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Epidermodysplasia verruciformis | |
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Other names | Lewandowsky–Lutz dysplasia, treeman syndrome |
Abul Bajandar from Bangladesh | |
Specialty | Dermatology |
Causes | Genetic |
Treatment | Surgery |
Epidermodysplasia verruciformis (EV) is a skin condition characterised by warty skin lesions.[1] It results from an abnormal susceptibility to HPV infection (HPV) and is associated with a high lifetime risk of squamous cell carcinomas in skin.[1] It generally presents with scaly spots and small bumps particularly on the hands, feet, face and neck; typically beginning in childhood or in a young adult.[1] The bumps tend to be flat, grow in number and then merge to form plaques.[1] On the trunk, it typically appears like pityriasis versicolor; lesions there being slightly scaly and tan, brown, red or looking pale.[1] On the elbows, it may appear like psoriasis.[1] On the forehead, neck and trunk, the lesions may appear like seborrheic keratosis.[1]
It is most frequently inherited as an autosomal recessive trait, with some reports of autosomal dominant and X-linked inheritance.[1][2] Other types include atypical EV which develops due to gene mutations that cause an impaired immune system, and acquired EV which occurs due to acquired immunodeficiency.[2][3] It is characterized by an inability to protect against HPV infection of skin.[4][5] HPV types 5 and 8 are detected in around 90% of skin cancers in people with EV.[1] Other types are also associated with EV.[1] In rare cases, the warts may develop into giant horns resulting in treeman syndrome.[6]
Prevention of skin cancer requires sun protection.[1] Treatment typically involves surgery; sometimes with the addition of skin grafting.[1] Medications used to treat the lesions include ALA-PDT (photodynamic therapy with aminolevulinic acid), applying 5-FU, imiquimod, and retinoids by mouth.[1] The lesions tend to recur on stopping treatment.[1]
The condition is rare.[1] The lesions have been noted to occur at a younger age in warmer climates.[1] EV associated skin cancer develops less frequently in Africans.[1] The condition was first described by Felix Lewandowsky and Wilhelm Lutz in 1922.[7]