Table 2.

Systemic Criteria for Hypermobile Ehlers Danlos

1.	Unusually soft or velvety skin  This should be assessed on the volar aspect of the forearm. Skin softness and texture is subjective but is marked in patients with hEDS. A high threshold for positivity is recommended
2.	Mild skin hyperextensibility  Skin extensibility can be affected by location and extrinsic factors such as sun exposure and weight fluctuations. This should be measured in the middle of the volar surface of the non-dominant forearm. Skin should stretch >1.5cm to be considered positive. Stretch >2cm should prompt consideration for alternative diagnosis such as classic EDS or classic-like EDS
3.	Unexplained striae in adolescents, men, or prepubertal women without a history of significant gain or loss of body fat or weight
4.	Bilateral piezogenic papules of the heel
5.	Recurrent or multiple abdominal hernia(s) (e.g., umbilical, inguinal, crural)  Congenital umbilical hernias are common particularly in preterm infants and are generally not counted towards this criterion unless they recur
6.	Atrophic scarring involving at least two sites without papyraceous or hemosideric scars  Atrophic scars are typically wide and sunken due to impaired wound healing and subsequent dermal atrophy. Linear scars are more reliable than elliptical scars (such as from shave biopsies) which are more difficult to assess without knowing the size of the original wound. Scars that result from multiple incisions, wound infections, or inflammatory conditions should not be considered.
7.	Pelvic floor, rectal, and/or uterine prolapse in children, men or nulliparous women without a history of morbid obesity or other known predisposing medical conditions  Pregnancy, morbid obesity, and other conditions such as chronic constipation predispose patients to pelvic organ prolapse. This criterion should be considered positive in the absence of those predisposing conditions.
8.	Dental crowding and high or narrow palate  This is a subjective measure but history of extensive orthodontia, tooth extractions, or palate expansion suggest the presence of significant crowding
9.	Arachnodactyly, as defined by a bilateral positive wrist (Steinberg) sign and/or bilateral positive thumb (Walker) sign.  Wrist sign is only considered positive if the tip of the thumb overlaps the entire nail of the pinky. Thumb sign is only considered positive if the entire distal phalanx of the adducted thumb extends beyond the ulnar border of the palm.
10.	Arm span-to-height ≥1.05
11.	Mitral valve prolapse (MVP) mild or greater based on strict echocardiographic criteria
12.	Aortic root dilatation with Z-score > +2