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Epidermodysplasia Verruciformis : Clinical

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Clinical Features: The clinical manifestations of epidermodysplasia verruciformis (EV) become apparent during infancy or childhood in almost 70% of cases and by puberty in the remainder. Patients develop hypo- or hyperpigmented, scaly, macular plane warts on trunk, face, neck, arms, dorsal hands and feet (sun-exposed areas). There are no mucosal lesions.

Some cases resemble pityriasis versicolor or seborrheic keratosis. Uncontrolled HPV infection can result in scaly papules and macules on hands and feet resembling tree bark, hence the name treeman syndrome, which has been sometimes used to refer to this condition.

About 30-60% of patients develop non-melanoma skin cancers on sun-exposed areas by 4th or 5th decade of life. Most are squamous cell carcinomas that remain localized. Metastases are rare. Rare cases of sweat gland carcinoma and merkel cell carcinoma have been reported in EV.

The image shows higher magnification of the previous image. The keratinocytes in the upper epidermis have enlarged nuclei and voluminous cytoplasm with bluish-gray pallor. The papular lesions were present over a tatoo in a HIV-positive individual.

Image courtesy of: Carles Saus, MD; Hospital Son Espases, Mallorca, Spain.

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