Major diagnostic criteria should all be met to establish a diagnosis of EDS, hypermobility type:
Joint hypermobility, which is often confirmed by a score of five or more on the nine-point Beighton scale [Beighton et al 1973], although some individuals with objective joint laxity score fewer than five points. One point is scored for each of the following:
-Passive dorsiflexion of each fifth finger greater than 90°
-Passive apposition of each thumb to the flexor surface of the forearm
-Hyperextension of each elbow greater than 10°
-Hyperextension of each knee greater than 10°
-Ability to place the palms on the floor with the knees fully extended
-Soft skin with normal or only slightly increased extensibility.
-Soft skin is subjectively assessed, preferably in an area in which moisturizer has not been applied.
Skin hyperextensibility is assessed at a site lacking excess or loose skin and without evidence of prior trauma by gently pulling until resistance is met. An ideal location is the volar surface of the forearm, where the upper limit of normal extensibility is 1-1.5 cm. Extensor surfaces of joints have excess skin and should not be used.
Minor diagnostic criteria are supportive of but not sufficient to establish a diagnosis of EDS, hypermobility type:
-Positive family history of EDS, hypermobility type (or family history of joint laxity), without significant skin or soft tissue fragility, in a pattern consistent with autosomal dominant inheritance
-Recurrent joint dislocations or subluxations
-Chronic joint, limb, and/or back pain
-Easy bruising
-Functional bowel disorders (functional gastritis, irritable bowel syndrome)
-Neurally-mediated hypotension or postural orthostatic tachycardia
-High, narrow palate
-Dental crowding
The above list of symptoms is courtesy of the National Institute of Health: http://www.ncbi.nlm.nih.gov/books/NBK1279/
