TABLE 1.
Classification of EDS subtypes over time
| 1988, Berlin | 1998, Villefranchea | 2017, International |
|---|---|---|
| Type I, Gravis | ||
| Classical type | Classical EDS (cEDS) | |
| Type II, Mitis | ||
| Type III | Hypermobility type | Hypermobile EDS (hEDS) Hypermobility spectrum disordersb (HSD) |
| Type IV (A, B, C, D) | vascular type | Vascular EDS (vEDS) |
| Type VI | Kyphoscoliosis type | Kyphoscoliotic EDS (kEDS) |
| Type VII | ||
| A | Arthrochalasia type | Arthrochalasia EDS (aEDS) |
| B | ||
| C | Dermatospraxis type | Dermatospraxis EDS (dEDS) |
| Type VIII | Periodontitis type | Peridontal EDS (pEDS) |
| Type V | X-linked type | |
| Type IX | Occipital horn syndrome | |
| Type X | Fibronectin-deficient type |
No longer part of EDS |
| Type XI | Familial hypermobility syndrome | |
| Progeroid type | Spondylodysplastic EDS (spEDS) Brittle cornea syndrome (BCS) Cardiac-valvular EDS (cvEDS) Classical-like EDS (clEDS) Myopathic EDS (mEDS) Musculocontractural EDS (mcEDS) AEBP1 variant EDSc |
|
a
Often referred to as Villefranche, 1997, however not published until 1998.
b
HSD may include diagnoses such as Joint hypermobility (JH or JHM), Joint hypermobility syndrome (JHS), Benign joint hypermobility (bJHS), and generalized joint hypermobility (GJH).
c
Not part of the 2017 International classification; was published separately18 in 2018.