Litcius/Paper detail

Oral manifestations of <scp>Ehlers‐Danlos</scp> syndromes

Ulrike Lepperdinger, Johannes Zschocke, Ines Kapferer‐Seebacher

2021American Journal of Medical Genetics Part C Seminars in Medical Genetics17 citationsDOIOpen Access PDF

Abstract

Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders. Patients with EDS exhibit distinct pathologies of the teeth and the oral cavity. Here, we summarize the current knowledge in the various EDS types, in particular regarding severe changes in oral health-related quality of life, the differential emergence of periodontitis, characteristic yet highly cumbersome dental manifestations, apparent anomalies of oral soft tissues, and relevant issues related to dental implantology. Resolution of remaining open questions will primarily rely on the standardization of diagnostic criteria. Clinical centers that specialize on this rare pathology need to apply congruent approaches for exact characterization of clinical features in conjunction with genetic validation that should be reached without exception in all patients and relevant family members.

Topics & Concepts

Oral cavityEhlers–Danlos syndromeStandardizationPeriodontitisMedicineConnective tissueOral healthDifferential diagnosisPathologyDentistryIntensive care medicineComputer scienceOperating systemConnective tissue disorders researchPeptidase Inhibition and AnalysisProteoglycans and glycosaminoglycans research
Oral manifestations of <scp>Ehlers‐Danlos</scp> syndromes | Litcius