Wo u n d M a n a g e m e n t
f o r C h i l d re n wi t h
Epidermolysis Bullosa
Jacqueline E. Denyer, RGN, RSCN, RHVa,b,*
KEYWORDS
 Epidermolysis bullosa  Wounds  Dressings  Atraumatic
Skin and wound care in EB is specific both to the
type of EB and to individual wounds within each
child. Availability of dressings and personal prefer-
ence are also paramount in the selection of mate-
rials. The ideal dressing is yet to be developed,
although there are now a variety of suitable dress-
ings available. Wound healing is challenging and
chronic wounds often feature. Factors adversely
affecting healing include anemia, malnutrition,1
infection, and pruritus.
CARE AND MANAGEMENT OF NEONATES
Appearance at birth may not necessarily indicate
the type of EB or its severity. Factors such as
mode of delivery and level of intrauterine move-
ments are reflected in the amount of skin loss at
birth, and those delivered by cesarean section
may appear deceptively mildly affected but have
a severe form of EB.2
To minimize further damage to this vulnerable
group it is recommended that term infants are
not nursed in an incubator, as the hot and humid
environment can encourage blistering. Wounds
should be covered with a nonadherent dressing
such as Mepitel (Mölnlycke Healthcare, Sweden)3
or Urgotul (Urgo, France)4 (Tables 1–3) with
secondary foam dressings used for absorption of
exudate and protection from baby movements
such as kicking. Where two raw surfaces are adja-
cent to each other, dressings should be placed
between the digits to prevent fusion (Fig. 1). This
procedure is of particular importance in those
with dystrophic forms of EB, but fusion is possible
in all types if digits are dressed without due care. It
a
Department of Dermatology, Great Ormond Street Hospital, London WC1N 3JH, UK
b
DebRA UK, 13 Wellington Business Park, Dukes Ride, Crowthorne, RG5 6LS, UK
* Department of Dermatology, Great Ormond Street Hospital, London WC1N 3JH, UK.
E-mail address: Jackie.denyer@debra.org.uk
Dermatol Clin 28 (2010) 257–264
doi:10.1016/j.det.2010.01.002
0733-8635/10/$ – see front matter ª 2010 Elsevier Inc. All rights reserved.
may be necessary to apply dressings in such
a way to minimize deformity, for example, exerting
a slight pull in the opposite direction to a rotated
foot.
Umbilical venous catheters are rarely necessary
and attempted insertion of these can cause major
skin damage (Fig. 2). Prophylactic antibiotic cover
is not indicated, and oral feeding should be
possible provided a specialized teat such as
a Haberman/Special Needs Feeder is used in
conjunction with topical analgesia. Breast feeding
may also be possible.
If intravenous access is necessary, the cannula
should be secured using silicone-based, rather
than adhesive tapes (Mepitac, Mölnlycke Health-
care or Siltape, Advancis, Nottinghamshire, UK).
Cannulae must be well padded to avoid damage
to the skin from baby movements and in particular
the risk of corneal abrasions if the cannula is sited
in the hand. Periumbilical damage is common from
trauma caused by plastic cord clamps, and it is
recommended that these be removed and re-
placed by a ligature.
To minimize trauma from handling the baby
should be nursed on a soft mattress recommen-
ded for infants, such as an incubator pad. The
infant can be lifted onto this. When it is necessary
to handle the baby off the mattress employ a ‘‘roll
and lift’’ technique: roll the baby onto his or her
side, place one hand flat behind the head and
the other under the buttocks, press down onto
the cot surface, and allow the baby to roll back
onto your hands and lift.
Bathing should be delayed until the interuterine
and birth damage have healed, because it is
derm.theclinics.com
258 Denyer

Table 1
Recommended dressings for EB simplex

Contraindication/
Type Brand Manufacturer Indication Comments
Primary Mepitel Mölnlycke Wound Dowling-Meara
Healthcare
Urgotul Urgo Wound Very moist
(problems with
retention)
Foam Mepilex/Mepilex Mölnlycke Protection Heat-related
Lite/Mepilex Healthcare blistering
Transfer
Hydrogel Intra site Smith & Nephew Cooling; pain
Conformable reduction
Biosynthetic SuprasorbX Activa Cooling; pain
cellulose reduction
Bordered Mepilex Border/ Mölnlycke Protection May require
dressings Border Lite Healthcare removal
Allevyn Gentle Smith & Nephew assisted by
Border Silicone Medical
Urgotul Duo Urgo Removers such
Border as Appeel
(Clinimed) or
Niltac (Trio
Healthcare) to
avoid skin
stripping
Hydrofiber Aquacel Convatec Dowling-Meara
Powder Cornflour Apply following Nappy area
Catrix Cranage lancing of
Healthcare blister

Table 2
Recommended dressings for junctional EB

Type Brand Manufacturer Indication Contraindication Wear Time

Hydrogel Intra Site Smith & Infant Herlitz Change daily
Conformable Nephew junctional EB or when dry
Hydrofiber Aquacel Convatec Very moist Lightly exuding Change every
wounds where or dry wounds 3–4 d
difficult to keep
dressing in
place
Primary Urgotul Urgo Primary dressing Change every
dressing 3–4 d
Soft silicone Mepilex/Mepilex Mölnlycke Protection; As determined
foam Lite/Mepilex Healthcare absorption by exudate
Transfer level
Polymeric PolyMem Ferris Chronic wounds; As determined
membrane critical by exudate
colonization/ level
infection
Table 3
Recommended dressings for dystrophic EB; for antimicrobial management please see Table 4

Contraindication/
Type Brand Manufacturer Indication Comments Wear Time
Soft silicone primary Mepitel Mölnlycke Healthcare Moist wound Silicone sensitivity 3–4 d depending on
dressing Silflex Advancis Medical presence of infection
and patient choice
Lipidocolloid Urgotul Urgo Moist wound, drier Where retention is
Primary dressing wounds and difficult
protection of
vulnerable skin
Foam dressings Mepilex Mölnlycke Healthcare Absorption of exudate Overheating Every 3–4 d
Soft silicone Protection May need to apply over
Mepilex Lite Lightly exuding wounds recommended
Mepilex Transfer To transfer exudate to atraumatic primary
absorbent dressing dressing such as
Where conformability Mepitel or Urgotul

Management of Epidermolysis Bullosa

required—digits,
axillae
Foam dressings Alleyvn Smith & Nephew Absorption May need to apply over Every 3–4 d
Urgocell Urgo Protection recommended
atraumatic primary
dressing
Polymeric membrane PolyMem Ferris Where cleansing Depending on exudate
required levels
Bordered dressings Mepilex Border/Border Mölnlycke Healthcare Isolated wounds May require removal 3–4 d depending on
Lite Dominant dystrophic with Silicone Medical personal choice
Alleyvn Gentle Border Smith & Nephew and mild dystrophic Adhesive Remover to
EB avoid skin stripping

259
260 Denyer

Regular analgesia is paramount in this age

group, as evidence suggests neonates may be
highly sensitive to pain,5 and poor management
in the neonatal period can cause heightened
sensitivity throughout life. Assessment may be
complex in this age group as a lack of vigorous
responses to pain may be demonstrated.6

DRESSING MANAGEMENT IN EB SIMPLEX

This group of children is difficult to manage, as
dressings may cause blistering around the edges
and heat from the dressing can result in additional
Fig. 1. Dress digits individually to avoid fusion. blistering. Probably the most challenging are
infants who are severely affected with Dowling-
Meara EB simplex. These infants often have large
difficult to prevent damage to the naked infant. For
areas of skin loss, but traditional dressing
this reason dressings should be changed on a limb
management frequently leads to blistering around
by limb basis rather than all dressings being
the edges of the dressings (Fig. 3).7 The best toler-
removed at one time. If the correct dressing is
ated dressing is a Hydrofiber (see Table 1) and this
used the wound should not require cleansing,
can be used to protect skin from friction from the
but if needed gentle irrigation can be done using
edges of other dressings. As soon as the wound
warmed saline delivered via a syringe.
is healed it is recommended that dressings be
Nappy area care requires adaptation in those
removed and the infant dressed in soft, flat,
with EB. Cleansing with water can sting open
seamed clothing or that clothes with raised seams
wounds and therefore cleansing with equal parts
are worn inside out. The main management is to
of liquid and white soft paraffin in the form of an
lance the multiple blisters as soon as they arise
ointment or aerosol spray is advocated (commer-
(Fig. 4). Simple cornflour applied to the blistered
cially available as Emollin 50/50 emollient spray,
area helps it to dry and reduces friction. Catrix
CD Medical Ltd). The nappy should be lined with
powder (bovine cartilage powder; Cranage Health-
a soft disposable cloth to prevent friction and
care International) (see Table 1) is a prescribable
trauma from the edges of the nappy. Open
medically approved alternative, and early work
wounds in the nappy area should be covered
has suggested more rapid resolution of blisters
with a hydrogel-impregnated gauze (Intrasite
when Catrix is applied. Children often suffer from
Conformable, Smith & Nephew) which is replaced
repeated infections, and the author has had
at every nappy change. Avoid the temptation to
success in reducing the incidence of bacterial
use a larger size nappy than indicated by the size
overgrowth by using garments containing a silver
of the baby or to fasten the nappy loosely, as
thread.
this will encourage friction when the baby moves
Older children with Dowling Meara and those
inside the nappy.
with localized EB simplex encounter a higher

Fig. 2. Damage from attempted insertion of umbilical

catheter. Fig. 3. Blistering from tubular retention bandage.

Fig. 4. Lance blisters to prevent enlargement.
incidence of blistering in hot and humid conditions.
Dressings are principally required for the feet, and
preference varies. Thin silicone-based foams are
generally well tolerated. Many like to use sheet hy-
drogels, as these have a cooling effect that
reduces pain.
A large proportion of affected children prefer not
to use any dressings at all. Commercial socks con-
taining a silver thread help to reduce heat and
bacterial load, and are popular with this group of
patients. Dermasilk socks provide an anti friction
layer and also reduce the bacterial load.
DRESSING MANAGEMENT FOR THOSE WITH
JUNCTIONAL EB
Herlitz Junctional EB
Affected infants often present with minimal skin
damage at birth, but there is a marked tendency
for chronic wounds to develop early in this group
of largely life-limited children. A small number of
infants have large, heavily exuding wounds
present at birth, and these are very difficult to
dress as there is a tendency for the dressings to
slip. In these cases the author has found that using
a Hydrofiber dressing directly to the wound and
covering this with an absorbent dressing provides
a stable option.
In general, dressings such as soft silicone and
foams that aid healing in other types of EB provide
comfort, but do not appear to be effective in those
with Herlitz Junctional EB. In the author’s experi-
ence the best form of management is to use a lipi-
docolloid dressing to cover open wounds, and use
hydrogel-impregnated gauze as a secondary
dressing (see Table 2) which is then secured
with tubular bandage that must be cut shorter
than the secondary dressing to avoid blistering
from the edges of both the dressing and retention
agent. The hydrogel dressings should be changed
as soon as they begin to dry out, but the primary
dressing can be left in place for several days.
Management of Epidermolysis Bullosa 261
Successful healing has resulted from this method
and despite progressive cachexia and dysphasia,
infants have maintained largely intact skin
throughout life.
Those with non-Herlitz forms of Junctional EB
and longer-term survivors with Herlitz Junctional
EB benefit from more traditional dressings recom-
mended for dystrophic EB, as the method
described here is not always practical in older
children.
DRESSING MANAGEMENT FOR THOSE WITH
DYSTROPHIC EB
Mild Recessive and Dominant Dystrophic
Children with mild recessive or dominant dystro-
phic EB generally require dressings for wounds
that have developed over bony prominences
such as knees, ankles, backs of hands, and digits.
Many can tolerate bordered silicone or adhesive
dressings (see Table 3) although a Silicone
Medical Adhesive Remover may be required if
skin stripping occurs on removal.8,9 Children often
like these dressings, as they do not need retention
bandaging and look like regular sticking plasters
as used by their peers.
Severe Generalized Dystrophic EB
Those affected by severe forms of dystrophic EB
may require extensive dressings in an attempt to
heal wounds, and to offer protection against fric-
tion and shearing forces. A range of dressings is
suitable for those with dystrophic EB but choice
is often limited by size of the dressing. A large
proportion will suffer from chronic wounds, which
are challenging for all concerned. Atraumatic
primary dressings include soft silicone mesh and
lipidocolloid dressings. These require a secondary
dressing for exudate management and protection.
Foam dressings are commonly used, although
additional highly absorptive dressings may be
required if exudate is excessive.
Infection and critical colonization appear to be
a major factor in the persistence of wounds, and
use of an antimicrobial applied topically or medi-
cated dressings are recommended (Table 4).10
Silver-impregnated dressings are very effective in
reducing the bioburden11 but as there is a concern
about raised plasma silver levels the author
currently does not use these in children. Honey
dressings or stabilized topical hydrogen peroxide
cream is prescribed in preference to silver prod-
ucts. Honey is available in the form of impregnated
dressings and ointments, and is effective both in
the management of chronic wounds and reduction
of the bioburden.12 Unfortunately, some patients
experience stinging and pain in response to the
 262
Denyer
Table 4
Recommended dressings for infected and critically colonized wounds and where biofilm is present

Type Brand Manufacturer Specific Indication Contraindication/Comments Wear Time

Honey Malodorous wounds Pain 3–7 d depending on patient
Infection/critical In conditions where insects choice
colonization are rife May need to change
May need to use over secondary dressing more
recommended primary frequently due to increase
dressing to ensure in exudate
nonadherence
Algivon Advancis Medical
Medihoney Gel Medihoney Sensitive wounds where Replace when no evidence of
Sheet removal resisted gel sheet remains
Mesitran S (ointment) Aspen Medical Sensitive wounds Apply at each dressing change
Silver Mepilex Ag Mölnlycke Health Where foam dressing
care required
Urgotul Silver/SSD Urgo Primary dressing
PolyMem Silver Ferris
Aquacel Ag Convatec EB simplex with
diabetes mellitus
Other Suprasorb X1 PHMB Activa Healthcare Pain Daily for optimum pain relief
Lohmann & Rauscher Itching and cooling effect
Other Cutimed BSN As dictated by strike through
Sorbact on secondary dressing
Other PolyMem Ferris When strike through observed
Other Crystacide Derma Superficial infection

pH level and the osmotic pull. Cutimed Sorbact
(BSN) dressings remove bacteria by the process
of hydrophobic interaction; the dressings are
coated with a fatty acid derivative that attracts
bacteria to the dressing, where they become
bound. Initial studies have shown this dressing to
be effective in wound healing in those with chronic
wounds associated with EB.
Dressings containing Polyhexanide (PHMB)
such as Suprasorb X1 PHMB (Activa Healthcare,
Lohmann & Rauscher, UK) offer antimicrobial
management of critically colonized and infected
wounds, and are recommended for long-term
use. Polymeric membrane dressing (PolyMem,
Ferris, OH, USA) contains a cleanser (surfactant)
that also reduces the bioburden and has enabled
healing of recalcitrant wounds. Polymeric
membrane dressings also have the advantage of
being a ‘‘stand-alone’’ dressing without the need
for a nonadherent primary dressing or secondary
dressing for protection or exudate management.
Frequency of dressing changes is determined by
personal choice, time available, and level of
exudate. Infected or critically colonized wounds
require more frequent dressing changes. Use of
honey products and polymeric membrane dress-
ings increase exudate initially, so commitment to
daily dressing changes must be ascertained
before starting these.
Bathing is encouraged for those with severe
forms of EB but may not be tolerated due to diffi-
culties resulting from pain management and
handling. It has been reported that adding an
unspecified large amount of salt to the water
reduces pain both while in the bath and while wait-
ing for the wounds to be redressed. The author’s
experience reflects this. When bathing is not
possible and the child also refuses cleansing of
the wound, polymeric membrane dressings have
proved very effective in reducing the bioburden.
Adequate pain relief before use of dressings is
essential, and additional sedation may also be
required. Need for medication varies from simple
analgesia such as paracetamol or ibuprofen to
opiates including tramadol and morphine.13,14
Dressings impregnated with ibuprofen (Biatain-
Ibu) have proved helpful for some wounds,
although these are not licensed for children
younger than 12 years.15 Topical morphine in hy-
drogel is also effective. Other dressings with
pain-relieving properties include biosynthetic
cellulose such as Suprasorb X, which has an addi-
tional cooling effect and is helpful in reducing pain
associated with both blisters and wounds. Thera-
pies such as guided imagery and distraction tech-
niques can be taught to families and carers to
reduce the distress of procedural pain.
Management of Epidermolysis Bullosa 263
Fig. 5. Template for foot dressing.
TIPS AND HINTS
 Turn off fans before removing dressings to
reduce pain from the circulating air.
 Cut templates of dressing shapes to aid
carers who are unfamiliar with the wound
requirements (Fig. 5).
 Cut all dressings anticipated for dressing
change before starting.
 Demonstrate new products in advance of
their use, FOR EXAMPLE, Silicone Medical
Adhesive Removers
 If using new products such as honey based
products that have the potential to sting, try
it on a small wound first before widespread
application.
 Use tubular bandage as retention where
possible. Place bandage above or below
wound before dressing is applied, so it
can be pulled into place quickly before
dressing moves.
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