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Inflammatory linear verrucous epidermal nevus in Adult
See also in: Anogenital
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Inflammatory linear verrucous epidermal nevus in Adult

See also in: Anogenital
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Contributors: Craig N. Burkhart MD, Dean Morrell MD, Whitney A. High MD, JD, MEng
Other Resources UpToDate PubMed

Synopsis

Inflammatory linear verrucous epidermal nevus (ILVEN) is a type of epidermal nevus that results in a linear, hyperkeratotic plaque on the skin. The condition occurs most often on the buttocks and lower extremities, particularly in girls, and often on the left side of the body.

ILVEN is an ectodermal disorder, present from birth, but it typically only becomes clinically apparent in early childhood. While typically sporadic in occurrence, familial forms have been described. Pruritus is common with ILVEN, and the condition is typically only partially responsive to management.

The cause of ILVEN is unknown. Localized upregulation of interleukin (IL)-1, IL-6, tumor necrosis factor alpha (TNF-alpha), and intercellular adhesion molecule 1 (ICAM-1) may play a role in its development and perpetuation.

Codes

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

SNOMEDCT:
399995006 – Inflammatory linear verrucous epidermal nevus

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Other diagnoses likely to create a linear hyperkeratotic lesion in childhood are:
  • Linear psoriasis – Some authors have postulated a relationship between linear psoriasis and ILVEN, and the independent existence of the two conditions have been questioned. Histopathology of ILVEN and linear psoriasis greatly overlap.
  • Linear porokeratosis – Unlike ILVEN, linear porokeratosis may occur in adulthood, but it also occurs more often in females and follows the lines of Blaschko. Skin cancer can arise within linear porokeratosis. Histopathology of linear porokeratosis maintains characteristic coronoid lamellae.
  • Lichen striatus – A self-limited, unilateral inflammatory dermatosis of erythematous, flat-topped papules with scale arranged in a linear band that progresses down an extremity. Histopathology reveals a lichenoid condition with interface reaction and a deeper periadnexal lymphocytic infiltrate.
  • Papular epidermal nevus with "skyline" basal cell layer (PENS) is a rare newly described entity where affected children develop few hyperkeratotic polygonal or rectangular papules in one or more body locations. PENS syndrome describes a subset of patients with PENS with associated neurological findings such as epilepsy or neurodevelopmental delay.

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Last Updated: 05/06/2019
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Inflammatory linear verrucous epidermal nevus in Adult
See also in: Anogenital
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Inflammatory linear verrucous epidermal nevus : Leg, Linear configuration, Unilateral, Whorled configuration, Verrucous scaly papules, Blanching patches, Scaly plaques
Clinical image of Inflammatory linear verrucous epidermal nevus
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