Taking multiple precautions can ensure that pregnancy, delivery, and the postpartum period are uncomplicated in women with epidermolysis bullosa (EB) simplex, a case study reports.
The research, “Management of epidermolysis bullosa simplex in pregnancy: A case report,” was published in the journal Case Reports in Women’s Health.
EB simplex is the most common type of epidermolysis bullosa — a group of rare skin disorders that lead to blistering of the skin — but data on managing pregnancy and childbirth in these patients is scarce.
Previous research indicated that women with EB are not at an increased risk of pregnancy-related complications, and that the skin condition does not appear to worsen during pregnancy. However, malnutrition, severe anemia, and chronic infection need to be monitored, as the skin and mucous membranes are very fragile.
A team at Westchester Medical Center, in New York, described the case of a 27-year-old woman with generalized severe EB simplex who was first treated at the hospital at 26 weeks gestation. She was prone to lesions in the trunk, extremities, and mouth with minimal friction.
A lumbar spine exam found areas of scarring around the mouth, and an echocardiogram — a graphic outline of the heart’s movement — revealed mild mitral regurgitation. With that condition, there is a backward leakage of blood each time the heart’s left ventricle contracts.
The woman’s medical history also revealed iron deficiency anemia. She reported having no relatives with EB and declined prenatal genetic testing.
A multidisciplinary plan — involving an obstetrician, anesthesiologist, neonatologist, dermatologist and nursing personnel — was put into place to play to design the best strategy for the patient’s pregnancy. The main goal was to decrease trauma and thereby the formation or worsening of blisters.
An ultrasound done at 37 weeks showed signs of fetal skin denudation, or loss of skin layers in the baby.
Under spinal anesthesia, the woman underwent a successful cesarean delivery at 40 weeks, following a failed induction of labor and evidence of abnormal fetal heart rate. Clinicians took a series of precautions, including the use of padding and non-adhesive tape, less handling and transfer of the patients, and making larger incisions to help with delivery.
The baby showed extensive blisters indicative of EB.
According to the team, vaginal delivery is generally preferred over cesarean delivery even in possible EB cases, as it may have a lower risk of blistering lesions. Cesarean wounds tend to heal well in women with EB. However, the risks and benefits of the two procedures should be discussed with patients in all cases, the researchers said.
The mother’s postpartum course remained uncomplicated and she did not develop blisters. In this period, multimodal analgesia, or the combined use of multiple pain relief medicines, was used to reduce any excessive movement and skin trauma.
Preserving skin and mucous membrane integrity represents a challenge to the administration of anesthetics, the investigators noted. The subsequent formation of blisters can result in pain, heat, fluid loss, and secondary infection.
However, several precautions can reduce this risk. Any equipment, including the providers’ hands, should be well-lubricated, and non-adhesive materials should not be used. Skin antisepsis, which is the removal of skin microorganisms to prevent infections, can be achieved using dabbing rather than rubbing motions to prevent damage. Neuraxial anesthesia — local anesthetics placed around the nerves of the spine or into the cerebrospinal fluid that surrounds the spinal cord — is preferred over general anesthesia.
These precautions all were utilized in this patient.
Overall, “our patient’s pregnancy and postpartum course remained uneventful,” the clinicians said.
“Parturients [women about to give birth] with EB often need considerable support due to their disability. A coordinated team approach can facilitate uneventful outcomes,” they added.