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Epidermodysplasia verruciformis

Peter Itin, MD
Bettina Burger, DSc
Section Editor
Jennifer L Hand, MD
Deputy Editor
Rosamaria Corona, MD, DSc


Epidermodysplasia verruciformis (EV, MIM #226400) is a rare autosomal recessive skin disease characterized by an abnormally high susceptibility to infection with certain types of human papillomavirus (HPV) called EV-HPV, mostly beta-HPV [1-5]. Patients have disseminated polymorphic lesions, including flat-topped, wart-like papules and pityriasis versicolor-like macules, predominantly on skin areas exposed to sunlight. In addition, patients with EV have an increased risk of developing nonmelanoma skin cancers, mainly cutaneous squamous cell carcinomas, at an early age [6-9].

This topic will discuss the pathogenesis, clinical manifestations, diagnosis, and treatment of epidermodysplasia verruciformis. The epidemiology, virology, and natural history of HPV infection are discussed separately.

(See "Human papillomavirus infections: Epidemiology and disease associations".)

(See "Virology of human papillomavirus infections and the link to cancer".)


The prevalence of epidermodysplasia verruciformis (EV) is not known. A total of 501 patients have been described worldwide; most cases are sporadic, but there are several reports of familial cases [10].

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Literature review current through: Dec 2017. | This topic last updated: Jul 25, 2017.
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