Oral health in prevalent types of Ehlers-Danlos syndromes.
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).


CONCLUSIONS
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.
De Coster
Peter J
PJ
Department of Paediatric Dentistry, Centre for Special Care, Paecamed Research, Ghent University, B-9000 Ghent, Belgium.
Martens
Luc C
LC
De Paepe
Anne
A
D000293:Adolescent; D000328:Adult; D000704:Analysis of Variance; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D017742:Dental Care for Chronically Ill; D003731:Dental Caries / Q000209:etiology*; D003782:Dental Pulp / Q000002:abnormalities; D003784:Dental Pulp Calcification / Q000209:etiology*; D004535:Ehlers-Danlos Syndrome / Q000145:classification / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010510:Periodontal Diseases / Q000209:etiology*; D010512:Periodontal Index; D012044:Regression Analysis; D018709:Statistics, Nonparametric; D013705:Temporomandibular Joint Disorders / Q000209:etiology*