Subtype
Classical Ehlers-Danlos syndrome (cEDS)
The archetypal skin-predominant type. Molecular confirmation via COL5A1/COL5A2 identifies the large majority of cases meeting clinical criteria.
| Classification | 2017 International Classification |
|---|---|
| Inheritance | autosomal dominant |
| Genes | COL5A1, COL5A2 |
| Genetic status | confirmed genes |
| Villefranche (1997) | EDS types I and II |
| Prevalence | Estimated around 1 in 20,000 to 1 in 40,000 |
| Distinguishing features | Skin hyperextensibility with widened atrophic scars, generalized joint hypermobility, easy bruising |
| Last checked | 2026-09-16 |
Records about cEDS
Subluxation, braces, taping, and joint-stabilization self-management
patient-reported patternevidence: community signalMuch of the practical discussion in patient communities is about joints that partly slip out of place (subluxation) rather than fully dislocate. Members swap taping, bracing, and stabilizing techniques, often for subluxations no doctor has confirmed. Clinical literature treats subluxation as close to, but not itself, a diagnostic criterion; the community discussion is about day-to-day management rather than diagnosis.
Craniocervical instability and tethered cord in hEDS: contested
findingevidence: contestedindependent evidence agreesCraniocervical instability (CCI) and tethered cord come up often in patient communities, and cervical instability was one of the diagnoses participants most often endorsed as accurate in a Global Registry survey of people with hEDS. Controlled evidence is limited, and surgery for these conditions carries real risk.
Records that apply to every EDS type
- Medication, dental, and procedural caution knowledge
- The 2017 classification papers cover symptoms beyond joints and skin
- Twelve subtypes require molecular confirmation; hEDS remains clinical
- Criteria-era drift makes old and new EDS literature non-equivalent
- The 2017 classification groups EDS genes by the process they affect
- Beighton score published for epidemiological joint-mobility assessment
- Berlin nosology expands EDS to eleven numbered types
- Villefranche nosology consolidates to six types
- 2017 International Classification defines thirteen subtypes
- Local anesthetic resistance in EDS: patient reports, two surveys, and a 2026 trial
- A 2017 review of pain management in EDS
- Surgical and tissue-handling caution
- Diagnosing EDS and HSD often takes years
- The DICE Global Registry collects patient-reported data on EDS and HSD
- EDS ECHO teaches clinicians and community members about EDS
- The ClinicalTrials.gov EDS portfolio
- Orphanet and GARD keep reference records for rare diseases, including EDS