Oral Manifestations of Ehlers Danlos Syndrome

Gadwa, M., C. Silk, P. Sitt, and B. Yousif

Purpose

Ehlers Danlos Syndrome (EDS) is the name given to over ten different phenotypes of an inherited disorder, all involving a genetic defect in collagen and connective tissue synthesis and structure. The purpose of this table clinic is to make dental professionals aware of this rare and intriguing disorder, specifically EDS Type VIII and its relationship to the oral cavity.

Summary

EDS can have many different clinical manifestations and levels of severity, depending on which type is diagnosed. Examples of clinical manifestations include joint hypermobility, skin extensibility, and scarring tendency. Overlap of clinical manifestations are common; one third of cases of patients with EDS do not exactly fit into a single type. Although rare, EDS Type VIII is of specific interest to dental professionals from its association to the destruction of the periodontium. It is considered the periodontal form due to the specific clinical feature of severe early-onset or aggressive periodontitis, which distinguishes EDS Type VIII from other forms. For patients with this type of EDS, periodontal involvement may appear as early as puberty and can lead to tooth loss or extensive mobility before the age of thirty, which may cause patients to become edentulous early in life. EDS Type VIII can also present more subtly, as in a case of a 48-year old Caucasian female who was misdiagnosed with persistent hyperplastic gingivitis, but in fact was later diagnosed with EDS Type VIII. EDS Type VIII can be a devastating disorder to both the patient and dental professional, and is best treated when diagnosed early.

Conclusion

Continuously gaining knowledge of various disorders, such as Ehlers-Danlos Syndrome, opens the practice of dental hygiene to better serve our patients. EDS Type VIII presents as yet another example of how periodontal involvement may lead to an underlying systemic condition, and as dental professionals we are many times the first to detect these conditions, regardless of how rare.