Ehlers Danlos syndrome in an edentulous patient with temporomandibular joint dislocation

Abstract

Background: Ehlers Danlos syndrome (EDS) is heterogeneous genetic syndrome involving connective tissue. It is characterized by clinical signs of skin hyper-extensibility, delayed wound healing with atrophic scarring, joint hypermobility, easy bruising and generalized connective tissue fragility. Oral cavity is not immune to these systemic effects. Case Presentation: A 40 year old Indian male patient reported to the Department of Oral Medicine and Radiology with chief complaint of clicking in front of ear and difficulty in closing the mouth properly as his jaw seems to get stuck sometimes. The problem started about two years back when patient underwent full mouth extraction due to mobility in all teeth. On general examination certain anomalous features were observed which included hyper-extensibility of fingers of the hands and cigarette paper scars on legs and foot. The patient was completely edentulous and could touch tip of tongue to the tip of nose. Panoramic and temporomandibular joint (TMJ) limitation views were taken and MRI of TMJ was advised. Based on history and clinical features; we came to diagnosis of TMJ dislocation and Ehlers Danlos syndrome. Patient was advised to avoid wide opening of the mouth. Since the medical condition of the patient was capricious for implants prosthesis, the patient was advised to use relined complete denture. Conclusion: Ehlers Danlos syndrome is a rare genetic disorder of connective tissue. Oral manifestation and dental consideration for such cases should be well evaluated and managed appropriately.