What is epidermolysis bullosa?
In patients with epidermolysis bullosa, large blisters containing clear fluid appear on the skin. These blisters can be found on the feet, hands, and sometimes on mucous membranes (mouth, genital area). When the blisters burst, they leave the skin intact, which is difficult to heal.
There are several forms of epidermolysis bullosa:
– Epidermolysis bullosa simplex, where the epidermis (the top surface layer of the skin) peels off. This is the most common form.
– Junctional epidermolysis bullosa , where the dermis and epidermis separate.
– Dystrophic epidermolysis bullosa , where the dermis (the deepest layer) separates. This is the least common form.
In the most severe forms of epidermolysis bullosa (junctional and dystrophic), blisters may appear all over the body. The skin peels off, leaving one or more lesions (called “erosions”), most of which form scabs.
When the skin surface is lost, the risk of infection is also greater. In addition, bacteria grow and transform blisters, forming pus, drainage and redness around the wound.
Reason
Epidermolysis bullosa is a rare genetic condition. It is caused by a defect in certain genes that produce proteins that keep the different layers of the skin together. The first symptoms appear at birth or in early childhood.
Therefore, the epidermis is poorly adherent to the dermis, a small impact or very light friction (clothes, diapers, shoes, gloves, etc.) is enough to form blisters on the skin surface. However, blisters can also appear spontaneously on the body.
How to treat patients with epidermolysis bullosa?
Unfortunately, there is no cure for epidermolysis bullosa. Therefore, only general treatment measures can be given to relieve pain and minimize complications for the patient.
Daily care in a highly sterile environment (usually in a hospital), treatment with non-adhesive dressings and Vaseline if available. Covering with sterile dressings secured with appropriate dressings to aid healing and mobility (especially in the joints).
After that, the bandage should be changed daily or every 2 to 3 days when bathing, which is also the time to remove the bandage on the wound.
In junctional and dystrophic cases, healing may be prolonged and the lesion may develop into a chronic wound (LINK), which will require specific treatment.
Advice
Epidermolysis bullosa varies considerably from patient to patient and from form to form. Therefore, regular consultation with medical professionals is necessary.
Ultimately, these precautions will help you, as a patient or parent of a patient, better understand the consequences of epidermolysis bullosa on your family’s daily life:
- Keep the living environment cool, as high temperatures increase the risk of damage.
- Avoid getting anything on your skin and use only non-stick bandages.
- Choose soft mattresses, sheets and towels. Secure the area around the child’s bed to avoid shock.
- Prefer loose cotton clothing, without labels or plastics.
Urgo’s Commitment to Epidermolysis Bullosa
Since its establishment, the URGO Foundation
supports projects aimed at improving the quality of life of patients with epidermolysis bullosa and the quality of their treatment. Urgo also supports DEBRA [EXTERNAL LINK], the global network for the disease.
Working in countries with the highest incidence of the disease and where treatment is scarce or even non-existent, the URGO foundation supports initiatives to treat this disease, of which there is still little known:
- Organize nursing training with the help of experts.
- Assist family in wound treatment and care instructions.
- Fund equipment to improve patient care.
To learn more about this disease and support research: DEBRA logo with link to their website
