All about epidermodysplasia verruciformis (EV): Aninherited skin disorder

Epidermodysplasia verruciformis (EV) is an uncommon genetic skin condition characterized by a high vulnerability to specific types of human papillomavirus (HPV), increasing the likelihood of non-melanoma skin cancer. The disorder is inherited in an autosomal recessive pattern, and certain families exhibit mutations in the TMC۶ or TMC۸ genes situated on chromosome ۱۷q۲۵.۳. During childhood, individuals with EV frequently develop flat-topped, papular lesions that resemble verrucae planae on their limbs, reddish-brown plaques, or lesions resembling pityriasis versicolor on their neck, trunk, and face. In addition, more than half of patients may experience actinic keratoses and cutaneous malignancies during their twenties or thirties. Diagnosis of EV involves a skin biopsy to examine histological characteristics and detect HPV presence in a skin lesion. Additionally, there is an acquired form of EV observed in individuals with weakened immune systems. Although no definitive treatments exist for EV, patients are advised to rigorously practice sun protection measures to reduce the risk of developing non-melanoma skin cancer. Regular skin examinations throughout a patient›s lifetime are also recommended to detect precancerous skin lesions and early-stage cancer.Epidermodysplasia verruciformis (EV) is an uncommon genetic skin condition characterized by a high vulnerability to specific types of human papillomavirus (HPV), increasing the likelihood of non-melanoma skin cancer. The disorder is inherited in an autosomal recessive pattern, and certain families exhibit mutations in the TMC۶ or TMC۸ genes situated on chromosome ۱۷q۲۵.۳. During childhood, individuals with EV frequently develop flat-topped, papular lesions that resemble verrucae planae on their limbs, reddish-brown plaques, or lesions resembling pityriasis versicolor on their neck, trunk, and face. In addition, more than half of patients may experience actinic keratoses and cutaneous malignancies during their twenties or thirties. Diagnosis of EV involves a skin biopsy to examine histological characteristics and detect HPV presence in a skin lesion. Additionally, there is an acquired form of EV observed in individuals with weakened immune systems. Although no definitive treatments exist for EV, patients are advised to rigorously practice sun protection measures to reduce the risk of developing non-melanoma skin cancer. Regular skin examinations throughout a patient›s lifetime are also recommended to detect precancerous skin lesions and early-stage cancer.

کلیدواژه ها:

Epidermodysplasia verruciformis ، Human papillomavirus ، Skin disorder