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Subtypes of Ehlers-Danlos syndrome

Created 2003.

Classical Type I (gravis)
  • 43% of all cases
  • Show all EDS features including mitral valve prolapse
  Type II (mitis)
  • 34% of all cases
  • Mild but similar to type I
Hypermobility Type III
  • 10% of all cases
  • Skin unaffected
  • Extensive hypermobility of joints
Vascular Type IV
  • 6% of all cases
  • Skin is noticeably translucent with visible veins but is not elastic
  • Joints are unaffected
  • Complications common and sudden death may occur at any time from large vessel rupture
Kyphoscoliosis or ocular Type VI
  • 2% of all cases
  • Eye involvement
  • Scoliosis common (curved spine)
Arthrochalasia Type VII A and B
  • 3% of all cases
  • Noticeable joint hypermobility but less skin involvement
  • Spontaneous joint dislocation common
Dermatosparaxis Type VII C
Other Type V
  • 5%
  • Extensive skin hyperelasticity with splitting, bruising and scarring
  Type VIII (periodontal)
  • Hyperelastic fragile skin
  • Severe periodontitis (gum disease)
  Type IX
  • Skeletal defects, characteristic bony horn-like overgrowths on back part of the skull
  Type X (fibronectin deficiency)
  • Both rare
  • These 2 types are so similar that they possibly should be classified together
  Type XI (benign hypermobile joint syndrome)

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Watch Dr Amanda Oakley presenting 'The History Of DermNet NZ' at The International Society Of Teledermatology.

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