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Inflammatory linear verrucous epidermal nevus - Anogenital in
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Inflammatory linear verrucous epidermal nevus - Anogenital in

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Contributors: Nnenna Agim MD, FAAD, Craig N. Burkhart MD, Dean Morrell MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Inflammatory linear verrucous epidermal nevus (ILVEN) is a blaschkoid dermatosis that presents with erythematous, hyperkeratotic plaques following Blaschko lines. It occurs most often on the buttocks, perineal area, and lower extremities, particularly in girls, and often on the left side of the body.

ILVEN typically becomes clinically apparent in early childhood; however, adult-onset variants have been reported. While typically sporadic in occurrence, familial forms have been described. Pruritus is common with ILVEN, and the condition is typically only partially responsive to topical management.

ILVEN was historically classified as a type of epidermal nevus. However, current thinking by many experts is that, rather than being a true epidermal nevus, ILVEN may represent a mosaic inflammatory disorder akin to linear psoriasis and linear lichen planus or a mosaic ichthyosiform disorder. This is theorized because ILVEN has shown response to therapies for psoriasis, such as topical calcipotriene, topical corticosteroids, 308 nm excimer laser, and biologics such as etanercept. ILVEN has been reported in patients who go on to develop widespread psoriasis. Additionally, further investigation has shown aberrant interleukin (IL)-36R / IL-17R pathways in ILVEN, similar to psoriasis and psoriasiform ichthyoses. Further, localized upregulation of IL-1, IL-6, tumor necrosis factor alpha (TNF-α), and intercellular adhesion molecule 1 (ICAM-1) may play a role in its development and perpetuation. About 10% of patients were genetic mosaics for CARD14 in DNA from blood and affected skin.

Some cases of ILVEN have been associated with autoimmune thyroiditis. A rare complication of ILVEN is development of superimposed verruciform xanthomas in adulthood.

Codes

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

SNOMEDCT:
399995006 – Inflammatory linear verrucous epidermal nevus

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Last Reviewed:04/26/2020
Last Updated:01/04/2022
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Inflammatory linear verrucous epidermal nevus - Anogenital in
See also in: Overview
A medical illustration showing key findings of Inflammatory linear verrucous epidermal nevus : Leg, Linear configuration, Whorled configuration, Verrucous scaly papules, Scaly plaques
Clinical image of Inflammatory linear verrucous epidermal nevus - imageId=2308799. Click to open in gallery.  caption: 'A linear array of flat, pink and reddish, scaly papules and plaques on and around the popliteal fossa.'
A linear array of flat, pink and reddish, scaly papules and plaques on and around the popliteal fossa.
Copyright © 2024 VisualDx®. All rights reserved.