nevi and may potentially affect additional organ systems [5].

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Literature review current through: Nov 2017. | This topic last updated: Sep 06, 2017.
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