431 - Epidermolysis Bullosa and Dental Management: A Case Report of a 14-Year-Old Patient

Epidermolysis bullosa (EB) is a group of inherited chronic disorders characterized by fragile skin and mucous membranes in response to minor injury or friction. EB presents clinically in a wide spectrum and is classified in 4 types. This case report presents dental restorative and recall management of a 14-year-old male diagnosed with recessive dystrophic EB caused by a collagen VII gene mutation. He has been followed by UCSF Pediatric Dentistry since 2015 for routine preventive care in chair and multiple instances of comprehensive operative care under general anesthesia. The patient’s medical and dental history includes chronic malnutrition, anemia requiring transfusions, corneal scarring, upper GI bleeding, syndactyly, history of dilated cardiopathy, severe skin blistering and extremity deformation, as well as severe dental anxiety, extreme difficulty for dental hygiene maintenance, rampant caries, microstomia/trismus, severe crowding/malocclusion, and oral blistering/scarring. Operative dental treatment required profound precautions to minimize trauma to his fragile skin and mucosa. Treatment plan considerations included crowding alleviation for a functional and aligned dentition without the need of orthodontic treatment. This case highlights the importance of understanding the unique oral and systemic manifestations of EB critical for delivering tailored care and ensuring optimal oral health and overall quality life.