Ehlers-Danlos syndromes represents another set of systemic conditions associated with an increased risk of TMJ disorders and other chronic pain conditions (De Coster et al., 2005; Chopra et al., 2017; Mitakides and Tinkle, 2017). Chronic pain is highly prevalent in this syndrome, including both regional and widespread pain and TMJ-related pain has been reported in up to 71% of patients with Ehlers-Danlos syndromes (De Coster et al., 2005).
Symptoms of TMJ disorders in this syndrome are generally attributed to joint hypermobility and the resultant instability of the mandible during masticatory function as well as during maximal opening, which leads to protective muscle contraction and subsequent further problems in functioning. Previous findings have demonstrated associations between joint hypermobility and TMJ symptoms among individuals without Ehlers-Danlos (Perrini et al., 1997; Ogren et al., 2012). However, pain in the syndrome has also been associated with other mechanisms, including neuropathic features and signs of central sensitization (Syx et al., 2017; Benistan and Martinez, 2019), as well as myofascial pain due to protective muscle contraction. (NAM, page 3-20)
What is Ehlers-Danlos?
Ehlers-Danlos syndromes are a group of disorders that affect connective tissues supporting the skin, bones, blood vessels and many other organs and tissues. Defects in connective tissues cause the signs and symptoms of these conditions, which range from mildly loose joints to life-threatening complications. Features shared by many types include joint hypermobility and soft, velvety skin that is highly elastic (stretchy) and bruises easily. Mutations in a variety of genes may lead to EDS; however, the underlying genetic cause in some families is unknown. Depending on the subtype, EDS may be inherited in an autosomal dominant or an autosomal recessive manner. There is no specific cure for EDS. The treatment and management is focused on preventing serious complications and relieving associated signs and symptoms.