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Linear epidermal nevus - Skin
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Linear epidermal nevus - Skin

See also in: Hair and Scalp
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Contributors: Amy Fox MD, David Dasher MD, Jeffrey D. Bernhard MD, Sarah Stein MD, Karen Wiss MD, Sheila Galbraith MD, Craig N. Burkhart MD, Dean Morrell MD, Lynn Garfunkel MD, Nancy Esterly MD
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Synopsis

Epidermal nevi are hamartomatous proliferations. They may occur alone in several recognized varieties, or they may be associated with a number of other malformations, in which case they constitute the signature of several "epidermal nevus syndromes."

One specific subtype, the linear epidermal nevus, is not uncommon. Linear epidermal nevi tend to fall along relaxed skin tension lines, or in Blaschko's lines.

Histologically, linear epidermal nevi are benign growths comprised of keratinocytes, apocrine glands, eccrine glands, sebaceous glands, or components of pilosebaceous units.

Lesions occur sporadically, although they may also occur as a component of several syndromes. There is an equal prevalence between both sexes. Epidermal nevi tend to appear between birth and adolescence.

Linear epidermal nevi are traditionally asymptomatic, but a specific subtype called inflammatory linear verrucous epidermal nevus (ILVEN) can be associated with pruritus, erythema, and scaling.

Epidermal nevi comprise a heterogeneous group of diseases. Nomenclature and classification is confusing and controversial. Six different epidermal nevus syndromes have been described:
  1. Nevus sebaceous – Relatively common, these represent roughly 50% of epidermal nevi. Typically present at birth, they undergo thickening and papillomatous changes at puberty. After puberty, these can be associated with basal cell carcinoma. The syndrome implies CNS involvement, which seems to be related to extent of nevus and location.
  2. CHILD syndrome – Congenital hemidysplasia with ichthyosiform nevus and limb defect. Thought to be X-linked dominant. Unilateral inflammatory erythematous plaques with overlying yellow scale. Often occurs in body folds and is associated with ipsilateral limb hypoplasia.
  3. Nevus comedonicus – Localized collection of dilated follicles containing keratin. Can be found on the face, trunk, and upper extremities. Follicular tumors have been reported within these and are rarely associated with EEG abnormalities, skeletal abnormalities, and cataracts.
  4. Becker's nevus – Focal area of hyperpigmentation with hypertrichosis. Can have a pseudo-Darier's sign when stroked. Typically seen in late childhood or adolescence. Syndrome can be associated with skeletal abnormalities, hypoplasia of breast/musculature, and others.
  5. Proteus syndrome – Complex syndrome including overgrowth of multiple tissues with one diagnostic criterion including epidermal nevi.
  6. Phacomatosis pigmentokeratotica – Syndrome including speckled lentiginous nevi, hemiatrophy with muscle weakness, as well as epidermal nevi, among other criteria.

Codes

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

SNOMEDCT:
399995006 – Inflammatory linear verrucous epidermal nevus

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Last Updated: 02/12/2015
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Linear epidermal nevus - Skin
See also in: Hair and Scalp
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Linear epidermal nevus : Linear configuration, Verrucous scaly plaque, Verrucous scaly papules
Clinical image of Linear epidermal nevus
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