The impact of oral manifestations on Oral Health-Related Quality of Life in adults with Ehlers-Danlos Syndrome

Abstract

Ehlers-Danlos syndromes (EDS) are heritable connective tissue disorders (HCTDs) characterised by multisystemic involvement including clinically significant oral and craniofacial manifestations. The literature indicates that these oral features may affect oral-health-related quality of life (OHRQoL). This review presents an analysis of the relationship between oral manifestations and OHRQoL in adults with EDS, synthesising quantitative Oral Health Impact Profile (OHIP) evidence with qualitative lived experiences. Chronic orofacial pain, temporomandibular disorders (TMD), mucosal fragility, periodontal susceptibility and resistance to local anaesthetic (LA) were consistently associated with functional limitation, psychological distress and social withdrawal. OHIP-14 mean scores typically reflected a two to four-fold reduction in wellbeing compared to population norms, with the greatest burden in Hypermobile EDS (hEDS) and periodontal EDS (pEDS). Qualitative findings further identified stigma, diagnostic delay and anxiety as additional determinants of impaired OHRQoL. Despite limitations including small sample sizes, cross-sectional design and self-reported diagnoses, thematic convergence suggests a genuine negative association. This review highlights the need for earlier recognition, multidisciplinary management and future research with more robust study designs.