| 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). |
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| | 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 |
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| 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. |
| Human papillomaviruses are commonly found in normal skin of immunocompetent hosts. |
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