A review of the obstetric management of patients with epidermolysis bullosa

Abstract

Summary: Epidermolysis bullosa (EB) is a rare inherited skin condition characterized by the development of blisters after minor mechanical friction or trauma. There are few reported pregnancies in the literature in these women. We describe a pregnancy we recently managed. The collated series of pregnancies show that there are no additional antenatal or postnatal problems and that the skin itself does not worsen during pregnancy. Vaginal delivery is most favourable, but if a caesarean section is required, regional anaesthesia should be attempted. A multidisciplinary team should be active throughout the pregnancy of these women and a well-documented labour plan should be made in advance.

Management of epidermolysis bullosa simplex in pregnancy: A case report

“…Malnutrition, severe anemia and chronic infection may be associated and should be addressed [10]. Genetic counseling should be offered to patients, given the inheritance pattern [8,9,14], as evident from our case.…”

“…Pregnancy is relatively uncommon, due to the associated disability and concerns regarding affected children [8,9]. Nevertheless, successful pregnancy outcomes have been reported even in women with severe types of EB [3,[9], [10], [11], [12], [13]]. Women with EB are not at increased risk of pregnancy-related complications [8], and the skin itself does not seem to worsen during pregnancy [9].…”

“…Nevertheless, successful pregnancy outcomes have been reported even in women with severe types of EB [3,[9], [10], [11], [12], [13]]. Women with EB are not at increased risk of pregnancy-related complications [8], and the skin itself does not seem to worsen during pregnancy [9]. Malnutrition, severe anemia and chronic infection may be associated and should be addressed [10].…”

“…Normal vaginal delivery is considered safe and preferred to CD even if the mother is expected to deliver a baby with EB [8]. There is a theoretical risk of blistering of vaginal mucosa, possible sloughing of the cervical and perineal epithelium and damage to soft tissues of pelvic floor [9]. Prolonged labor and associated immobility may predispose to lesions in the lower back, buttocks and arms [10].…”

“…Episiotomy is acceptable to reduce perineal tears [8]. Besides obstetric indications, CD may be indicated with genital tract involvement to minimize perineal bullae [9]. Although, blistering and scarring can occur at the incision site, cesarean wounds tend to heal well in women with EB [8].…”