What is the treatment of Epidermolysis Bullosa?

People with mild forms of EB may not require extensive treatment. However, they should attempt to keep blisters from forming and prevent infection when blisters occur. Individuals with moderate and severe forms may have many complications and require psychological support along with attention to the care and protection of the skin and soft tissues. Patients, parents, or other care providers should not feel that they must tackle all the complicated aspects of EB care alone. There are doctors, nurses, social workers, clergy members, psychologists, dietitians, and patient and parent support groups that can assist with care and provide information and emotional support.

Preventing Blisters

In many forms of EB, blisters will form with the slightest pressure or friction. This may make parents hesitant to pick up and cuddle young babies. However, a baby needs to feel a gentle human touch and affection, and can be picked up when placed on a soft material and supported under the buttocks (bottom) and behind the neck. A baby with EB should never be picked up under the arms.

A number of things can be done to protect the skin from injury. These include:

• Avoiding overheating by keeping rooms at an even temperature.
• Applying lubricants to the skin to reduce friction and keep the skin moist.
• Using simple, soft clothing that requires minimal handling when dressing a child.
• Using sheepskin on car seats and other hard surfaces.
• Wearing mittens at bedtime to help prevent scratching.

Caring for Blistered Skin

When blisters appear, the objectives of care are to reduce pain or discomfort, prevent excessive loss of body fluid, promote healing, and prevent infection.

The doctor may prescribe a mild analgesic to prevent discomfort during changes of dressings (bandages). Dressings that are sticking to the skin may be removed by soaking them off in warm water. Although daily cleansing may include a bath with mild soaps, it may be more comfortable to bathe in stages where small areas are cleaned at a time.

Blisters can become quite large and create a large wound when they break. Therefore, a medical professional will likely provide instructions on how to safely break a blister in its early stages while still leaving the top skin intact to cover the underlying reddened area. One technique is to pat the blister with an alcohol pad before popping it at the sides with a sterile needle or other sterile tool. The fluid can then drain into a sterile gauze pad that is used to dab the blister. After opening and draining, the doctor may suggest that an antibiotic ointment be applied to the area of the blister before covering it with a sterile, nonsticking bandage. To prevent irritation of the skin from tape, a bandage can be secured with a strip of gauze that is tied around it. In milder cases of EB or where areas are difficult to keep covered, the doctor may recommend leaving a punctured blister open to the air.

A moderately moist environment promotes healing, but heavy drainage from blister areas may further irritate the skin, and an absorbent or foam dressing may be needed. There are also contact layer dressings where a mesh layer through which drainage can pass is placed on the wound and is topped by an outer absorbent layer. The doctor or other health care professional may recommend gauze or bandages that are soaked with petroleum jelly, glycerin, or lubricating substances, or may suggest more extensive wound care bandages or products.

Treating Infection

The chances of skin infection can be reduced by good nutrition, which builds the body’s defenses and promotes healin...