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Ehlers-Danlos Syndrome and the Affects on the Oral Cavity Courtney S. CampbellTever JeffersonLamar Institute of TechnologyPatient education is one of the main focuses and duties of a Dental Hygienist. Dental Hygienists have the opportunity to inform patients of possible diseases or disorders and educate them on how to care for their oral cavity with such diseases. The following paragraphs will discuss a rare disease called Ehlers-Danlos Syndrome (EDS) and it’s oral implications. Being informed about Ehlers-Danlos Syndrome will provide the clinician with adequate knowledge on how to treat these individuals in the dental office and provide the patient with the best optimum care. Ehlers-Danlos Syndrome (EDS) is a connective tissue disorder caused by a defect in the type I, III, and IV collagen gene (Trudgian & Trotman 2011). EDS affects all races and ethnic groups without sex predominance, but is only seen in approximately one in 5000 individuals (Trudgian & Trotman 2011). EDS was first discovered in 1892, but was not named Ehlers-Danlos Syndrome until 1936 by British physician, Frederick Parkes-Webber (Trudgian & Trotman 2011). There are six main forms of EDS; the most common form is classic EDS (Trudgian & Trotman 2011). The majority of EDS cases are diagnosed on the clinical findings of joint pain, easy bruising, dystrophic scarring, delayed healing and joint hypermobility (Perez, Al-Shammari, Giannobile, Wang 2002). However, EDS symptoms may involve other characteristics and have a wide variety of severity. The periodontal ligaments found in the oral cavity are made up of collagen; therefore defects in collagen affect periodontal tissue in the oral cavity. One oral characteristic found in individuals with classic EDS is early onset of periodontitis. Periodontal disease is the irreversible destruction of periodontal ligaments and alveolar bone (Mataix, Banuls, Munoz, Bermejo & Climent 2008). Periodontal disease is usually seen in patients with very poor oral hygiene, heavy calculus build up, and old age. As seen in a case study by Perez, Al-Shammari, Giannobile and Wang (2002) an intraoral examination of patients with EDS displayed “good oral hygiene with minimal plaque with mild to moderate subginigival calculus”(Perez et al., 2002 pg 565). These findings do not coincide with the gingival appearance seen in EDS patients of “thin translucent alveolar mucosa with swollen receded attached gingiva mainly on maxillary and mandibular premolars”(Perez et al., 2002 pg 565). It has been researched that the defect in collagen compromises the integrity of the periodontal ligaments, which is why an early onset of periodontal disease occurs in EDS patients. Without aggressive periodontal treatment the early onset of periodontitis can lead to early loss of teeth. Many EDS patients also have extreme joint hypermobility which, often leads to chronic dislocation of the temporomandibular (jaw) joint. TMJ problems cause patients to grind or clench their teeth leading to the attrition or the wearing down of the teeth. Another abnormality in the oral cavity seen in EDS patients is the absence of the lingual and inferior labial frenula (Machet, et. al., 2010). Absence of frenum is not a disability and doesn’t cause harm to the patient, but can be used by the clinician as a diagnostic tool to identify EDS. Although the symptoms of EDS can be treated there is currently no specified overall treatment for individuals with EDS. In individuals that have periodontitis “early recognition and treatment of periodontal disease may improve their long term prognosis” (Mataix et al., 2008 pg 829). These individuals should aim to control microbial levels via meticulous oral hygiene. Research by Perez, Al-Shammari, Giannobile and Wang (2002) has shown that regular scheduled scaling and root planning have shown to halt the progression of periodontal disease and improve health of surrounding gingiva. In cases where gingiva is severely swollen antibiotics may be prescribed or surgery may be deemed necessary to improve oral health. Orthodontic apparatuses may also be used as a form of treatment depending on the integrity of the periodontal ligaments (Mataix et al., 2008). For EDS patients with pain due to hypermobility “ non-steroidal anti-inflammatory drugs (NSAIDs) seem to work significantly, especially when the pain has an inflammatory component”(Prokop, Adamczyk, & Krajnik 2010). However it is important to keep in mind that “long term use bears a great risk of complications to the gastrointestinal tract, kidneys, and circulatory system”(Prokop et. al., 2010). The use of an opioid for pain is not suggested due to the risk of addiction. In conclusion, early onset of periodontal disease can be used to diagnosis an underlying systemic condition such as EDS (Reinstein et. al., 2012). Although there is no clinical treatment for EDS, with suitable understanding of the disease and appropriate precautions Dental Hygienist can still adequately treat these patients. Education is the main key in dealing with patients with EDS it is important to educate the individual about overall wound care to reduce chances of bacterial infection and maintaining regular dental visit to address oral heath needs. References Machet, L., Hüttenberger, B., Georgesco, G., Doré, C., Jamet, F., Bonnin-Goga, B., & ...Vaillant, L. (2010). Absence of Inferior Labial and Lingual Erenula in Ehlers-Danlos Syndrome. American Journal Of Clinical Dermatology, 11(4), 269-273.Mataix, J., Ba?uls, J., Mu?oz, C., Bermejo, A., & Climent, J. (2008). Periodontal Ehlers–Danlos syndrome associated with type III and I collagen deficiencies. British Journal Of Dermatology, 158(4), 825-830. Perez, L., Al-Shammari, K., Giannobile, W., & Wang, H. (2002). Treatment of periodontal disease in a patient with Ehlers-Danlos syndrome. A case report and literature review. Journal Of Periodontology, 73(5), 564-570.Prokop, A., Adamczyk, A., & Krajnik, M. (2010). Chronic use of opioids in a patient with Ehlers-Danlos syndrome -- a case report. Advances In Palliative Medicine, 9(2), 53-59.Reinstein, Eyal, Celia Dawn Delozier, Ziv Simon, Serguei Bannykh, David L. Rimoin, and Cynthia J. Curry. "Ehlers-Danlos Syndrome Type VIII Is Clinically Heterogeneous Disorder Associated Primarily with Periodontal Disease, and Variable Connective Tissue Features." European Journal of Human Genetics 21.2 (2013): 233-36. Web. 24 Mar. 2015.Trudgian, J., & Trotman, S. (2011). Ehlers-Danlos syndrome and wound healing: injury in a collagen disorder. British Journal Of Nursing, 20S10-20. ................
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