What is epidermodysplasia verruciformis (EV)?


A, Multiple reddish-brown macules of the back in a patient with epidermodysplasia verruciformis. B, 'Kissing' warts produced by inoculation of the toe web space.  (Courtesy of James E. Fitzpatrick, MD.)
Fig. 26.2 A, Multiple reddish-brown macules of the back in a patient with epidermodysplasia verruciformis. B, 'Kissing' warts produced by inoculation of the toe web space. (Courtesy of James E. Fitzpatrick, MD.)
EV is a rare, inherited disorder in which cutaneous HPV infection is generalized and persistent. Most cases are autosomal recessive, but autosomal dominant and X-linked dominant forms are also reported. It is caused by mutations in either the EVER1 or EVER2 genes. The lesions are either flat warts or reddish-brown plaques, often developing in sun-exposed areas (Fig. 26-2A). Malignant change occurs in about 10% of cases, but metastasis is uncommon. HPV types 5 and 8 are most commonly seen in EV patients, although several other HPV types may also been seen.

Gül U, Kiliç A, Gönül M, et al: Clinical aspects of epidermodysplasia verruciformis and review of the literature, Int J Dermatol 46:1069– 1072, 2007.