Epidermodysplasia Verruciformis

Epidermodysplasia Verruciformis

About Epidermodysplasia Verruciformis

Epidermodysplasia Verruciformis (also called Lewandowsky-Lutz dysplasia or Lutz-Lewandowsky epidermodysplasia verruciformis) is an extremely rare autosomal recessive genetic hereditary skin disorder associated with a high risk of carcinoma of the skin. It is characterized by abnormal susceptibility to human papillomaviruses (HPVs) of the skin. The resulting uncontrolled HPV infections result in the growth of scaly macules and papules, particularly on the hands and feet. It is typically associated with HPV types 5 and 8, which are found in about 80% of the normal population as asymptomatic infections, although other types may also contribute. The condition usually has an onset of between the ages of 1_20, but can occasionally present in middle-age. It is named after the physicians who first documented it, Felix Lewandowsky and Wilhelm Lutz. The cause of the condition is an inactivating HP mutation in either the EVER1 or EVER2 genes, which are located adjacent to one another on chromosome 17. The precise function of these genes is not yet fully understood, but they play a role in regulating the distribution of zinc in the cell nucleus. Zinc is a necessary cofactor for many viral protein.

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