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Epidermal nevus - Skin in Child
See also in: Anogenital
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Epidermal nevus - Skin in Child

See also in: Anogenital
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Contributors: Sarah Stein MD, Karen Wiss MD, Sheila Galbraith MD, Craig N. Burkhart MD, Dean Morrell MD, Cynthia Christy MD, Nancy Esterly MD
Other Resources UpToDate PubMed

Synopsis

Epidermal nevi (nevi verrucosus) affect 0.1% of the population and are usually recognized at birth but may not appear until childhood when they are seen as a linear array of smooth, hyperpigmented, thin plaques or rough skin-colored papules and plaques. Epidermal nevi are hamartomas of ectodermal origin; the term encompasses a variety of lesions with various histologic and clinical patterns. The most typical presentation is as a linear verrucous epidermal nevus. Most develop sporadically, although familial cases have been reported.

Occasionally, epidermal nevi may be associated with other cutaneous, CNS, skeletal, and ocular abnormalities previously known as epidermal nevus syndrome. This entity is now thought to include many distinct genetic diseases, all sharing a phenotype reflecting genetic mosaicism.

Cutaneous skeletal hypophosphatemia syndrome, which has epidermal or melanocytic nevi, elevated fibroblast growth factor-23, and hypophosphatemia, has been found to have a genetic mosaicism of the RAS gene in affected tissues.

Codes

ICD10CM:
D23.9 – Other benign neoplasm of skin, unspecified

SNOMEDCT:
239107007 – Epidermal nevus

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Verruca vulgaris – Verrucous papules and plaques, however, do not tend to assume a whorled pattern and do not respect the midline.
  • Linear lichen planus
  • Epidermal nevi may reflect a somatic mosaicism for K1/K10 mutations as seen in epidermolytic ichthyosis. If these patients have gonadal mosaicism, offspring may have full-blown epidermolytic ichthyosis. Mosaicism for ATP2A2 mutations can cause segmental Darier's disease. As in epidermolytic ichthyosis, if these patients have gonadal mosaicism, offspring may have full-blown Darier's disease.
  • Nevus sebaceus – Papillomatous, yellow-orange, linear plaque on the scalp or face.
  • Psoriasis – May present in a segmental fashion (nevoid psoriasis) but responds to topical steroids.
  • Inflammatory linear verrucous epidermal nevus (ILVEN) – Congenital pruritic, linear, psoriasiform plaque.
  • Lichen striatus – Presents in childhood as asymptomatic linearly arranged, small, flat-topped, pink to skin-colored papules within the lines of Blaschko, usually on an extremity. These lesions spontaneously resolve over months to a few years.
  • Mosaic Darier's disease – Skin biopsy reveals acantholytic dyskeratosis.

Best Tests

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Therapy

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References

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Last Updated: 07/28/2016
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Epidermal nevus - Skin in Child
See also in: Anogenital
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Epidermal nevus : Linear configuration, Verrucous scaly plaque, Whorled configuration
Clinical image of Epidermal nevus
Scaly, verrucous papules and plaques following Blaschko lines on the leg.
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