Dominant dystrophic epidermolysis bullosa (DDEB)

• Use non-stick dressings and gentle bandaging. Apply dressings to open wounds to encourage healing and to prevent wounds from sticking to clothing.
• It’s common for the skin to blister on bony bits of the body, such as elbows and knees. You can use non-stick dressings to pad and protect these areas to help prevent damage to the skin.
• If you can use a non-sting medical adhesive remover if dressings become stuck to the skin. This will help remove the dressing without causing pain or further damage.
• Keep the skin clean to prevent infection. Infected wounds can be treated with topical wound gels, creams, and ointments. However, if your child has a fever, you should contact the GP- they may need antibiotics to help treat the infection. Please ask the GP to take a wound swab before prescribing antibiotics.
• If you are concerned about your child’s wounds, please contact the EB nursing team to discuss the best treatment options.

Blisters and wounds can itch and be painful.

Over the counter medications such as paracetamol and ibuprofen can help. Please speak with your child’s EB team about how to manage your child’s pain if you have any concerns.

It can also help to moisturize the skin daily to reduce dryness.

• Choose soft, seamless, loose-fitting clothing.
• Choose footwear to avoid rubbing.
• Avoid knocks/bumps and overheating.

It’s important to pop blisters to stop them from getting bigger and becoming painful or infected.

• Use a piece of soft gauze to gently compress the blister from the side to increase tension.
• Use a small hypodermic sterile needle (or small clean sharp scissors) to pierce the blister.
• Slide the needle through the blister to create an entry and exit point. If the blister is large, you may need to do this more the once.
• Withdraw the needle and gently press the blister with the gauze to remove the fluid.
• Dispose of the needle into a sharps bin.
• You don’t need to use a dressing if the top of the blister is still in place.
• Use a dressing is there is skin loss or if the area looks particularly sore.

If blisters are left this can result in further damage or wounds to the surrounding skin.

We recommend bathing or showering regularly to help cleanse the skin. Commercial shower gels and washes are suitable for most children with DDEB. If your child has recurrent skin infections, please contact your EB team who can recommend a mild antiseptic wash for the skin.

Tips for skin cleansing:

• Salt baths when feet are blistered and sore. Your EB team can advise on this.
• If needed, give prescribed painkillers before bathing.
• Pat the skin dry with a soft towel rather than rubbing.

In DDEB nails (especially toenails) can become fragile, thickened, change colour, or even fall off.

Therefore it’s important to look after them.

Tips for managing nails:

• Keep nails short and smooth. Trim nails regularly with gentle, rounded scissors or a nail file.
• File rough edges carefully to avoid scratching nearby skin.
• Soften nails before trimming by soaking them in warm salt water. Your EB team can also recommend a urea cream to help soften the nails.
• Avoid tight shoes or socks that rub the nails.
• Use protective dressings around the fingers/toes if they blister often.
• Watch for signs of infection. Look for redness, swelling, pus, or increased pain around the nail.
• Moisturise regularly. Use gentle moisturisers around the nail folds and cuticles.
• It is common for nails to fall off in DDEB. If they do, keep the area clean and covered. New nail growth many be abnormal, or the nail may not grow back at all.

For thickened or very painful nails which are difficult to manage at home your EB Team can refer you to an EB Podiatrist who can help and show you what to do.

Try to pick shoes to prevent friction, reduce pressure, and protect fragile skin.

Tips for choosing shoes:

• Avoid rough seams or stitching inside that could rub or cause blisters. Some brands offer seamless socks/shoes specifically for sensitive skin.
• Avoid shoes that are too tight- choose shoes with extra depth and width. This allows room for dressings and bandages.
• Soft, padded insoles can help absorb shock and reduce impact on the feet.
• Velcro or soft laces are best for adjusting fit without pressure points. Avoid zips or buckles near sensitive areas.
• Try to pick breathable materials- leather or mesh can help reduce heat and sweating and help reduce the risk of blistering.
• A soft, flexible sole moves with the foot and reduces friction while walking.

• Avoid using scented baby wipes if the nappy area is red or broken.
• Cleanse with 50/50 ointment or Emollin® emollient spray.
• Pop blisters (as above).
• Apply a layer of barrier cream, to any blistered areas or to any areas that the nappy may rub.
• Cover any open wounds with a dressing. You may also need to line the edges of the nappy with a soft material to prevent rubbing.

Constipation is a frequent problem with all types of EB. Blistering and soreness around the bottom can make it uncomfortable for your child to poo, and pain medicines and iron supplements can also cause constipation.

Please speak with your child’s EB team who will be able to advise on medicine to help them poo or make pooing more comfortable. A dietitian will be able to offer advice on how diet can improve this.

Children with DDEB can sometimes develop blisters/small ulcers in the mouth. This can sometimes make cleaning the teeth painful.

To help we suggest:

• Brush teeth with a soft toothbrush.
• Try to avoid very hot or very cold food and drinks.
• Have regular check-ups with the dentist. They may also prescribe fluoride supplements.

Dry eyes caused by a reduced tear film can sometimes cause blistering on the surface of the eyes which can be painful. An ophthalmologist (eye specialist) can prescribe eye drops or ointment to help keep the eyes moist and reduce the chance of blistering.