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



Fig. 1


Dress digits individually to avoid fusion.


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.


Feb 12, 2018 | Posted by in Dermatology | Comments Off on Wound Management for Children with Epidermolysis Bullosa

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