A patient with epidermolysis bullosa (EB) often requires special care in the perioperative period in order to prevent unnecessary complications.


General recommendations for perioperative management:

(1) Schedule the patient as the first case of the day and for twice the usual duration.

(2) Ensure adequate anxiolytic therapy for the patient.

(3) Educate all persons involved in the patient's care about the unique problems encountered with these patients, especially skin fragility.

(4) Minimize abrasive forces (see below).

(5) Protect the eyes with saline-soaked gauze or with gel pads.

Problems Associated with Skin Fragility

Intervention to minimize abrasive forces

shearing forces while being moved

allow the patient to move self whenever appropriate; use a slide sheet and not a rigid plastic board to move the patient

complications of adhesive tape

avoid adhesives (tape, pads, etc) whenever possible, using an alternative

need for lubrication to minimize abrasion

liberally lubricate tubing, intubation blades and skin

intravenous access

identify potential sites well in advance


use a soft, pressure-relieving mattress; use padding under pressure cuffs


Evaluate and correect common complications of EB:

(1) anemia (transfuse if necessary)

(2) cardiomyopathy

(3) microstomia (evaluate for a difficult orotracheal intubation)

(4) loose and/or carious teeth (evaluate for loss during intubation)


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