Ehlers-Danlos syndrome (EDS) is the name given for 6 types of connective tissue disorders. While the prevalence of this disease is small, it is seen on every continent and affects both sexes and all races. The various types of EDS are reviewed with reference given to both the older Berlin nosology and the newer Villefranche nosology. Phenotypes of EDS vary depending upon which type of collagen is altered, leading the practitioner to the diagnosis before biochemical confirmation is obtained. In this regard, because collagen is present throughout the head and neck, oral and maxillofacial manifestations of the disease are discussed and are readily noticeable to the astute dentist. Specialists in several fields of dentistry are made aware of the complications EDS can pose on treatment, healing, and follow-up care.

Ehlers-Danlos syndrome : classifications, oral manifestations, and dental considerations

Aim: Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders, characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Periodontal EDS (pEDS) ...

Periodontal manifestations of Ehlers–Danlos syndromes: A systematic review

Clinical, genetic, and histologic features of the trichoonychodental (TOD) syndrome

Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and variable tissue fragility. However, there are limited published data on the dental manifestations of EDS. This review systematically assessed the spectrum of published dental anomalies in various types of EDS. Twenty-four individual case reports/series and 3 longer case-control studies, reporting on a total of 84 individuals with a clinical diagnosis of EDS, were included in the data analysis. The main dental features listed in classical EDS were pulp calcification and localized root hypoplasia. Common dental abnormalities observed in vascular EDS were pulp shape modifications (52.2%), exceeding root length (34.8%), and molar root fusion (47.8%). Dentinogenesis imperfecta is a consistent finding in osteogenesis imperfecta/EDS overlap syndrome. Data on dental manifestations in other types of EDS are both rare and generally inconclusive.

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Dental Manifestations of Ehlers-Danlos Syndromes: A Systematic Review.

A 7-year-old girl is described with Ehlers-Danlos syndrome Type VIII. Symptoms mainly consisted of rapid breakdown of the periodontal tissues. The gingiva exhibited the presence of peculiar previously unidentified cell-poor masses resembling fibrin by their staining properties. Possibly, this histologic feature may be a diagnostic aid in Ehlers-Danlos syndrome Type VIII.

Gingival fibrinoid deposits in Ehlers-Danlos syndrome

Up to 1955 the literature reports approximately 100 cases of Ehlers—Danlos Syndrome The characteristic symptoms are:Extreme elasticity of skin and joints, easily traumatised skin and blood vessels producing disfiguring scars Histological examination shows fewer collagen fibrils than usual The elastic fibres are not spun round the collagen bundles, and the collagen does not show the usual three-dimensional net pattern Electron-microscopy does not show changes in the molecular or chemical configuration of the collagen fibresPrevious reports on this syndrome do not concentrate to any great extent on the teeth The case described in this study presented grave dental disturbances of calcification Root formation ended prematurely in the premolars and the upper and lower central incisors Pulp stones and calcifications in the pulp cavities were found, and root formation of the lower central incisors was incomplete and atypicalOne must assume that the collagen configuration in the jaw areas was atypical, a

[dental conditions in a patient with ehlers-danlos syndrome].

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[dental conditions in a patient with ehlers-danlos syndrome].