Management of epidermolysis bullosa simplex in pregnancy: A case report

Abstract

Epidermolysis bullosa (EB) encompasses a group of diseases characterized by extreme fragility of skin and mucous membranes, resulting in blister formation following minimal injury. There are 4 types of EB, with epidermolysis bullosa simplex (EBS) being the most common. We report our experience with the care of a parturient woman diagnosed with EBS. There is little literature on pregnancy in women with this condition. Special precautions are necessary during diagnostic and therapeutic interventions to avoid bullae formation or exacerbation of existing lesions. Frictional or shearing forces are typically more damaging than compressive forces. Multidisciplinary planning was done for our patient to ensure uneventful labor and delivery. Elective induction of labor was started at 40 weeks of gestation. She eventually underwent a cesarean delivery after failed trial of labor. We present this case to highlight the obstetric and anesthetic implications of caring for a parturient with EBS.

Recommendations on pregnancy, childbirth and aftercare in epidermolysis bullosa: a consensus‐based guideline*

“…R26 ↑↑ Offer pressure relief adaptations throughout labour. Frequent position changes will reduce potential pressure damage to the skin 19,29,49 . Consider use of pressure‐relieving mattresses or pillows (e.g.…”

“… 17 , 18 , 19 Depending on the inheritance pattern (autosomal recessive, autosomal dominant) the risk assessment for the pregnancy can be estimated and prenatal testing offered. 11 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 Couples at reproductive risk of severe forms of EB may wish to pursue prenatal or preimplantation genetic testing, accounting for family preferences, religious/cultural beliefs and national regulations. Carrier screening of an unaffected and unrelated partner may be offered according to the individual situation and national regulations, after genetic counselling.…”

“…Where available, a team consisting of midwife, obstetrician, dermatologist, clinical nurse specialist, anaesthetist, occupational therapist and psychologist will improve pregnancy management. 9 , 19 , 29 , 43 , 46 If an infant with EB is expected, timely involvement of the neonatal team and paediatric EB nurse is needed.…”

“…Having a diagnosis of EB is not a contraindication to vaginal birth. 9 , 12 , 19 In some cases, there may be a strong maternal preference for caesarean section (e.g. owing to fear and anxiety regarding trauma to the birth canal resulting in blistering/wounds); the benefits and risks of all options should be discussed with an obstetrician and dermatologist ideally, and an individualized birth plan agreed.…”

Complex Pathology and Management in the Obstetric Patient: A Narrative Review for the Anesthesiologist

“…We have included the following tables for further guidance. Tables 1 and 2 summarize components of the obstetric preanesthetic assessment that have specific considerations [5][6][7][8][9][10][11][12]. Table 3 summarizes benefits, risks, and alternatives of neuraxial and general anesthesia when providing counseling to patients [4,[13][14][15].…”