U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Epidermodysplasia verruciformis(EV)

MedGen UID:
41831
•Concept ID:
C0014522
•
Neoplastic Process
Synonyms: Disease, Lewandowsky-Lutz; Disease, Lutz-Lewandowsky; Epidermodysplasia Verruciformis; Lewandowsky Lutz Disease; Lewandowsky-Lutz Disease; Lutz Lewandowsky Disease; Lutz-Lewandowsky Disease
SNOMED CT: Epidermodysplasia verruciformis (19138001); Lewandowsky-Lutz disease (19138001)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
•Concept ID:
C0441748
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
Autosomal recessive inheritance (Orphanet)
 
Monarch Initiative: MONDO:0009176
OMIM®: 226400
Orphanet: ORPHA302

Definition

Epidermodysplasia verruciformis (EV) is a rare inherited genodermatosis characterized by chronic infection with human papillomavirus (HPV) leading to polymorphous cutaneous lesions and high risk of developing non melanoma skin cancer. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpidermodysplasia verruciformis
Follow this link to review classifications for Epidermodysplasia verruciformis in Orphanet.

Professional guidelines

PubMed

Multimodal Treatment of Epidermodysplasia Verruciformis in an HIV-Positive Man.
Boettler MA, Cartron AM, Shearer SM, Chung C, Trinidad J
Cutis 2023 Feb;111(2):E13-E15. doi: 10.12788/cutis.0726. PMID: 37075182
Clinical guideline for the diagnosis and treatment of cutaneous warts (2022).
Zhu P, Qi RQ, Yang Y, Huo W, Zhang Y, He L, Wang G, Xu J, Zhang F, Yang R, Tu P, Ma L, Liu Q, Li Y, Gu H, Cheng B, Chen X, Chen A, Xiao S, Jin H, Zhang J, Li S, Yao Z, Pan W, Yang H, Shen Z, Cheng H, Song P, Fu L, Chen H, Geng S, Zeng K, Wang J, Tao J, Chen Y, Wang X, Gao XH
J Evid Based Med 2022 Sep;15(3):284-301. Epub 2022 Sep 18 doi: 10.1111/jebm.12494. PMID: 36117295Free PMC Article
Acquired epidermodysplasia verruciformis: clinical presentation and treatment update.
Moore S, Rady P, Tyring S
Int J Dermatol 2022 Nov;61(11):1325-1335. Epub 2021 Aug 17 doi: 10.1111/ijd.15857. PMID: 34403500

Suggested Reading

PubMed

Genetics of Skin Cancer (PDQ®): Health Professional Version.
PDQ Cancer Genetics Editorial Board
2002 PMID: 26389333
Cancer Genetics Risk Assessment and Counseling (PDQ®): Health Professional Version.
PDQ Cancer Genetics Editorial Board
2002 PMID: 26389258

Recent clinical studies

Etiology

Condyloma and coincidental epidermodysplasia verruciformis acanthoma positive for human papillomavirus-14 and -21.
Bartley B, Cho WC, Rady PL, Dai J, Curry JL, Milbourne A, Tyring SK, Torres-Cabala CA
J Cutan Pathol 2023 Jan;50(1):47-50. Epub 2022 Sep 14 doi: 10.1111/cup.14319. PMID: 36039682
Skin Cancer Associated Genodermatoses: A Literature Review.
Schierbeck J, Vestergaard T, Bygum A
Acta Derm Venereol 2019 Apr 1;99(4):360-369. doi: 10.2340/00015555-3123. PMID: 30653245
Epidermodysplasia verruciformis: three case reports and a brief review.
Shruti S, Siraj F, Singh A, Ramesh V
Acta Dermatovenerol Alp Pannonica Adriat 2017 Sep;26(3):59-61. doi: 10.15570/actaapa.2017.19. PMID: 28941263
Epidermodysplasia verruciformis.
Burger B, Itin PH
Curr Probl Dermatol 2014;45:123-31. Epub 2014 Mar 17 doi: 10.1159/000356068. PMID: 24643182
HPV-associated skin disease.
Akgül B, Cooke JC, Storey A
J Pathol 2006 Jan;208(2):165-75. doi: 10.1002/path.1893. PMID: 16362995

Diagnosis

Acquired Epidermodysplasia Verruciformis in a Solid Organ Transplant Recipient.
di Prinzio A, Torre AC, Mazzuoccolo LD
Actas Dermosifiliogr 2023 Nov-Dec;114(10):911. Epub 2023 Apr 21 doi: 10.1016/j.ad.2022.11.014. PMID: 37088290
Clinical guideline for the diagnosis and treatment of cutaneous warts (2022).
Zhu P, Qi RQ, Yang Y, Huo W, Zhang Y, He L, Wang G, Xu J, Zhang F, Yang R, Tu P, Ma L, Liu Q, Li Y, Gu H, Cheng B, Chen X, Chen A, Xiao S, Jin H, Zhang J, Li S, Yao Z, Pan W, Yang H, Shen Z, Cheng H, Song P, Fu L, Chen H, Geng S, Zeng K, Wang J, Tao J, Chen Y, Wang X, Gao XH
J Evid Based Med 2022 Sep;15(3):284-301. Epub 2022 Sep 18 doi: 10.1111/jebm.12494. PMID: 36117295Free PMC Article
Skin Cancer Associated Genodermatoses: A Literature Review.
Schierbeck J, Vestergaard T, Bygum A
Acta Derm Venereol 2019 Apr 1;99(4):360-369. doi: 10.2340/00015555-3123. PMID: 30653245
Epidermodysplasia verruciformis in an HIV-infected man: a case report and review of the literature.
Kaushal A, Silver S, Kasper K, Severini A, Hamza S, Keynan Y
Top Antivir Med 2012 Dec;20(5):173-9. PMID: 23363696Free PMC Article
Epidermodysplasia verruciformis.
Lane JE, Bowman PH, Cohen DJ
South Med J 2003 Jun;96(6):613-5. doi: 10.1097/01.SMJ.0000054544.47233.7C. PMID: 12938792

Therapy

Acquired epidermodysplasia verruciformis: clinical presentation and treatment update.
Moore S, Rady P, Tyring S
Int J Dermatol 2022 Nov;61(11):1325-1335. Epub 2021 Aug 17 doi: 10.1111/ijd.15857. PMID: 34403500
Acquired epidermodysplasia verruciformis (AEV) in three children after cardiac transplantation: A case series and review of the literature.
Kinariwalla N, Coromilas AJ, Garzon MC, Levin LE, Magro C, Lauren CT
Pediatr Dermatol 2021 Nov;38(6):1541-1545. Epub 2021 Oct 20 doi: 10.1111/pde.14701. PMID: 34669978
Tumour-inducing viruses.
Weiss RA
Br J Hosp Med (Lond) 2016 Oct;77(10):565-568. doi: 10.12968/hmed.2016.77.10.565. PMID: 27723397
HPV-associated skin disease.
Akgül B, Cooke JC, Storey A
J Pathol 2006 Jan;208(2):165-75. doi: 10.1002/path.1893. PMID: 16362995
Epidermodysplasia verruciformis: immunological and clinical aspects.
Jablonska S, Majewski S
Curr Top Microbiol Immunol 1994;186:157-75. doi: 10.1007/978-3-642-78487-3_9. PMID: 8205840

Prognosis

Acquired epidermodysplasia verruciformis (AEV) in three children after cardiac transplantation: A case series and review of the literature.
Kinariwalla N, Coromilas AJ, Garzon MC, Levin LE, Magro C, Lauren CT
Pediatr Dermatol 2021 Nov;38(6):1541-1545. Epub 2021 Oct 20 doi: 10.1111/pde.14701. PMID: 34669978
Cervical dysplasia in a patient with inherited epidermodysplasia verruciformis-A mere coincidence?
Singh AP, Jeffus SK, Shalin SC
J Cutan Pathol 2021 Jun;48(6):763-770. Epub 2020 Dec 22 doi: 10.1111/cup.13937. PMID: 33319409
Lesions With an Epidermal Hyperplastic Pattern: Morphologic Clues in the Differential Diagnosis.
Fernandez-Flores A
Am J Dermatopathol 2016 Jan;38(1):1-16; quiz 17-9. doi: 10.1097/DAD.0000000000000324. PMID: 26730692
Flat-topped and condylomatous papules in a lung transplant patient.
Hoesly F, Cappel M
JAMA Dermatol 2014 Mar;150(3):327-8. doi: 10.1001/jamadermatol.2013.6332. PMID: 24402672
The EVER proteins as a natural barrier against papillomaviruses: a new insight into the pathogenesis of human papillomavirus infections.
Lazarczyk M, Cassonnet P, Pons C, Jacob Y, Favre M
Microbiol Mol Biol Rev 2009 Jun;73(2):348-70. doi: 10.1128/MMBR.00033-08. PMID: 19487731Free PMC Article

Clinical prediction guides

Recalcitrant Warts, Epidermodysplasia Verruciformis, and the Tree-Man Syndrome: Phenotypic Spectrum of Cutaneous Human Papillomavirus Infections at the Intersection of Genetic Variability of Viral and Human Genomes.
Uitto J, Saeidian AH, Youssefian L, Saffarian Z, Casanova JL, Béziat V, Jouanguy E, Vahidnezhad H
J Invest Dermatol 2022 May;142(5):1265-1269. Epub 2021 Nov 27 doi: 10.1016/j.jid.2021.10.029. PMID: 34843682Free PMC Article
Keratinocyte Carcinoma as a Marker of a High Cancer-Risk Phenotype.
Small J, Barton V, Peterson B, Alberg AJ
Adv Cancer Res 2016;130:257-91. Epub 2016 Feb 23 doi: 10.1016/bs.acr.2016.01.003. PMID: 27037755
Seborrheic keratosis-like lesions in epidermodysplasia verruciformis.
Tomasini C, Aloi F, Pippione M
J Cutan Pathol 1993 Jun;20(3):237-41. doi: 10.1111/j.1600-0560.1993.tb00649.x. PMID: 8396161
The human papillomaviruses.
Orth G, Jablonska S, Breitburd F, Favre M, Croissant O
Bull Cancer 1978;65(2):151-64. PMID: 212142
Epidermodysplasia verruciformis and epidermoid carcinoma. Electron microscopic observations.
Aaronson CM, Lutzner MA
JAMA 1967 Sep 4;201(10):775-7. PMID: 4291940

Recent systematic reviews

Acquired epidermodysplasia verruciformis syndrome in HIV-infected patients: a systematic review.
Cuestas D, Gómez A, Neri Morales C, Motta A, Rolon M, Suarez S, Polo R
Arch Dermatol Res 2024 Jul 13;316(7):471. doi: 10.1007/s00403-024-03016-x. PMID: 39001899Free PMC Article
Monogenic etiologies of persistent human papillomavirus infections: A comprehensive systematic review.
Biglari S, Moghaddam AS, Tabatabaiefar MA, Sherkat R, Youssefian L, Saeidian AH, Vahidnezhad F, Tsoi LC, Gudjonsson JE, Hakonarson H, Casanova JL, Béziat V, Jouanguy E, Vahidnezhad H
Genet Med 2024 Feb;26(2):101028. Epub 2023 Nov 14 doi: 10.1016/j.gim.2023.101028. PMID: 37978863Free PMC Article
The role of HPV in keratinocyte skin cancer development: A systematic review.
Neagu N, Dianzani C, Venuti A, Bonin S, Voidăzan S, Zalaudek I, Conforti C
J Eur Acad Dermatol Venereol 2023 Jan;37(1):40-46. Epub 2022 Sep 1 doi: 10.1111/jdv.18548. PMID: 36000380
Human papilloma virus infection in basal cell carcinoma of the skin: a systematic review and meta-analysis study.
Ramezani M, Sadeghi M
Pol J Pathol 2017;68(4):330-342. doi: 10.5114/pjp.2017.73929. PMID: 29517204
Novel TMC8 splice site mutation in epidermodysplasia verruciformis and review of HPV infections in patients with the disease.
Imahorn E, Yüksel Z, Spoerri I, Gürel G, Imhof C, Saraçoğlu ZN, Koku Aksu AE, Rady PL, Tyring SK, Kempf W, Itin PH, Burger B
J Eur Acad Dermatol Venereol 2017 Oct;31(10):1722-1726. Epub 2017 Aug 4 doi: 10.1111/jdv.14431. PMID: 28646613

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • GTR
      Related information in GTR
    • GTR(Clinical)
      Clinical tests in GTR
    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed
    • PubMed (OMIM)
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...