Inflammatory linear verrucous epidermal nevus

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Classification according to ICD-10
Q82.5 Congenital non-neoplastic nevus
D23.- Other benign neoplasms of connective and other soft tissues
ICD-10 online (WHO version 2019)
Verrucous linear plaque after the lines of Blaschko above the right upper arm of a young girl

The inflammatory linear verrucous epidermal nevus (ILVEN) is a form of an epidermal nevus with sharply demarcated , striped eczema- , lichen- or psoriasis-like papules .

Synonyms are: Inflammatory linear verrucous epidermal nevus

The first description comes from Paul Gerson Unna from 1894 (quoted from)

distribution

The frequency is not known. The female gender is affected more often in a ratio of 4 to 1. There is rarely an association with the epidermal nevus syndrome ; malignant degeneration is possible.

root cause

The disease is based on mutations in somatic cells (somatic mutation) in the form of a mosaic .

Clinical manifestations

Clinical criteria are:

  • Congenital or occurring in early childhood. Rarely first occurring in adulthood.
  • The left leg is often affected, but also the trunk and, less often, the face and neck .
  • itching
  • Sharply demarcated eczematous, lichenoid or psoriasiform papules following the Blaschko lines

diagnosis

The diagnosis is based on histology .

therapy

A causal treatment is not known, the disease is considered therapy-resistant .

Various therapeutic approaches have been described as successful: CO2 laser ablation , treatment with etanercept and calcipotriol .

literature

  • M. Ruggieri and JL Sugarman: Inflammatory Linear Verrucous Epidermal Nevus (Ilven). In: M. Ruggieri, I. Pascual-Castroviejo, C. Di Rocco (editors): Neurocutaneous Disorders Phakomatoses and Hamartoneoplastic Syndromes. Springer, 2008, pp. 575-580.
  • R. Happle: Linear psoriasis and ILVEN: is lumping or splitting appropriate? In: Dermatology. Vol. 212, No. 2, 2006, pp. 101-102, doi: 10.1159 / 000090647 , PMID 16484813 .

Individual evidence

  1. ↑ Epidermal nevus, inflammatory linear verrucous. In: Orphanet (Rare Disease Database).
  2. ↑ a b c Encyclopedia Dermatology
  3. ↑ PG Unna: The histopathology of skin diseases. Hirschwald Verlag, Berlin, 1994
  4. ↑ a b c d J. Altman, AH Mehregan: Inflammatory linear verrucose epidermal nevus. In: Archives of dermatology. Vol. 104, No. 4, October 1971, pp. 385-389, PMID 5114412 .
  5. ↑ a b Rare Diseases
  6. ↑ R. Happle: epidermal nevus syndromes. In: Seminars in dermatology. Vol. 14, No. 2, June 1995, pp. 111-121, PMID 7640190 .
  7. ↑ S. Gianfaldoni, G. Tchernev, R. Gianfaldoni, U. Wollina, T. Lotti: A Case of "Inflammatory linear verrucous epidermal nevus" (ILVEN) Treated with CO2 laser ablation. In: Open access Macedonian journal of medical sciences. Vol. 5, No. 4, July 2017, pp. 454–457, doi: 10.3889 / oamjms.2017.078 , PMID 28785331 , PMC 5535656 (free full text).
  8. ^ R. Renner, A. Colsman, M. Sticherling: ILVEN: is it psoriasis? Debate based on successful treatment with etanercept. In: Acta dermato-venereologica. Vol. 88, No. 6, 2008, pp. 631-632, doi: 10.2340 / 00015555-0522 , PMID 19002357 .
  9. ^ I. Bohm, T. Bieber, R. Bauer: Successful therapy of an ILVEN in a 7-year-old girl with calcipotriol. In: The dermatologist; Journal of Dermatology, Venereology, and Allied Fields. Vol. 50, No. 11, November 1999, pp. 812-814, PMID 10591793 (review).