Pregnancy and childbirth in epidermolysis bullosa: A clinical case

Background . Epidermolysis bullosa represents a group of genetic skin diseases caused by mutations in genes for structural proteins of the epidermis and dermo-epidermal junction. The population incidence of congenital epidermolysis bullosa in Russia amounts 1:50,000– 1:300,000. Improved early diagnosis and treatment of genetic diseases leads to patients reaching reproductive age, which makes it possible for them to make informed choices about pregnancy and childbirth. World literature contains little information on the management of pregnancy and childbirth in epidermolysis bullosa. In this regard, we consider it important to present the clinical experience of pregnancy and delivery of a patient with this disease. Clinical case description . The clinical experience of managing pregnancy and childbirth in a patient with generalized dystrophic epidermolysis bullosa is presented. The patient performed delivery by cesarean section at term using spinal anesthesia and observing no complications for both mother and newborn. Special precautions were taken during diagnostic and surgical interventions. Given the high probability of bullae formation due to friction and skin shear, a plan was developed to prepare the patient for delivery, measures were taken to prevent trauma to the skin and mucous membranes, and anesthesia method and postoperative care for the patient and the newborn were defined. In order to reduce skin traumatization during all manipulations, sanitary napkins, emollients, non-adhesive silicone tapes and dressings were used. Newborns were provided with special care to prevent possible skin injuries. Although epidermolysis bullosa increases the risk of maternal complications, joint efforts of obstetricians, dermatologists, anesthesiologists, neonatologists, and psychologists manage to minimize complications for both mother and newborn when managing pregnancy and childbirth. Conclusion . A multidisciplinary approach involving an obstetrician-gynecologist, dermatologist, anesthesiologist, neonatologist, and psychologist improves the outcomes of pregnancy and childbirth management and minimizes risks to both mother and newborn.