Subtype
Vascular Ehlers-Danlos syndrome (vEDS)
Vascular EDS is caused by COL3A1 variants. Arteries, the bowel, and the uterus can tear or rupture, and that risk shapes how surgery and anesthesia are approached.
Not medical advice. This page summarizes research and does not recommend any course of care.
| Classification | 2017 International Classification |
|---|---|
| Inheritance | autosomal dominant |
| Genes | COL3A1 |
| Genetic status | confirmed genes |
| Villefranche (1997) | EDS type IV |
| Prevalence | Rare |
| Distinguishing features | Arterial and organ rupture risk, thin translucent skin, characteristic facial features; the highest-acuity EDS type |
| Last checked | 2026-09-16 |
Records about vEDS
Georg Sack described a form with arterial rupture and organ fragility that was later formalized as the vascular type. This is the historical root of vEDS recognition.
Barabas establishes heterogeneity and the arterial complication profile
eventevidence: historical recordA. P. Barabas demonstrated that EDS was heterogeneous and delineated the form with arterial complications, the direct ancestor of the vascular subtype (type IV in Villefranche, vEDS today).
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