Epidermolysis bullosa

Caring for and preventing infections in wounds are among the most critical tasks when living with EB, alongside ongoing skin care and monitoring. Below is a step-by-step guide for wound care. 

To begin wound care, it is advisable to bathe the baby (this advice applies to children, adolescents, and adults with EB as well). Bathing helps prevent infections and, by moistening the dressings, minimizes pain and injury during removal. It is recommended to bathe and care for the skin every 2-3 days to support the natural healing process. However, this frequency should be adjusted based on individual circumstances, such as the type of dressing used, the presence of infections in the wounds, and seasonal climate variations. 

Place of the cure 

It is important to have a designated area for wound care, ensuring adequate temperature and lighting while adhering to basic hygiene rules. All materials should be organized, and it’s helpful to have a small dressing kit with travel supplies for when you need to move. The essential wound care materials include: 

• Sterile gauzes in two sizes 

• Soft bandages (cotton bandages and tubular support bandages) 

• Non-adherent dressings 

• Scissors 

• Tweezers 

• Sterile needles 

• Physiological saline or tepid water 

• Disinfectants (avoid povidone iodine and chlorhexidine; use polyhexanide-based cleaning solutions for more contaminated wounds) 

Additionally, it is advisable to have a designated bin for the safe disposal of needles and sharp objects. 

Before bathing and caring for the child, it is essential to wash your hands thoroughly. While the use of gloves is not mandatory in all cases, proper handwashing between procedures is crucial. It’s important to clearly separate clean and dirty zones during wound care. 

Removing bandages and dressings 

To begin the wound care process, carefully remove the clothing, bandages, and dressings. As previously mentioned, it’s advisable to do this inside the bathtub or while wet, especially for gauzes and dressings that are more adhered to the skin. If a bathtub or shower is unavailable, dampening the gauzes and dressings with physiological saline or water, or using adhesive removal products (available in sprays or wipes), can also be effective, though it may extend the process.  

Bath 

The bath can be done fully or in parts. During the bath, use a soap-free bath gel formulated for sensitive skin, following the recommendations of your dermatologist or specialist nurse. In some cases, diluted salt in water and/or disinfectant products may be recommended, but this should always be under medical guidance. The gel is typically diluted in the bathtub water and applied gently with your hands or gauzes, using light touches to clean the wounds without rubbing. 

To prevent injuries from the baby’s kicking, protect the bottom of the bathtub. Inflatable tubs can also be useful, as they can be cleaned thoroughly afterward. If you cannot regulate the water pressure, consider covering the tap with gauze. It is advisable not to extend the bath immersion for more than 20 minutes. 

Drying 

After finishing the bath, the baby must be held with soft towels and/or gauzes and the drying should be done softly and by touches. It is important to protect them from the cold, drying with special care the head area and the cutaneal folds (neck, groin, buttocks, etc.). Once dry, the wounds must be treated individually, hydrating the healthy skin and covering the body as fast as possible to avoid the appearance of new injuries. 

Wound care 

It is not recommended to routinely use antiseptics on wounds, as they may interfere with the healing process. After drying the area, we should inspect the skin for any new blisters or wounds. If small blisters appear, they should be punctured early to prevent them from growing larger over time. 

• Debridement involves removing dead tissue, such as tough scabs, skin pelts, dry exudate, and devitalized tissue from broken blisters. When done correctly, this procedure promotes healing and reduces bacteria in the dead tissue. It’s advisable to be gentle during debridement, but using disinfectant products and specific debridement techniques can be helpful. Applying vaseline to tough scabs can soften them, making gradual removal easier.  

• After a thorough inspection of the skin, all observed blisters should be punctured. To puncture a blister, first disinfect the skin, then use a sterile needle to create two holes at the lower and lateral sides of the blister, allowing the fluid to escape. If the fluid doesn’t come out on its own, gently pressing with gauze can help. If this causes significant pain, additional punctures may be necessary, followed by applying ice water to alleviate discomfort. It’s important to note the colour of the blister fluid; it may be bloody, yellow, or purulent, which could indicate an infection. 

• Typically, the fluid will be clear, and the blister roof should remain intact as it provides natural protection to the underlying tissue. If the fluid is not clear and appears bloody or purulent, or if the blister is red or swollen, the blister roof should be removed due to the risk of infection. After puncturing, the blister must be covered with an appropriate dressing, regardless of whether it looks infected. 

• Although various types of dressings are available, the key is to choose those that are soft, flexible, and non-adhesive to facilitate removal, minimize pain, prevent new injuries, and protect the new tissue as it heals. Additionally, they should maintain a moist healing environment that adapts to the amount of exudate from the wound. This type of dressing has been shown to be the most effective in supporting the natural healing process compared to traditional dry dressings. Moist dressings should manage exudate without allowing the wound bed to become too dry or too wet. For children with multiple wounds, it’s important to start by covering the diaper area and the back to enable the baby to rest comfortably on their back and avoid new injuries. Each dressing should be tailored to the child’s specific needs, but it’s advisable to cover the feet first, followed by the abdomen, chest, and arms. In cases of epidermolysis bullosa (EB) with a risk of adherence between fingers and toes, separate bandaging for hands and feet is recommended to prevent sticking, particularly in severe recessive dystrophic EB. 

• Once the primary dressing is applied, it should be secured with a bandage or tubular mesh to prevent movement and reduce the risk of friction-related injuries. It’s crucial that the bandages are not too loose or too tight, and the dressings should be as large as possible since blisters can sometimes form due to friction between the edges of different dressings. Tubular bandages designed for specific body parts, such as tubular bandage gloves for hands, can be very beneficial for children. For the feet, a sock-like bandage covering the entire foot is recommended to minimize injuries from impacts. 

• After covering the wounds, it’s important to hydrate the surrounding healthy skin with specific creams designed for sensitive skin, which can help make the skin more resilient and alleviate itching due to dryness. Special attention should be given to scarred areas, where the use of hyperoxygenated fatty acids in oil or milk may be beneficial.