When Epidermodysplasia verruciformis is a disease which is present in the patients from birth to the skin. In the context of epidermodysplasia verruciformis, a so-called generalized verrucosis develops in a very extreme form. Epidermodysplasia verruciformis is extremely rare and increases the risk of developing skin cancer. In addition, the skin of those affected is particularly sensitive to certain human papilloma viruses.
What is epidermodysplasia verruciformis?
According to POLYHOBBIES, epidermodysplasia verruciformis is synonymous with Lutz-Lewandowsky Epidermodysplasia verruciformis. The disease is usually inherited via an autosomal recessive route. Epidermodysplasia verruciformis occurs very rarely and is one of the so-called genodermatoses, i.e. congenital diseases of the skin. Patients are unusually susceptible to HP virus infections.
As a result of these infections with the virus, spots, scales and papules appear on the skin. These skin changes primarily affect the patient’s hands and feet. The anomalies grow in an uncontrolled manner and, depending on the individual case, lead to conspicuous phenomena in the corresponding parts of the body.
In the majority of cases, epidermodysplasia verruciformis manifests itself for the first time between the ages of one and twenty. However, in some cases, the disease does not appear in adults until mid-life. The synonymous name of the disease refers to the two physicians who scientifically described the disease for the first time. These are Lutz and Lewandowsky.
Causes
Epidermodysplasia verruciformis is a genetic skin disease that is present in those affected from birth. Genetic defects in two genes called EVER1 and EVER2, which are located in the area of the 17th chromosome, lead to the development of epidermodysplasia verruciformis. Due to the genetic defect, the patient’s skin is extremely susceptible to infections with HP viruses.
The genetic defect usually arises as a result of a mutation. The genes concerned are involved in controlling the substance zinc in the nucleus of cells. Various studies show that zinc plays an important role in viral proteins. Type V and VIII HP viruses pose a particular risk to the affected patients.
These viruses are present in around 80 percent of people, but they usually do not lead to any comparable symptoms in healthy people. Other types of human papillomavirus can also trigger epidermodysplasia verruciformis.
Symptoms, ailments & signs
Epidermodysplasia verruciformis manifests itself in individual cases with very different appearances on the skin. Scaly patches of skin, warts and papules are typical of Epidermodysplasia verruciformis. The anomalies primarily occur in the hands, feet, torso, and face area. Sometimes the skin changes appear as reddish to brownish spots.
In more severe cases, excesses develop from the scaly and wart-like areas of the skin. In extreme cases, this significantly limits the functions of the hands and feet. Reddish or pink papules often develop in the limb area.
Diagnosis
The diagnosis of epidermodysplasia verruciformis is usually made by a specialized dermatologist. The rarity of the disease sometimes means that the diagnosis is relatively tedious. The patient describes the individual clinical picture to the doctor and explains the time of the first manifestation of the disease. A family history is usually also done.
In this way, the doctor receives important information regarding potential comparable cases in the patient’s family. This quickly narrows down the spectrum of possible diseases and speeds up diagnosis. As part of the clinical examination, the doctor first inspects the affected skin area during visual examinations.
The typical papules, spots and warts, and possibly skin carcinomas, indicate epidermodysplasia verruciformis. Sometimes the changes in the skin show up on the entire surface of the body. In many patients, however, they are limited to certain areas, such as the limbs.
The dermatologist determines whether the epidermodysplasia verruciformis is benign or malignant. In the benign form, the patients only have benign skin changes such as warts or papules. In contrast, various types of skin cancer can be found in the malignant variant of Epidermodysplasia verruciformis.
Complications
Epidermodysplasia verruciformis can lead to various complaints and complications. The patient has an increased risk of skin cancer and in the worst case can die from it. As a rule, however, this case rarely occurs, whereby the patient must avoid direct sunlight and always protect his skin.
This can lead to restrictions in everyday life. The skin is also particularly sensitive to certain viruses and can react with rashes, warts or papules. These can not only lead to pain, but can also be visually negative, so that the patient suffers from inferiority complexes and a reduced self-esteem.
Often social contacts are avoided, which can also manifest itself in psychological problems. The person concerned also has to be examined more often for skin cancer. If this occurs, it can be surgically removed and there are no further complaints or complications.
A causal treatment and cure for Epidermodysplasia verruciformis is not possible, however, so that the person affected will have to rely on skin cancer screenings and strong sun protection for the rest of their lives. Life expectancy is not reduced if there are no tumors or if they are removed in good time.
When should you go to the doctor?
If the skin of a newborn child shows any abnormalities, these should be examined by a doctor as early as the first few days of life. Scalings on the face, hands, feet or trunk are considered unusual and should be examined medically.
In the event of swelling of the skin or the formation of poplars or warts, a doctor’s visit is necessary. If the discomfort spreads or if it intensifies, it is advisable to consult a doctor. If there is discoloration of the skin or abnormal sensations in the perception of temperature and touch, a doctor should be consulted.
If the use of creams, cosmetics or ointments leads to intolerance reactions, a doctor must also be consulted. In order to avoid recurring skin reactions, it should be found out which active ingredients have caused the hypersensitivity. If the functionality of the hands or feet is impaired due to the symptoms, a doctor is required.
Bad posture and damage to the skeletal system can occur, which must be corrected and treated. If emotional problems arise due to the skin abnormalities, it is advisable to seek the help of a doctor or therapist. It is advisable to consult a doctor in the event of a depressed mood, withdrawal behavior, aggression or feelings of inferiority.
Treatment & Therapy
At the present time, effective measures for the treatment of epidermodysplasia verruciformis have not yet been sufficiently developed. A long-term cure for the disease is currently not practical. Sometimes the patient is treated with medication, with the doctor usually prescribing interferons or the active ingredient acitretin. If tumor-like changes develop on the skin, these are usually removed surgically. In this way, malignant carcinomas of the skin are prevented.
It should be noted that people with epidermodysplasia verruciformis have an increased risk of squamous cell carcinoma. Accordingly, regular check-ups by the doctor in connection with skin cancer screenings are necessary. This enables timely intervention in the event of malignant changes in the skin.
Outlook & forecast
The prognosis of epidermodysplasia verruciformis is described as unfavorable. The genetic disease cannot be cured due to the current legal situation. A change in genetics is not permitted according to the current legal requirements. Therefore symptomatic therapy takes place, the effectiveness of which must be assessed individually.
In some patients, drugs can be found that are well accepted by the organism. The complexion of the skin improves and the symptoms are minimized overall. However, there is no prospect of a cure. As soon as the medication is discontinued, a worsening and regression of the symptoms can be expected.
Normally, the existing active ingredients in the medication alone do not provide adequate relief. In many cases, abnormalities or unpleasant areas of the skin must be removed by surgical intervention. The usual risks and side effects must be taken into account. In addition, the skin changes can return at any time despite an extension.
In the case of a severe course of the disease, malignant developments in the complexion of the skin occur. Carcinomas develop that need to be treated as quickly as possible. If there are delays in treatment or if the skin cancer goes unnoticed, it can be fatal. In addition to the physical abnormalities, psychological problems can also arise. This further worsens the overall prognosis of the person concerned.
Prevention
Epidermodysplasia verruciformis is a skin disease that is based on a genetic defect and is congenital. Medical research is currently not so advanced as to prevent hereditary diseases such as epidermodysplasia verruciformis. Early diagnosis is therefore all the more important.
Aftercare
In most cases, the person affected with epidermodysplasia verruciformis has no special follow-up options available. For this reason, those affected are primarily dependent on a quick and, above all, early diagnosis of the disease in order to prevent further complications or complaints. It cannot be predicted whether the epidermodysplasia verruciformis will also reduce the life expectancy of the patient or otherwise limit it.
In general, however, early detection of the disease always has a positive effect on the further course. In most cases, patients with this disease are dependent on taking medication to relieve the symptoms. The doctor’s instructions should always be followed. If there are any doubts or ambiguities, a doctor should always be contacted first.
It is not uncommon for surgical interventions to be necessary to remove the tumors. After such interventions, the person concerned should always rest and take care of his body. Refrain from exertion or other stressful activities. Even after the tumor has been successfully removed, regular examinations should be carried out on the body. A complete cure of the epidermodysplasia verruciformis is not possible in many cases.
You can do that yourself
Since epidermodysplasia verruciformis increases the risk of skin cancer, it is advisable for the patient to take special protection against the effects of UV radiation. Direct sunlight should be avoided in everyday life.
The body should be covered as completely as possible with clothing or accessories such as caps and hats. Visible areas of the skin should be fully protected by applying sunscreen. Care should be taken to use a high sun protection factor, which should not be below a value of 20. A visit to a solarium should be avoided, as the risk of skin cancer also increases due to the influence of artificial radiation.
The epidermodysplasia verruciformis patient suffers from a high susceptibility to HP viruses. For this reason, comprehensive protection against viruses is highly recommended in everyday life. Unprotected intercourse should be avoided. In addition to using a condom, it is advisable to avoid frequent partner changes. Since the viruses are transmitted through skin contact, it is advisable to avoid contact with strangers and also to use disinfectants regularly.
If possible, visits to public toilets should be avoided. Protective gloves can be worn in public facilities or in areas where there is an increased risk of infection. In swimming pools, it is advisable to avoid going barefoot.
