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)

Email Newsletter

Would you like to receive our dermatology updates by email?


Submit your images

We're seeking high-quality photos of skin diseases.  


Machine diagnosis

Watch DermNet's proposal to create a 'Skin Disease Image Recognition Tool' - winner of the 2017 'Clinicians' Challenge Active Award' by the NZ Ministry of Health and HiNZ.

Subscribe to our mailing list

* indicates required
DermNet NZ Newsletter