Epidermal nevus

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Classification according to ICD-10
D23.- Other benign neoplasms of the skin
ICD-10 online (WHO version 2019)

The epidermal nevus is a congenital hamartoma (nevus) derived from the ectoderm with a striped, usually brown-colored thickening of the epidermis and hyperkeratosis .

The epidermal changes follow the Blaschko lines , are already present at birth or occur in the first years of life.

If other organ systems are also involved - as is the case with a third of those affected - this is called an epidermal nevus syndrome (ENS, Solomon syndrome)

A combination of epidermal nevus and neurological changes is considered to be Schimmelpenning-Feuerstein-Mims syndrome .

Histopathological classification

Epidermal nevi can be classified according to their main histological component:

classification

The following clinical classification is used:

distribution

The frequency is given as 1–3 in 1,000 people. The nevi can be visible at birth or develop in childhood .

root cause

There does not seem to be a single cause. Mutations in the FGFR3 and PIK3CA genes are found in around 40% of keratinocytic epidermal nevi, mutations in the HRAS gene can be detected in nevus sebaceus, but also in keratinocytic epidermal nevi. Mutations in the KRAS and NRAS genes are less common.

All of the genes mentioned are involved in “cellular signaling”, a process that regulates the growth and division of skin cells. The mutations are only present in the nevus cells, not in the adjacent normal skin, a so-called mosaic .

diagnosis

The diagnosis is made on the basis of the clinic and histopathology .

Differential diagnosis

Demarcate are strip-shaped Psoriasis and Lichen other forms strip-shaped Lichen forms, in particular lichen planus , further

history

The first description as "nevus unius lateris" for a systematized verrucous nevus is said to come from the year 1863 by Friedrich Wilhelm Felix von Bärensprung . (quoted from)

literature

  • CA Zahn, P. Itin: Papular Epidermal Nevus with "Skyline" Basal Cell Layer Syndrome - Natural Course: Case Report and Literature Review. In: Case reports in dermatology. Vol. 9, No. 1, 2017 Jan – Apr, pp. 1–5, doi: 10.1159 / 000454757 , PMID 28203156 , PMC 5301115 (free full text).
  • R. Kim, S. Marmon, J. Kaplan, H. Kamino, MK Pomeranz: Verrucous epidermal nevus. In: Dermatology online journal. Vol. 19, No. 12, December 2013, p. 20707, PMID 24364998 .
  • ME Gonzalez, A. Jabbari, BE Tlougan, R. Mandal, JV Schaffer: Epidermal nevus. In: Dermatology online journal. Vol. 16, No. 11, November 2010, p. 12, PMID 21163163 .

Individual evidence

  1. ↑ a b c d Entry on epidermal nevus in the Flexikon , a Wiki of the DocCheck company
  2. ↑ a b c d emedicine.medscape
  3. ↑ a b Encyclopedia Dermatology
  4. ^ WP Su: Histopathologic varieties of epidermal nevus. A study of 160 cases. In: The American Journal of Dermatopathology . Vol. 4, No. 2, April 1982, pp. 161-170, PMID 7048967 (review).
  5. ↑ a b Genetics Home Reference
  6. ↑ Ichthyosis, epidermolytic. In: Orphanet (Rare Disease Database).
  7. ^ F. Von Baerensprung: Naevus unius lateris. In: Charité-Annalen , Vol. XI, 1863
  8. ↑ Lawrence M. Solomon, Nancy B. Esterly: Epidermal and other congenital organoid nevi. In: Current Problems in Pediatrics. Vol. 6, 1975, p. 3, doi: 10.1016 / S0045-9380 (75) 80010-7 .

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