116 - A Review of Oral Health in 75 patients with Epidermolysis Bullosa

Purpose: The purpose of this study was to review oral health findings and practices in a cohort of 75 subjects with Epidermolysis Bullosa (EB). Additionally, this study examined nutrition and pain impact.

Methods: Caregivers/Patients completed a 15-item oral health intake form in this IRB exempt study.  Questions surveyed included demographics, dental history, primary nutrition, oral findings associated with EB and access to dental care. 

Results: Data were collected from 75 patients over a three-year period. Most patients had a diagnosis of Dystrophic EB (61%) with 73% of those having a recessive inheritance pattern. Nearly two-thirds (65%) of patients had exclusive oral nutrition with a significant number of dystrophic (P=0.05) having exclusive g-tube nutrition. Over half of patients (58%) reported weekly oral blistering, with the majority (37%) being on the tonge. Additionally, 56% were found to have some degree of microstomia and ankyloglossia (49%), and dental crowding (62%). While 8% reported moderate-severe dental pain, 57% reported having to travel >50 miles for dental access, with no difference between EB subtype (P=0.78)

Conclusion:

Conclusion: Patients with Epidermolysis Bullosa presented specific oral challenges associated with EB subtype and oral anatomic findings. Findings for crowding, microstomia, g-tube nutrition in Recessive Dystrophic EB patients should be considered for posterior tooth extraction to potentially improve oral hygiene.