Wound Management for Children with Epidermolysis Bullosa

Skin and wound care in epidermolysis bullosa (EB) is specific both to the type of EB and to individual wounds within each child. Availability of dressings and personal preference are also paramount in the selection of materials. The ideal dressing is yet to be developed, although there are now a variety of suitable dressings available. This article discusses current techniques of wound and dressing management for EB simplex, junctional EB, and dystrophic EB. Factors adversely affecting healing include anemia, malnutrition, infection, and pruritus.

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 preference are also paramount in the selection of materials. The ideal dressing is yet to be developed, although there are now a variety of suitable dressings available. Wound healing is challenging and chronic wounds often feature. Factors adversely affecting healing include anemia, malnutrition, 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 movements 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.

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) or Urgotul (Urgo, France) ( 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 adjacent 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 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.

Table 1

Recommended dressings for EB simplex

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

Table 2

Recommended dressings for junctional EB

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

Table 3

Recommended dressings for dystrophic EB; for antimicrobial management please see Table 4

Type	Brand	Manufacturer	Indication	Contraindication/Comments	Wear Time
Soft silicone primary dressing	Mepitel Silflex	Mölnlycke Healthcare Advancis Medical	Moist wound	Silicone sensitivity	3–4 d depending on presence of infection and patient choice
Lipidocolloid Primary dressing	Urgotul	Urgo	Moist wound, drier wounds and protection of vulnerable skin	Where retention is difficult
Foam dressings Soft silicone	Mepilex Mepilex Lite Mepilex Transfer	Mölnlycke Healthcare	Absorption of exudate Protection Lightly exuding wounds To transfer exudate to absorbent dressing Where conformability required—digits, axillae	Overheating May need to apply over recommended atraumatic primary dressing such as Mepitel or Urgotul	Every 3–4 d
Foam dressings	Alleyvn Urgocell	Smith & Nephew Urgo	Absorption Protection	May need to apply over recommended atraumatic primary dressing	Every 3–4 d
Polymeric membrane	PolyMem	Ferris	Where cleansing required		Depending on exudate levels
Bordered dressings	Mepilex Border/Border Lite Alleyvn Gentle Border	Mölnlycke Healthcare Smith & Nephew	Isolated wounds Dominant dystrophic and mild dystrophic EB	May require removal with Silicone Medical Adhesive Remover to avoid skin stripping	3–4 d depending on personal choice

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.