Clinical Management of EB

• Prevent shear and friction injuries from the first touch.
• Always lift patients, or allow them to self transfer, rather than slide or pull on them.
• Consider using photographs from most recent bandage change to view all wounds, and only remove bandages to visualize the wounds you are concerned about for routine assessments. 
• Use non-adhesive padding or tubular gauze to protect high-friction areas.

• Select and secure dressings without adhesives.
• Never apply adhesives directly to the skin.
• Use non-adherent dressings (i.e., EB-Friendly Dressings List).
• Remove adhesives from monitors or apply over silicone/hydrogel barriers.
• Add padding over high-risk pressure points.

EB blisters are not self-limited; once they form, they often continue to enlarge unless they are drained and protected. Follow these step-by-step recommendations for safe lancing here. 

Guidelines for Safe Blister Drainage

• Wash hands and assemble supplies, wear gloves.
• Use a sterile needle, lancet, or 11 blade to lance tense blisters dime size or larger (*do NOT lance blisters in the mouth or near the eyes*).
• Gently push the fluid to one corner of the blister.
• Prick 1–2 holes on the raised side (not top) of the blister.
• Using gauze or a Q-tip, gently drain the fluid from the opening, keeping the blister roof intact.
• Reuse the lancet only if there are no signs of infection (white or cloudy drainage).
• Select a non-adherent dressing larger than the wound → lightly coat the patient facing side of the dressing with ointment or emollient to prevent sticking → apply to area → use rolled gauze and netting to secure bandage.

🚫 DO NOT USE ADHESIVE TAPE OR REGULAR BANDAIDS. In an incident where adhesives have been applied, leave in place until patient is stable. Use adhesive removal spray or emollient to gently loosen and carefully remove adhesives to minimize trauma. Alternatives include Vaseline®, mineral oil, coconut oil, normal saline, baby oil, or tap water.

• Use gentle technique to avoid trauma, do not grab or skin their skin.
• Cleanse intact area of skin by dabbing, not rubbing.
• Place tourniquets over gauze or bandage padding.
• Only allow the most experienced phlebotomists to attempt blood draw, ultrasound guidance can be helpful to find a vein.
• Give vaccines as usual; apply light pressure, not massage.
• Cover with silicone non adhesive dressing, do not use Band-Aids.

Refer to Emergency Management for EB for detailed recommendations.

• Recognize and treat infection promptly.
• EB wounds are prone to bacterial infection.
• Watch for increased redness, pain, pus, odor, honey-colored crusts, fever, or chills.
• Culture when suspicious; systemic cases with fever require labs and blood cultures.

• Control discomfort with over the counter analgesics when possible and opiods when necessary to manage pain.
• Antihistamines can reduce itching.
• Discuss strategies to prevent constipation if opioids are used.

• Address swallowing issues and maintain high-calorie intake.
• Dysphagia, strictures, and constipation are common.
• Dietitian involvement is essential.
• Encourage soft, high-protein foods and supplements.
• Assess the full esophagus; balloon dilatation may be needed.

• Monitor for chronic anemia and nutrient loss.
• Iron supplementation is often required.
• Check selenium and carnitine levels when indicated.

• Reduce friction through soft, seamless garments.
• Avoid seams, tags, and tight waistbands.
• Choose loose, padded shoes with rubber soles.
• Pad diapers and waistbands.

• Keep skin cool and dry to prevent new blistering.
• Avoid heat and humidity.
• Use body-temperature or cooler water.
• Maintain air-conditioned environments when possible.
• Pad diapers and waistbands.