Epidermodysplasia verruciformis

Summarize this article; https://www.statpearls.com/kb/viewarticle/21213/ Article Author: David Myers Article Editor: Eric Fillman Updated: 1/13/2019 11:27:37 PM PubMed Link: Epidermodysplasia Verruciformis Introduction The connection between viral infection and certain forms of carcinogenesis has been well established, especially with regards to human papillomavirus (HPV). Specific types of HPV have been implicated more strongly with carcinogenesis and the spectrum of dysplasia development. Epidermodysplasia verruciformis (EV) is a genetic dermatologic condition in which patients show a decreased immunologic ability to defend against and eradicate certain types of HPV, leading to persistent infection and increased lifetime risk of development of cutaneous dysplasia and malignancy. Persons with this disease develop HPV-derived cutaneous lesions at a much higher rate than the general population. This genodermatosis manifests mainly as verrucous cutaneous lesions such as multiple persistent verrucae, pityriasis versicolor-like lesions, and other verrucous or “warty” cutaneous lesions as well as the development of Bowen disease and squamous cell carcinoma. The lesions of EV tend to exhibit a characteristic histopathologic appearance. EV is a very rare genetic disease, but much research has been done on this entity as it has allowed insights into viral infection and their role in carcinogenesis pathways. There are broadly 2 forms of EV, the classic form being the inherited or primary type, inherited in an autosomal recessive pattern, while a separate acquired or secondary type is a clinically almost indistinguishable condition that is observed mainly in HIV-infected, immunocompromised, or immunosuppressed individuals.[1][2] Etiology Epidermodysplasia verruciformis (EV) is caused by a reduced ability by the immune system to fend off and eradicate human papillomavirus (HPV) infection. Since EV was first described in 1922 by Lewandowsky and Lutz[3], many different types of HPV have been implicated in the development of cutaneous lesions in patients with EV. The most commonly identified HPV types found in EV related cancers are HPV5 and HPV8. These 2 types have been found to be present in up to 90% of EV-related skin cancers.[4] Other types of HPV that are alternatively commonly found include 9, 12, 14, 15, 17, 19, 20, 21, 22, 23, 24, 25, 36, 38, 47, and 50.[5] One study showed that HPV5 was the most common single type isolated, and contrary to other literature results, HPV3, HPV14, and HPV20 were more commonly isolated than HPV8.[6] This autosomal recessive condition in its primary (in other words, inherited) form is caused by a mutation of TMC6/EVER1 or TMC8/EVER2, which is believed to impart a defect in the ability to mount an immune response to certain HPV types within keratinocytes.[7] Interestingly, these patients with typical EV do not generally display reduced immune capabilities against other infectious pathogens. The beta-HPV types identified in patients with EV who develop skin malignancies are found throughout the general population, and in persons without the EVER mutations or EV, these HPV types have not been shown to produce dysplasia or malignancy otherwise.[8]