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Epidermodysplasia verruciformis

Written1998-06Claude Viguié
Service de Dermatologie, Hôpital Tarnier-Cochin, 89 rue d'Assas, 75006 Paris, France

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Other namesLevandowsky-Lutz syndrome
Atlas_Id 10019
Genes implicated inTMC6   TMC8  
Inheritance autosomal recessive in 10 to 20 % of the patients; other cases are sporadic; rare disease.


Note epidermodysplasia verruciformis (EV) is a model of malignant transformation from benign cutaneous viral lesion.
Phenotype and clinics
  • age at onset is variable; more frequently: young adults or children
  • two types of elementary cutaneous lesions are observed :
    - persistant papule-like warts, isolated, or confluent with a psoriasic aspect
    - white spots, pityriasis versicolor-like
    both types of lesions are localized mainly on the outer part of the hands, on foreharms, legs, face, trunk and perianal zone.
  • immunodeficiency: decreased immune response of T lymphocytes to mitogens, decreased humoral response to Human Papilloma Virus antigens; EV lesions have been described in renal transplant recipients.
  • various Human Papilloma Virus (HPV) subtypes are regularly detected in the cutaneous lesions: HPV 5, 8, 9, 12, 14, 15, 17, 19, 25, 36, 38, 47, 50.
  • patients are simultaneously affected by differents HPV subtypes, according to disease localisation; these subtypes are different from those observed in common warts (HPV 2, 3, 4, 10).
      Top left: numerous papule-like warts on the skin of an hand; right: other aspect of papule-like warts on one hand; bottom left: basocellular carcinoma of the face developped from epidermodysplasia verruciformis lesions; right: carcinoma of the face developped from epidermodysplasia verruciformis lesions - Courtesy Daniel Wallach
    Neoplastic risk
  • risk of malignant transformation of cutaneous lesions is within a delay of 20 to 30 yrs (very slow process comparable to the genital carcinogenesis associated with high risk HPVs)
  • cytology: squamous cell carcinoma (spinocellular or basocellular carcinoma, Bowen disease).
  • correlation with oncogenic subtypes of HPV found in the transformed lesions: HPV 5, 8, 14; the most frequent subtypes are HPV 5 and 8 (90% of cases).
  • benign familial forms are associated with HPV 3, without malignant evolution.
  • HPV-5 is present in the macular lesions.
  • probable potentialisation by UV ligth: 25 to 30% of malignant lesions localised to the face and forehead (hypothetic role of P53 mutations).
  • protein E6 and/or E7 (tumor suppressor function) from HPV seem to be involved in the malignant transformation.
  • Treatment surgical resection of localized lesions; chemotherapy with Acitretin (25 mg/j) for multifocal lesions.
    Evolution local recidives, enhanced by UV exposition.

    Genes involved and Proteins

    Complementation groups genes and proteins are unknown.


    Human papillomaviruses are commonly found in normal skin of immunocompetent hosts.
    Astori G, Lavergne D, Benton C, Höckmayr B, Egawa K, Garbe C, de Villiers EM
    The Journal of investigative dermatology. 1998 ; 110 (5) : 752-755.
    PMID 9579540
    Detection of new human papillomavirus sequences in skin lesions of a renal transplant recipient and characterization of one complete genome related to epidermodysplasia verruciformis-associated types.
    Bens G, Wieland U, Hofmann A, Höpfl R, Pfister H
    The Journal of general virology. 1998 ; 79 ( Pt 4) : 779-787.
    PMID 9568973
    Epidermodysplasia verruciformis with numerous squamous cell carcinomas.
    Bonvalet D, BlanchetBardon C, Verola O
    Nouv Dermatol. 1997.
    Toxic epidermal necrolysis in Singapore, 1989 through 1993: incidence and antecedent drug exposure.
    Chan HL
    Archives of dermatology. 1995 ; 131 (10) : 1212-1213.
    PMID 7574849
    Psoriasis: A possible reservoir for human papillomavirus type 5, the virus associated with skin carcinomas of epidermodysplasia verruciformis.
    Favre M, Orth G, Majewski S, Baloul S, Pura A, Jablonska S
    The Journal of investigative dermatology. 1998 ; 110 (4) : 311-317.
    PMID 9540967
    Human papillomavirus DNA in non-melanoma skin cancers of a renal transplant recipient: detection of a new sequence related to epidermodysplasia verruciformis associated types.
    Höpfl R, Bens G, Wieland U, Petter A, Zelger B, Fritsch P, Pfister H
    The Journal of investigative dermatology. 1997 ; 108 (1) : 53-56.
    PMID 8980287
    E7 proteins of four groups of human papillomaviruses, irrespective of their tissue tropism or cancer association, possess the ability to transactivate transcriptional promoters E2F site dependently.
    Hiraiwa A, Kiyono T, Suzuki S, Ohashi M, Ishibashi M
    Virus genes. 1996 ; 12 (1) : 27-35.
    PMID 8879118
    Skin autografts in epidermodysplasia verruciformis: human papillomavirus-associated cutaneous changes need over 20 years for malignant conversion.
    Majewski S, Jablonska S
    Cancer research. 1997 ; 57 (19) : 4214-4216.
    PMID 9331078
    p53 mutations implicate sunlight in post-transplant skin cancer irrespective of human papillomavirus status.
    McGregor JM, Berkhout RJ, Rozycka M, ter Schegget J, Bouwes Bavinck JN, Brooks L, Crook T
    Oncogene. 1997 ; 15 (14) : 1737-1740.
    PMID 9349508
    Regulatory interactions of transcription factor YY1 with control sequences of the E6 promoter of human papillomavirus type 8.
    Pajunk HS, May C, Pfister H, Fuchs PG
    The Journal of general virology. 1997 ; 78 ( Pt 12) : 3287-3295.
    PMID 9400979
    [HPV-5 typing with nested PCR and sequencing in epidermodysplasia verruciformis]
    Schaller J, Rohwedder A, Fuchs M, Maron A, Kunze J
    Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete. 1996 ; 47 (6) : 454-458.
    PMID 8767661
    E2 represses the late gene promoter of human papillomavirus type 8 at high concentrations by interfering with cellular factors.
    Stubenrauch F, Leigh IM, Pfister H
    Journal of virology. 1996 ; 70 (1) : 119-126.
    PMID 8523515


    This paper should be referenced as such :
    Viguié, C
    Epidermodysplasia verruciformis
    Atlas Genet Cytogenet Oncol Haematol. 1998;2(4):159-160.
    Free journal version : [ pdf ]   [ DOI ]
    On line version :

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