A rare skin disorder and its management during pregnancy: epidermolysis bullosa: a case report and literature review

Abstract

Epidermolysis bullosa (EB) encompasses a rare group of genetic disorders characterised by extreme skin fragility and blistering in response to minor trauma. Its spectrum of clinical phenotypes range from near-normal life quality with minor blistering, to severe disability with extensive bullosis, scarring, contractures and reduced life expectancy. Pregnancy in those with EB is uncommon and poses significant challenges to the obstetrician, anaesthetist and dermatologist. We report a new case of maternity in a 26-year-old Caucasian with inherited EB, its uncomplicated antenatal course and delivery by elective caesarean section. To date, eleven cases of successful childbirth in women with EB have been documented. Of these, five had vaginal deliveries, two of which had successive deliveries; and six underwent caesarean section. Frequently occurring complications were anaemia, malnutrition, bullous ulceration of the lower abdomen secondary to gravid distension, and trauma to the nipple area following breastfeeding. Only one case was reported to have bulla form at the site of surgical incision and all vaginal deliveries, including those episiotomised, were without urogenital mucosal complications. Based on comprehensive literature review we present recommendations for the management of EB in pregnancy. Multi-disciplinary collaboration, pre-assessment and holistic approaches are vital. The underlying tenets of care for all patients are avoidance of blistering and prevention of secondary infection. These are achieved by padding of the skin where appropriate and the use of non-adhesive dressings. Severe anaemia and chronic infection should be treated early and evidence supports the safe use of regional anaesthesia during labour and/or at caesarean section.

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

“…• Both episiotomies and perineal lacerations tend to heal well in women with EB. 9,21,29,39,42,43 • Standard obstetric management (including sutures) of the episiotomy site is recommended. 9,21,42,43 Key recommendations are based on the results of the literature review.…”

“…Postpartum vaginal discharge (lochia) can last for up to 8 weeks. Advice on soft sanitary wear and skin protection around the groin should be provided. Both episiotomies and perineal lacerations tend to heal well in women with EB 9,21,29,39,42,43 Standard obstetric management (including sutures) of the episiotomy site is recommended 9,21,42,43 …”

“…Although skin involvement does not routinely worsen during pregnancy in EB, 9,12,[39][40][41] there have been occasional reports of wound deterioration related to abdominal distention. [42][43][44][45] A.5 Clinical examination of pregnant women during antenatal visits R9 ↑↑ Engage the multidisciplinary team (MDT) early during pregnancy for patients with complex forms of EB. Where available, a team consisting of midwife, obstetrician, dermatologist, clinical nurse specialist, anaesthetist, occupational therapist and psychologist will improve pregnancy management.…”

“…Although skin involvement does not routinely worsen during pregnancy in EB, 9 , 12 , 39 , 40 , 41 there have been occasional reports of wound deterioration related to abdominal distention. 42 , 43 , 44 , 45…”

“…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.…”