Ehlers-Danlos Syndrome (Classical type) Criteria
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Major Criteria
☐Skin hyperextensibility
☐Widened atrophic scars
☐Joint hypermobility
Minor Criteria
☐Smooth velvety skin
☐Molluscoid pseudo-tumors
☐Subcutaneous spheroids
☐Joint hypermobility complications (sprains, dislocations, subluxations, pes planus, etc)
☐Hypotonia or delayed gross motor development
☐Easy bruising
☐Manifestations of tissue extensibility and fragility (hiatal hernia, anal prolapse in childhood, cervical insufficiency)
☐Surgical complications (post-operative hernias)
☐Family history of this condition (is heritable and family history should be present)
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Ehlers-Danlos Syndrome (Classical type) Criteria

Classical Ehlers-Danlos Syndrome (EDS) is the most common form of EDS and ranges from being mild to severe. It is inherited in an autosomal dominant pattern. Clinical manifestations are marked skin hyperextensibility, widened atrophic scars and joint hypermobility. In addition to tissue fragility, and easy bruisability, hypotonia and delay of gross motor development may also occur.

Clinical diagnostic criteria requires 1 Major criteria to be met. Even though Minor criteria are listed; none are required for clinical diagnosis.

Major Criteria

  • Skin hyperextensibility
  • Widened atrophic scars
  • Joint hypermobility

Minor Criteria

  • Smooth velvety skin
  • Molluscoid pseudo-tumors
  • Subcutaneous spheroids
  • Joint hypermobility complications (sprains, dislocations, subluxations, pes planus, etc)
  • Hypotonia or delayed gross motor development
  • Easy bruising
  • Manifestations of tissue extensibility and fragility (hiatal hernia, anal prolapse in childhood, cervical insufficiency)
  • Surgical complications (post-operative hernias)
  • Family history of this condition (is heritable and family history should be present)

References:

  1. Beighton P, De Paepe A, Steinmann B, et al. Ehlers-Danlos syndromes: Revised nosology, Villefranche, 1997. Am J Med Genet . 1998;:77(1):31-7.
  2. Horii KA. Denouement and Discussion: Ehlers-Danlos Syndrome, Classical Type. Arch Pediatr Adolesc Med. 2006;160(7):706.