Epidermodysplasia verruciformis (EV) is usually suspected with the appearance of generalized wart-like lesions early in life. A major concern for those affected by this condition is that the lesions may transform into skin cancer. This article provides information on the causes, symptoms, and treatment of this skin condition.
Epidermodysplasia verruciformis, also known as Lewandowsky-Lutz dysplasia or Lutz-Lewandowsky, is a skin condition named after the famous doctors Felix Lewandowsky and Wilhelm Lutz. This is an uncommon skin condition that causes increased susceptibility to several distinct human papillomavirus (HPV). 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 (skin cancer).
Causes
It is a genetic disorder, which means that 2 abnormal EV genes (one from each parent) are needed to have this disease. It has been found that 10% of the EV patients, come from marriages between blood relatives (i.e., the parents share a common ancestor). The condition is characterized by increased susceptibility to various HPV infections of the skin. These uncontrolled HPV infections are followed by the growth of scaly macules or papules, all over the face, hands, and feet. The skin of the people affected by this condition resembles the bark of trees due to the growth of warts on the hands and feet. 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. Scientists are still conducting research to find the exact function of these genes. However, as of now, 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 co-factor 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.
Symptoms
There are several symptoms associated with this skin disease, which range from mild to severe. This condition is normally associated with HPV type 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 HPV skin infections
Diagnosis
This condition generally begins in infancy or childhood, and is very difficult to cure. The disease may affect both males and females, and people of all races. It can be of two different forms:
Type 1:
This type is characterized by multiple plane warts or plaques, that are persistent and widely distributed all over the body. Tendency for malignant transformation is not observed in this type.
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. In some studies, it was observed 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.
Treatment
Scientists are still carrying out research to find an effective treatment method against EV. However, several treatment methods have shown satisfactory results in dealing with this condition. Some of the medications that have proved to be effective include acitretin, interferons, and retinoids.
The skin lesions should be assessed regularly and removed in case of malignancy. Although there is no specific treatment for this condition, early diagnosis and treatment can prevent the progression of this condition into malignant tumors.
Disclaimer: This HealthHearty article is for informative purposes only, and should not be used as a replacement for expert medical advice.
