Premium
Oral manifestations of Ehlers‐Danlos syndromes
Author(s) -
Lepperdinger Ulrike,
Zschocke Johannes,
KapfererSeebacher Ines
Publication year - 2021
Publication title -
american journal of medical genetics part c: seminars in medical genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.419
H-Index - 101
eISSN - 1552-4876
pISSN - 1552-4868
DOI - 10.1002/ajmg.c.31941
Subject(s) - oral cavity , ehlers–danlos syndrome , standardization , periodontitis , medicine , connective tissue , oral health , differential diagnosis , pathology , dentistry , intensive care medicine , computer science , operating system
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.