Nevus verrucosus Q82.5

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

epidermal nevus; Epidermal nevus; epidermal nevus of the hard type; Hard epidermal nevus; Hyperkeratotic nevus; Naevus epidermal hard; nevus durus; Nevus Harder; nevus hyperkeratotic; Nevus verrucous; Severe nevus; verrucosal nevus; verrucous nevus; Verrucous nevus

Definition
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The Blaschko lines of the following, possibly hemiplegic (unilateral epidermal nevus), or also bilateral localized epidermal nevus (epidermal nevus). The common epidermal nevus of the hard type (nevus verrucosus) is linear or arranged in a whirling pattern and characterized by a hard, warty, dirty-brown surface.

Occurrence/Epidemiology
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Incidence is estimated at 1:1000 births.

Manifestation
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Congenital or occurring in early childhood. 80% of nevi are diagnosable in the first year of life.

Localization
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Face, neck, torso, extremities.

Clinical features
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Bizarrely configured, often following a swirling line pattern (fountain pattern), linear or planar, often yellow or yellow-brown in infancy or toddlers, later brown or brown-black papules and/or plaques with a warty surface.

In the case of a flat cutaneous mosaic pattern, usually abrupt limitation to the midline (naevus verrucosus unius lateralis).

A bilateral, generalized distribution pattern is rarer (see fig.).

The unique turbulence pattern (see Blaschko lines below) shows the skin change as a cutaneous mosaic.

Histology
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Differential diagnosis
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Therapy
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Possibly excision or dermabrasion of cosmetically disturbing areas. Alternative therapy with 0.05% vitamin A acid cream R256, if necessary retinoids internally.

Progression/forecast
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The skin changes are usually only discreetly visible at the initial manifestation, inconspicuous, flat, surface-smooth. In the course of the years, continuously increasing, verrucous aspect.

Surprisingly, at times almost complete regression of an extensive verrucous nevus, with recurrence in the same location after several months, has been observed (see Fig.).

Note(s)
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The epidermolytic epidermal nevus is a special form of the epidermal nevus of the hard type (= nevus verrucosus). Its histological peculiarity is the granular degeneration or acanthokeratolysis. Another special form is the inflammatory epidermal nevus(ILVEN).

Hamartomas of the skin in which the epidermis and/or skin appendages are altered (e.g. sebaceous nevus) are described as organoid epidermal nevi.

Literature
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  1. Kim R et al (2013) Verrucous epidermal nevus. Dermatol Online J 19: 20707
  2. Lapidoth M et al (2013) Treatment of verrucous epidermal nevus: experience with 71 cases. Dermatology 226:342-346
  3. Miranda LQ et al (2013) Analysis of mutations in the PIK3CA and FGFR3 genes in verrucous epidermal nevus. On Bras Dermatol 88(6 Suppl 1):36-38
  4. Rogers M et al (1989) Epidermal nevi and the epidermal nevus syndrome.
    A review of 131 cases. J Am Acad Dermatol 20:476-88.

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Last updated on: 29.10.2020