Acquired epidermodysplasia verruciformis in a renal transplant patient: a case report.

Acquired epidermodysplasia verruciformis is a rare disease. It can develop in immunocompromised patients due to infection with human papillomaviruses. Because such patients are at high risk of developing cutaneous squamous cell carcinoma, timely diagnosis and regular monitoring of the patient is essential. Here we present the case of a 46-year-old male patient with acquired epidermodysplasia verruciformis occurring 5 years after a kidney transplantation. A skin biopsy detected human papillomavirus genotype 20 with low oncogenic potential. Accordingly, a follow-up interval of 1 year was determined. He was instructed to follow strict photoprotection and to visit earlier if atypical lesions appeared. Overall, our case emphasizes the consideration of possible squamous cell carcinoma in such patients and the importance of appropriate preventive measures.

Acquired epidermodysplasia verruciformis (AEV) in three children after cardiac transplantation: A case series and review of the literature.

Acquired epidermodysplasia verruciformis (AEV) describes epidermodysplasia verruciformis developing in an immunocompromised host. There is limited information in the literature regarding AEV in the pediatric population; of the patients reported, most patients described had HIV, with only two reported cases of children who developed AEV post-transplantation. This case series describes three pediatric patients who developed AEV on immunosuppressant therapy following cardiac transplantation. We review risk factors, treatment options, and prognosis of AEV in the pediatric population.

Human Papillomaviruses and Skin Cancer.

Human papillomaviruses (HPVs) infect squamous epithelia and can induce hyperproliferative lesions. More than 220 different HPV types have been characterized and classified into five different genera. While mucosal high-risk HPVs have a well-established causal role in anogenital carcinogenesis, the biology of cutaneous HPVs is less well understood.From patients with the rare genetic disorder epidermodysplasia verruciformis (EV) and animal models, evidence is accumulating that cutaneous PV of genus ß synergize with ultraviolet (UV) radiation in the development of cutaneous squamous cell carcinoma (cSCC). In 2009, the International Agency for Research on Cancer (IARC) classified the genus ß-HPV types 5 and 8 as "possible carcinogenic" biological agents (group 2B) in EV disease. Epidemiological and biological studies indicate that genus ß-PV infection may also play a role in UV-mediated skin carcinogenesis in non-EV patients. However, they rather act at early stages of carcinogenesis and become dispensable for the maintenance of the malignant phenotype, compatible with a "hit-and-run" mechanism.This chapter will give an overview on genus ß-PV infections and discuss similarities and differences of cutaneous and genus α mucosal high-risk HPV in epithelial carcinogenesis.

A novel approach to the classification of epidermodysplasia verruciformis.

BACKGROUND: Epidermodysplasia verruciformis (EV) is a rare genodermatosis that causes disseminated eruptions of hypo- or hyperpigmented macules and wart-like papules that can coalesce and scale. It is uniquely characterized by an increased susceptibility to specific human papillomavirus (HPV) genotypes. Classically, EV is associated with mutations of the EVER1/TMC6 and EVER2/TMC8 genes. The term "acquired" epidermodysplasia verruciformis was coined to describe an EV-like syndrome that can develop in patients with a compromised immune system. Recent discoveries of other genes implicated in EV, including RHOH, MST-1, and CORO1A, have complicated the classification of EV and EV-like syndromes. METHODS: We review the available data on epidermodysplasia verruciformis in the literature in order to propose a new classification system to encompass current and future developments on EV and EV-like syndromes. RESULTS: We propose classifying EV into: (1) classic genetic EV, (2) non-classic genetic EV, and (3) acquired EV. CONCLUSION: The proposed categorization scheme provides a simple and logical way to organize the different cases of EV that have been described in the literature. This system organizes EV by its cause, allowing for a better understanding of the disease and helps differentiate EV from other causes of generalized verrucosis.

The biology of beta human papillomaviruses.

The beta genus comprises more than 50 beta human papillomavirus (HPV) types that are suspected to be involved, together with ultraviolet (UV) irradiation, in the development of non-melanoma skin cancer (NMSC), the most common form of human cancer. Two members of the genus beta, HPV5 and HPV8, were first identified in patients with a genetic disorder, epidermodysplasia verruciformis (EV), that confers high susceptibility to beta HPV infection and NMSC development. The fact that organ transplant recipients (OTRs) with an impaired immune system have an elevated risk of NMSC raised the hypothesis that beta HPV types may also be involved in skin carcinogenesis in non-EV patients. Epidemiological studies have shown that serological and viral DNA markers are weakly, but significantly, associated with history of NMSC in OTRs and the general population. Functional studies on mucosal high-risk (HR) HPV types have clearly demonstrated that the products of two early genes, E6 and E7, are the main viral oncoproteins, which are able to deregulate events closely linked to transformation, such as cell cycle progression and apoptosis. Studies on a small number of beta HPV types have shown that their E6 and E7 oncoproteins also have the ability to interfere with the regulation of key pathways/events associated with cellular transformation. However, the initial functional data indicate that the molecular mechanisms leading to cellular transformation are different from those of mucosal HR HPV types. Beta HPV types may act only at early stages of carcinogenesis, by potentiating the deleterious effects of other carcinogens, such as UV radiation.

Acquired epidermodysplasia verruciformis in an HIV-positive patient.

Epidermodysplasia verruciformis (EV) is a rare dermatological manifestation of the human papillomavirus (HPV) infection, which causes distinctive skin lesions in sun-exposed areas. Both inherited and acquired forms exist. Immunocompromised individuals, such as HIV patients, are at risk of acquired EV. EV poses challenges in its management and variable responses are seen in different individuals. In addition, EV carries a significant risk of skin malignancy with certain HPV types that require skin surveillance. A case of acquired EV in a HIV-positive patient is presented in this report.

Deleterious Effect of Radiation Therapy on Epidermodysplasia Verruciformis Patients.

BACKGROUND: Epidermodysplasia verruciformis (EV) is a rare genodermatosis caused by specific human papillomavirus (HPV) types associated with the development of multiple squamous cell carcinomas (SCC). The treatment for this skin tumour may be difficult. Among the therapy options, radiotherapy (RT) should be avoided due to its deleterious effects on HPV-induced carcinogenesis. OBJECTIVE: To describe 4 patients with EV who underwent radiotherapy to treat cutaneous SCC. METHODS: This is a retrospective study. The evolution of cutaneous SCC after adjuvant radiotherapy in 4 patients with EV was observed. RESULTS: This study included 4 patients with diagnosis of EV. All 4 of the patients had cutaneous SCC. They underwent surgical resection and adjuvant radiotherapy. Over a period of up to 2 years, there was aggressive tumour recurrence. CONCLUSION: Radiotherapy might be associated with progression of SCC in patients with EV, and it is recommended that radiotherapy should be avoided in this patient population.