Jacqueline Denyer, Clinical Nurse Specialist (paediatric)Great Ormond Street Hospital, London and DEBRA UK,
Co authors: Lesley Foster & Juliette Turner, Clinical Nurse Specialists (paediatric) Great Ormond Street Hospital, London and DEBRA UK




Epidermolysis bullosa (EB) comprises a group of genetically

determined skin disorders. The common factor is the tendency for

the skin and mucous membranes to break down in response to

minimal everyday trauma and friction. Affected infants may present

with extensive wounds resulting from inter-uterine movements and

damage during the birthing process.

There are few specialised centres for EB and therefore care

generally takes place in local hospitals where experience of this

rare disorder is limited. We do not recommend transfer of a severely

affected infant to a specialised EB centre because the journey and

handling are hazardous for a severely affected infant.




The study aims and objectives were to evaluate the ease of use of

Guidelines for Immediate Care of the Infant with EB (the Guidelines)

and the anticipated improvement in wound healing and reduction of

traumatic injuries.


Newborn infants with skin and mucosal fragility who required
modified handling, feeding and specialised wound care were
selected for the study. Following telephone or email contact with
the specialist centre for EB (Great Ormond Street Hospital (GOSH),
London UK) the nursing and medical staff based at the referring
hospital were directed to the Guidelines.
If recommended dressings were not available immediately
suggestions were made to modify existing materials, e.g. by
application of a greasy emollient to reduce the risk of traumatic
One of the EB Specialist Nurses based at GOSH arranged to travel
to the referring hospital within 48 hours of the referral to modify care
to suit the individual infant, teach handling, feeding and dressing
techniques and take a diagnostic skin biopsy.
Subsequent visits took place 1-2 times weekly depending on current
workload. Factors considered included:
• Ease of application and removal of dressing materials.
• Healing
• Duration of dressing changes
• Pain control (Neonatal Infant Pain Scale)
• Minimal trauma from handling
• Adequate nutritional intake
Using the Guidelines correctly will minimize trauma from handling
and promote wound healing, pain control and general well-being.
The Guidelines are now widely used in our practice.
The high numbers of staff employed in a neonatal unit and the
12 hour shift pattern can result in inconsistency in allocation of
staff to each baby, often a different nurse each day. Although the
Guidelines are prominently displayed by the cot there is not always
compliance with them. Parents are fundamentally important in
ensuring correct care is given but this places an additional burden
on them during this stressful time.


Baby with fragile skin - handle with care!


• No shearing forces or friction!

• Remove cord clamp and replace with a ligature to
avoid trauma to surrounding skin

• Nurse in cot/bassinette unless incubator required for
medical reasons such as prematurity

• No adhesive products or name-bands (use
photographic ID for consent and medication)

• If policy dictates wearing gloves then apply greasy
ointment or lubricant in aerosol form to the fingertips
to prevent friction with the skin


Immediate care of newborn infant with epidermolysis bullosa


If recommended products are not available discuss with EB nurses for advice

on adaptation of alternatives. These suggestions are for immediate care and will be

adapted by the EB nurses  during their first and subsequent visits.



Lift on soft pad. Avoid sliding your hands under the baby as
shearing forces cause damage. Use a “roll and lift technique”
- The infant is gently rolled onto their side, the carers’ hands
placed behind the baby’s head and bottom, the infant rolled
back onto the carers’ hands and lifted.

Nappy area

• Cleanse with 50% liquid paraffin, 50% white soft paraffin mix
or emollient spray and soft gauze
• Line nappy with soft liner to prevent elastic from rubbing.
• Apply an emollient / barrier cream
• Cover open lesions with hydrogel impregnated gauze and
change at every nappy change



• Use a Special Needs Feeder if mouth is sore. Protect lips with petroleum jely.     

• Moisten teat with cooled boiled/  sterile water prior to feeding to

avoid sticking, or use teething gels if mouth is very sore.

• Avoid naso-gastric tube if possible.

If naso-gastric feeding essential,

use tube suitable for longterm feeding and secure with soft silicone tape.



Dress in soft, front fastening baby suit over dressings and nappy. Turn
baby suit inside out to avoid damage from seams and labels.

How to cannulate*
• Do not rub area when cleaning as blisters or skin loss will result
• Do not use a tourniquet or stretch the skin
• Protect skin with soft gauze if assistant needed to squeeze the limb
• Secure cannula with a Soft Silicone Tape
*(IV fluids / antibiotics only necessary in the presence of
sepsis or dehydration) Raised CRP level in a baby with EB is
not necessarily an indication of infection in the presence of
wide-spread inflammation



Regular analgesia is required with additional doses prior to dressing
changes. A combination of paracetamol and oral morphine is
effective. 24% sucrose solution is helpful in reducing procedural pain
in combination with pharmacological management. Feeding the infant
during dressing changes has a calming effect and is encouraged.


Management of blisters

Blisters are not self- limiting and will enlarge if not lanced.
• 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
• It is not necessary to dress the blister site if the roof has remained on
the blister


Wound care

*Ensure adequate analgesia given prior to wound care
• Prepare a clean trolley with clinical waste bag, hypodermic needles,
all dressings (cut to shape) and tape cut into short lengths.
• Carefully remove soiled dressings using the medical adhesive
removers or greasy emollient if stuck.
• Lance any new blisters.
• Raw wounds: Apply polymeric membrane dressings* (PMDs).
• Further secure dressing with wrap-around bandage and or tubular
• Change dressing when “strike through” observed.
• Dress fingers and toes individually if raw to avoid digital fusion- use
lipidocolloid / hydrofiber/ one-sided soft silicone dressings.
• Secure dressing by overlapping and taping to itself. Take care that no
tape comes into contact with the skin.
• Avoid bathing until inter-uterine and birth damage have healed.

    Dressing tips

Example of a template for the
foot and leg

Digits wrapped with hydrofiber and soft
silicone, hand wrapped with PMD


Change dressings before they
become too wet to prevent

Change PMD when exudate is visible
from the top of the dressing.


Remove cord clamp. to avoid

Cut through tape before removal as
pulling can cause blistering.



    Wound healing


All infants achieved good healing using PMD which was easy to apply and
remove. Images show a newborn with Herlitz Junction EB. The first image is prior
to application of PMD, the second shows the improvement two days later.


    How to apply PMD to the newborn

Overlap PMD and secure to itself


Cut slits over the joints to allow

Secure dressing tightly to itself


Secure with tubular bandages.


*PolyMem® and PolyMem®
MAX Wound Dressings
Manufactured by 5133 Northeast Parkway, Fort Worth, TX 76106, USA