Epidermal Nevus

 Localized, wart-like variants, solitary or multiple

• Nevus unius lateris
image Long, linear lesions on unilateral extremities

• Ichthyosis hystrix
image Large, bilateral lesions on trunk


• Epidermal nevus syndrome includes neurological, ocular, and skeletal abnormalities

Clinical Issues

• Common sites include neck, trunk, and extremities

• May present together with nevus sebaceus, woolly hair nevus, and nevus comedonicus

• Associated with number of diseases and syndromes; look for other clinical findings

• Small lesion can be excised, larger lesions can be treated by laser or cryotherapy


• At least 10 different patterns, > 1 pattern can coexist in single lesion

• Common pattern includes hyperkeratosis with papillomatosis and acanthosis

• Inflammatory linear verrucous epidermal nevus is considered subtype of epidermal nevus

Top Differential Diagnoses

• Seborrheic keratosis

• Acanthosis nigricans

• Confluent and reticulated papillomatosis of Gougerot and Carteaud

• Organoid nevus (nevus sebaceous)

Hyperpigmented Epidermal Nevus
An example of an epidermal nevus exhibits hyperpigmented, curvilinear, mammillated plaque image on the anterior, midline neck of a girl. (Courtesy J. Finch, MD.)

Classic Histology of Epidermal Nevus
Epidermal nevus shows orthokeratotic hyperkeratosis image overlying epidermal papillomatosis image. Note hyperpigmentation in the basal keratinocytes. (Courtesy C. Cockerell, MD.)

Hypopigmented Epidermal Nevus
In this example of epidermal nevus in another child, a cauliflower-like, hypopigmented, and exophytic tumor clinically resembles a wart. Note the finger-like projections image. (Courtesy J. Finch, MD.)

Histology Resembles Seborrheic Keratosis
Under low magnification, prominent orthokeratotic hyperkeratosis image (scale without nucleated cells) and papillomatosis image (church spire-like acanthosis) are constant features of epidermal nevus.



• Nevus verrucosus: Localized, wart-like variants, solitary or multiple

• Nevus unius lateris: Long, linear lesions on unilateral extremities

• Ichthyosis hystrix: Large, bilateral lesions on trunk


• Developmental malformation of epidermis with hyperplasia of keratinocytes

• Specific entity that does not include adnexal malformations or appendageal tumors, such as organoid/sebaceous nevus


Epidermal Nevus Syndrome

• Epidermal nevi (usually extensive) with
image Neurological abnormalities (epilepsy, mental retardation)

image Ocular abnormalities (cataracts)

image Skeletal anomalies (kyphoscoliosis, limb hypertrophy)

• Various systemic cancers at young age

Other Associations

• Polyostotic fibrous dysplasia

• Proteus syndrome

• Phakomatosis pigmentokeratotica



• Age
image Usually present at birth; develops during early childhood or can be seen in adulthood

Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Epidermal Nevus
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