title: Clinical aspects of epidermodysplasia verruciformis.
abstract: Thirteen patients with epidermodysplasia verruciformis (EV) were studied over a period of 7 years. EV is a rare genodermatosis characterized by a generalized infection with a specific group of human papilloma virus (HPV) and a propensity for developing skin malignant tumours in 30%-50% of patients. The diagnosis of EV was confirmed by histopathological and immunohistochemical findings. Three of our patients had the benign form of EV, which is characterized by monomorphous lesions and no malignant changes, whereas 10 had the malignant form, which is characterized by polymorphic lesions and development of cutaneous malignant tumours. All EV patients with the malignant form developed multiple skin tumours (77%). They started to appear at age 20, predominantly on the forehead (50%). Most were squamous cell carcinoma, extremely aggressive and invasive, which provoked metastasis and death in two patients.
authors.affiliation: Department of Dermatology, University of São Paulo, São Paulo, Brazil. walmarroncalli@uol.com.br
authors.forename: W R P
authors.initials: WR
authors.lastname: de Oliveira
authors.forename: C
authors.initials: C
authors.lastname: Festa Neto
authors.forename: P L
authors.initials: PL
authors.lastname: Rady
authors.forename: S K
authors.initials: SK
authors.lastname: Tyring
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D001707:Biopsy, Needle; D002471:Cell Transformation, Neoplastic / Q000473:pathology*; D004279:DNA, Viral / Q000032:analysis; D018450:Disease Progression; D004819:Epidermodysplasia Verruciformis / Q000453:epidemiology / Q000473:pathology* / Q000821:virology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D008297:Male; D027383:Papillomaviridae / Q000302:isolation & purification*; D011230:Precancerous Conditions / Q000473:pathology*; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D012720:Severity of Illness Index; D017678:Sex Distribution; D013997:Time Factors
chemical_list: D004279:DNA, Viral