EVIDERMODYSPLASIAVERRUCIFORMIS
Anonymous
321 x views • 6 years ago
Background
Epidermodysplasia verruciformis (EV) is a rare, inherited disorder that predisposes patients to widespread human papillomavirus (HPV) infection and cutaneous squamous cell carcinomas. [1, 2, 3, 4] Although epidermodysplasia verruciformis is most commonly inherited in an autosomal recessive manner, [5] sporadic, sex-linked, and autosomal dominant inheritance have been described. In those cases of autosomal recessive inherence, there may be a history of consanguinity in the parents of the afflicted individual. In those cases of atypical inheritance, there may be an association with chronic lymphopenias. Regardless of the mode of inheritance, the phenotype of the disease is characterized by chronic infection with HPV. Widespread skin eruptions of flat-to-papillomatous, wartlike lesions and reddish-brown pigmented plaques on the trunk, the hands, the upper and lower extremities, and the face are typical.
Malignant skin tumors (carcinomas), especially squamous cell carcinoma (in situ or invasive), develop frequently in these patients (30–70%), most commonly in sun-exposed areas starting between the ages of 20 and 40 years, which reflects the high-risk nature of the HPV infection. Skin cancers initially appear on sun-exposed areas, such as the face, neck, chest, and arms, reflecting the role of ultraviolet light and HPV infection in the promotion of skin cancer development. Patients with epidermodysplasia verruciformis are usually infected with multiple types of HPV, including common types that affect individuals without epidermodysplasia verruciformis (eg, HPV types 3 and 10) and those unique to epidermodysplasia verruciformis, the so called epidermodysplasia verruciformis–associated HPVs (EV-HPVs).
Epidermodysplasia verruciformis (EV) is a rare, inherited disorder that predisposes patients to widespread human papillomavirus (HPV) infection and cutaneous squamous cell carcinomas. [1, 2, 3, 4] Although epidermodysplasia verruciformis is most commonly inherited in an autosomal recessive manner, [5] sporadic, sex-linked, and autosomal dominant inheritance have been described. In those cases of autosomal recessive inherence, there may be a history of consanguinity in the parents of the afflicted individual. In those cases of atypical inheritance, there may be an association with chronic lymphopenias. Regardless of the mode of inheritance, the phenotype of the disease is characterized by chronic infection with HPV. Widespread skin eruptions of flat-to-papillomatous, wartlike lesions and reddish-brown pigmented plaques on the trunk, the hands, the upper and lower extremities, and the face are typical.
Malignant skin tumors (carcinomas), especially squamous cell carcinoma (in situ or invasive), develop frequently in these patients (30–70%), most commonly in sun-exposed areas starting between the ages of 20 and 40 years, which reflects the high-risk nature of the HPV infection. Skin cancers initially appear on sun-exposed areas, such as the face, neck, chest, and arms, reflecting the role of ultraviolet light and HPV infection in the promotion of skin cancer development. Patients with epidermodysplasia verruciformis are usually infected with multiple types of HPV, including common types that affect individuals without epidermodysplasia verruciformis (eg, HPV types 3 and 10) and those unique to epidermodysplasia verruciformis, the so called epidermodysplasia verruciformis–associated HPVs (EV-HPVs).
