Epidermodysplasia Verruciformis (EV)

Epidermodysplasia Verruciformis is a rare autosomal recessive inherited skin disorder that is characterized by eruptions of wart-like lesions that may occur anywhere on the body.  A common name usually heard with this disease is tree man disorder because the symptoms on the body appear as bark-like lesions.  The lesions are caused by an infection with the human papillomavirus.  There are over 70 different types of HPV that are responsible for causing a wide range of viral warts. It has been found that patients with EV have an abnormal or impaired immune response to HPV or wart viruses. A major concern for patients with EV is that the wart-like lesions may transform into skin cancers.

EV is an autosomal recessive inherited disorder, which means that 2 abnormal EV genes (one from each parent) are needed to have this disease.  Ten percent of the EV cases comes from marriages between blood relatives (the patents share a common ancestor).  In few cases, sex-linked and autosomal dominant inheritance patterns have also been found.

The warts usually start to develop sometime during childhood.  Only 7.5% of cases appear in infancy while 22.5% appear in puberty.  The disease affects both males and females and people of all races. The lesions have been described as flat, wart -like lesions that look like plane warts.  Flat wart lesions also commonly appear on sun-exposed areas such as the hands, feet, face, and ear lobes. Plaque-like lesions usually appear on the trunk, neck, arms, and legs. The palms, soles, armpits and external fenitals may also be involved.  In most cases the warts occur in clusters and can be anything from a few warts to over a hundred.

EV is a lifelong disease, even though the lesions can be treated or removed as they appear, patients will still continue to develop the lesions throughout life. In many cases lesions may develop and remain unchanged for years.  There is no current treatment to prevent new EV lesions from occurring. The management of EV involves a combination of medical and surgical treatments alongside patient counseling and education.