Neonatal guidelines for suspected Epidermolysis Bullosa (EB)

1. Epidermolysis Bullosa (EB) is a group of inherited diseases, characterized by mechanical fragility of the skin and mucous membranes. Handling and feeding techniques require modification when nursing an affected neonate, otherwise clinical intervention may result in unnecessary blistering or skin loss.
2. EB is a spectrum of disease, therefore some patients may present with more skin fragility than others, even if they have the same type of EB. Therefore, management of these patients must be tailored to the individual patient
3. Severely affected infants often present with extensive wounds over their limbs resulting from inter uterine movements and compounded by trauma during delivery.
4. Find out here the Neonatal Epidermolysis Bullosa: clinical practice guideline

There are four main types of EB, and within each different type there are various subtypes, ranging from mild to severe. The four main groups of EB are:

• EB simplex (EBS)
• Junctional EB (JEB)
• Dystrophic EB (DEB)
• Kindler EB (KEB)

The cause of each type lies in the absence or reduction of specific proteins which are responsible for maintaining skin integrity. EB is a spectrum of disease, therefore some babies may present with more skin fragility than others, even if they have the same type of EB.

The EB nursing team will take a biopsy to confirm diagnosis and genetics bloods from the baby and both parents to determine the inheritance of the EB on their first outreach visit. The EB nursing team will also support clinical staff unfamiliar with EB to care for these fragile patients. Multiple outreach visits may be required for neonates with significant skin loss/severe EB.

If recommended products outlined in this guideline are not available - discuss with the EB nurses for advice on adaptation or alternatives. These suggestions are for immediate care and may be changed by the EB nurses during their first and subsequent visits.

• Handle with care - avoid rubbing or friction/shearing forces which can result in skin damage.
• Remove cord clamp and replace with ligature to avoid trauma to surrounding skin.
• Heat exacerbates the condition. Nurse in cot/bassinette unless incubator required for medical reasons such as prematurity.
• Try not to apply any sticky tape or name bands. Use photo ID where possible.
• Always remove anything sticky with Medical Adhesive Remover or 50:50.
• We advise against wearing gloves when handling babies with fragile skin. If policy dictates wearing gloves then apply 50:50 white soft paraffin: liquid paraffin or Emollin Spray to the fingertips to prevent friction with the skin.

• Avoid sliding your hands under the baby as shearing forces may cause skin damage. Gently roll the baby onto their side and then roll back onto your hands and lift.
• Lift on a soft pad/ cushion. This is also a safer way for the baby to be held by parents.
• Ensure baby is clothed, with dressings on before holding or lifting. Avoid handling naked babies which can result in accidental damage

• (IV fluids / antibiotics are only necessary in the presence of sepsis or dehydration) Raised CRP in baby with EB is not necessarily an indication of infection in the presence of wide-spread inflammation
• Do not rub area as it may result blisters or skin loss
• Do not use a tourniquet, instead protect skin with soft gauze or a padded dressing to hold, and if needed to squeeze the limb
• Dab skin with cleansing agent if required (rather than rubbing)
• EMLA or AMETOP is safe to use in EB patients. However, DO NOT use adhesive film dressings to cover. Cling film can be used as a safe alternative
• Secure cannula with a Soft Silicone Tape (Siltape, Mepitac or 3m Kind Removal Silicone Tape). If Soft Silicone Tape not available, use adhesive tape but remove following guidelines below

For removal of tape without damaging skin:

• Use a Silicone Medical Adhesive Remover (SMAR) such as Apeel. If SMAR not available, cover with 50% liquid / 50% white soft paraffin, which will dissolve the adhesive and enable safe removal
• Roll any tape back on itself rather than lifting it and pulling the skin

Ensure adequate analgesia given 30 minutes prior to wound care (avoid PR medication if possible as this can blister the anal margin)

• Prepare a clean trolley with a clinical waste bag, hypodermic needles, all dressings (cut to shape using supplied templates) and tape cut into short lengths
• Carefully remove soiled dressings using the SMARs or Emollin spray if stuck
• Lance any new blisters
• Raw wounds: Apply PolyMem/Mepilex Transfer as skin contact, secure by taping it to itself. Do not apply tape directly to the skin
• Secure PolyMem/Mepilex Transfer with a bandage or a retention garment such as Tubifast
• Change dressings daily OR when strike through observed – using adequate analgesia
• Dress fingers and toes individually if raw to avoid digital fusion- use Urgotul / Aquacel/ Mepitel One. Take care that no tape adheres to the skin
• If wearing gloves – please ensure they are well lubricated and change when soiled. If not wearing gloves, ensure adequate hand washing between each limb

If Polymem/Mepilex Transfer is not available, seek advice from EB nurses regarding an alternative or use either a soft silicone dressing or non- adhesive dressing.

Avoid any bathing until inter-uterine and birth damage has healed.

• Blisters are not self-limiting and will enlarge if not lanced. This can result in further tissue damage, pain or infection if left untreated
• Use a piece of soft gauze to gently compress the blister from the side to increase tension
• Use an orange or blue hypodermic needle and pierce the blister at its lowest point
• Slide the needle through the blister to create an entry and exit point
• Withdraw the needle and gently press the blister with the gauze to expel the fluid
• There is no need to dress the blister site if the roof has remained on the blister

When using needles there is a risk of needle stick injury. Therefore, DO NOT re-sheath any used needles, and dispose of any sharps in accordance with the hospital’s waste management policy, or at home following community waste disposal guidelines.

• Cleanse with 50% liquid paraffin, 50% soft white paraffin mix or Emollin Spray and gauze. Avoid using baby wipes which can irritate the skin
• Line nappy with soft liner (dry patient wipes) to prevent elastic rubbing, or apply liberal amount of barrier cream
• Recommended barrier creams: Prosheild Plus, Medihoney, Bepanthen
• Cover open lesions in nappy area with Intrasite Conformable (hydrogel dressing) and change at every nappy change to prevent it from drying out

• Ensure baby is dressed as soon as possible, as clothing offers protection from damage when being handled
• Dress in soft, front fastening baby grow; turn inside out to avoid damage from seams and label
• Neck and feet can be cut out if needed to prevent friction and allow for extra room for limb dressings
• EB nursing team can advise parents further on appropriate clothing for baby once wound care has been established

• Protect infants’ lips with Vaseline (for breast and bottle feeding)
• If breastfeeding, Vaseline can also be used on Mum’s nipple and barrier creams on her breast if having any skin-to-skin contact
• If bottle feeding, choose a soft silicone teat and moisten with cooled boiled/sterile water prior to feeding, or use teething gels if mouth is very sore
• If baby has extensive mucosal damage, the EB nurses can provide a Haberman feeding bottle
• Avoid a naso-gastric tube, if possible, as securing the tube is often problematic and can cause skin damage
• If NG feeding is essential, use tube suitable for long-term feeding and secure with Soft Silicone Tape, or contact the EB nursing team to learn about the Lasoo technique

As a tertiary care hospital, all children who attend GOSH must be referred via Paediatric Dermatology Consultants, Community Paediatricians, Neonatal Units or via a GP.

Please contact the GOSH switchboard and ask for the Dermatology Registrar on call:

Telephone: 020 7405 9200

All new suspected EB referrals should be discussed with the Dermatology Registrar.
A written referral should then follow with an email with pictures.
All relevant imaging should be sent electronically via secure email at the time of the referral.

Dermatology Department
Level 8, Nurses Home
Great Ormond Street Hospital
Great Ormond Street
London
WC1N 3JH

Dermatology referrals: gos-tr.Dermatology-Gosh@nhs.net