Epidermodysplasia Verruciformis

Epidermodysplasia verruciformis, also known as Lewandowsky-Lutz dysplasia or Lutz-Lewandowsky This is an uncommon skin condition, that causes increased susceptibility to several distinct human papillomaviruses. It is named after the famous doctors Felix Lewandowsky and Wilhelm Lutz. It is a genetic skin disorder, associated with the autosomal recessive genes. People with this condition are at a high risk of developing carcinoma of the skin.

Epidermodysplasia Verruciformis Causes
Epidermodysplasia verruciformis is a genetic disorder, which means, it is the result of mutation in some gene. The condition is characterized by increased susceptibility to various human papillomaviruses (HPV) of the skin. These uncontrolled HPV infections are followed by growth of scaly macules or papules, all over the face, hands and feet. The people suffering from this genetic skin disorder, with the growth of warts on the hands and feet, resemble bark of trees. It is believed that this condition arises as a result of inactivating mutation in the EVER1 or EVER2 genes. These genes lie adjacent to one another and are present on the chromosome 17. Research is still going on to find the exact function of these genes, however, it is known that these genes play a role in the regulation and distribution of zinc in the nucleus of the cells. Zinc is a vital cofactor for many viral proteins and any change in the normal activity of EVER1 or EVER2 genes can restrict the access of proteins to the cellular zinc stores. This can, in turn, limit their normal growth.

Epidermodysplasia Verruciformis Symptoms
There are several signs and symptoms associated with this skin disease, which range from mild to severe. This condition is normally associated with HPV types 5 and 8, which are generally asymptomatic infections. However, there may be several other types which cause specific symptoms.

• Warts all over the body
• Flat red or brown macules
• Skin bumps on hands and feet
• Flattened warty lesions
• High susceptibility to human papillomaviruses of the skin

Epidermodysplasia Verruciformis Diagnosis
This condition generally begins in infancy or childhood and is very difficult to cure. It can be of two different forms:

Type 1:
Type 1 is characterized by multiple plane warts or plaques, that are persistent and widely distributed all over the body. This type has no tendency to malignant transformation.

Type 2:
People with this form of inherited skin problem have plane warts or reddish brown patches or lesions that resemble scaling pityriasis versicolor or seborrheic keratosis. Studies show that this form was malignant in about 25% of the patients and resulted in invasive squamous cell carcinoma. However, it has a very low rate of metastasis.

Unlike the benign form, that shows only flat wart like lesions over the body, type 2 or the malignant form, shows a higher rate of polymorphic skin lesions and development of multiple cutaneous tumors. These cutaneous tumors can be distributed all over the body or can be limited to one extremity.

Epidermodysplasia Verruciformis Treatment
Research is still going on to find an effective treatment method against epidermodysplasia verruciformis. However, several treatment methods have shown satisfactory results in dealing with this condition. Some of the medications such as acitretin, interferons and retinoids have proved effective.

The skin lesions should be assessed regularly and removed in case of malignancy. Although there is no specific treatment for this condition, early diagnosis, can prevent development of malignant tumors.