Epidermolysis bullosa simplex (EBS) severe

• 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.
• IfYou 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 knock/bumps and overheating.
• Treat hard and thickened skin on the soles of the feet with urea cream.
• The edges of dressing can cause blisters. Try to reduce dressings once raw areas have healed to help prevent rubbing.

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.

If your child has a lot of damaged skin at birth, we recommend delaying bathing until healing has taken place. This is because bathing can be painful and it’s difficult to protect against further skin damage when all the dressings are removed at once.

When you and your child are ready, we recommend bathing or showering regularly to cleanse the skin to help prevent infections.

Tips for bathing:

• Pre-cut replacement dressings before the bath so they’re ready to put on.
• If needed, give prescribed painkillers before bathing.
• Use a toweling baby seat rather than a plastic one if it’s difficult to hold your baby in the bath.
• If your baby has a lot of dressings, sometimes it is safer to bathe them with their dressings on, then change them after the bath.
• Give salt baths when feet are blistered and sore. Your EB nurse can advise on how to do this.
• Use an antimicrobial wash to help prevent skin infections. These should be rotated on a regular basis to prevent antimicrobial resistance.
• Milton baths 1-2 times weekly. Your EB nurse can advise on how to do this.
• Pat skin dry with a soft towel rather than rubbing.

Initially dress your baby in a soft cotton baby grow that fastens at the front. They can wear this inside out to stop the seams from rubbing and causing blisters. Watch out for fasteners that may rub.

Older children can wear ordinary clothes if they’re easy to put on and take off and don’t rub. Some children prefer clothes which don’t have to go over their head and don’t have prominent seams.

Your child’s GP may sometimes be able to prescribe specialist garments that can help keep dressings in place.

If your baby has a lot of very fragile skin, you can nurse them on a small soft pillow/ mattress.

To lift your baby from the mattress, apply a ‘roll and lift’ technique:

1. Roll your baby onto their side.
2. Place one hand behind their head and one hand under their bottom.
3. Allow them to roll back onto your flat hands.
4. Lift.

As you become confident in lifting your baby you may find you don’t need the pillow/ mattress.

To reduce the risk of skin blistering or tearing we also recommend:

• Try to avoid picking babies and toddlers up by their armpits.
• Pad areas of the body prone to knocks and friction with dressings when your baby becomes more mobile.
• Apply dressing to open wounds to help reduce pain and potential infection.

• 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.

Nail care in EBS severe is important because nails can become fragile, thickened, change colour or even fall off.

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.

Blisters in the mouth can make feeding sore and difficult.

Tips for breastfeeding:

• Protect your baby’s face with a layer of emollient to reduce any friction from feeding.

Tips for bottle feeding:

• A special feeder bottle which is easier for the baby to suck can be helpful, as it has a longer, slimmer teat made of ultra-soft silicone.
• You can apply a teething gel to the bottle teat or directly to the mouth before feeding to help ease pain. Gelclair® (a specialist gel) can also be used around or inside the mouth to help reduces pain.

Tips for eating:

Your child’s EB dietitian can advise on appropriate soft foods and supplements to make sure your child can meet their nutritional needs.

They can also advise on how many calories your child needs to support their growth and development.

Some children may need to have regular blood tests to make sure they aren’t lacking in any vitamins or iron.

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 EBS severe 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.

If the eye becomes scratched or blistered:

• Make an appointment to see the ophthalmologist.
• Use antibiotic eye drops rather than the eye drops or ointment.
• Give pain-relief if needed.
• Your child may find it more comfortable to keep their eye closed and avoid bright lights whilst the eye is healing.