BACKGROUND: The Ehlers-Danlos syndromes (EDS) comprise a heterogenous group of 
heritable disorders of connective tissue, characterized by joint hypermobility, 
skin hyperextensibility and tissue fragility. Most EDS types are caused by 
mutations in genes encoding different types of collagen or enzymes, essential 
for normal processing of collagen.
METHODS: Oral health was assessed in 31 subjects with EDS (16 with hypermobility 
EDS, nine with classical EDS and six with vascular EDS), including signs and 
symptoms of temporomandibular disorders (TMD), alterations of dental hard 
tissues, oral mucosa and periodontium, and was compared with matched controls.
RESULTS: All EDS subjects were symptomatic for TMD and reported recurrent 
temporomandibular joint (TMJ) dislocations. Abnormal pulp shape (13%) and pulp 
calcification (78%) were observed in subjects affected with classical EDS. 
Caries experience was higher in EDS compared with controls and was related to 
poor oral hygiene, influenced by increased mucosal fragility and restraint of 
(wrist) joint mobility. The overall periodontal status in EDS was poor, with 62% 
of EDS subjects presenting high periodontal treatment needs (community 
periodontal index for treatment need, CPITN = II).
CONCLUSION: Oral health may be severely compromised in EDS as a result of 
specific alterations of collagen in orofacial structures. When considering 
dental treatment in EDS, a number of tissue responses (mucosa, periodontium, 
pulp) and precautions (TMJ dislocation) should be anticipated.