Subtype
Classical-like Ehlers-Danlos syndrome (clEDS)
Recessive classical-like presentation caused by tenascin-X deficiency; distinguished from cEDS by absent atrophic scarring and recessive inheritance.
| Classification | 2017 International Classification |
|---|---|
| Inheritance | autosomal recessive |
| Genes | TNXB |
| Genetic status | confirmed genes |
| Prevalence | Rare; exact prevalence unknown |
| Distinguishing features | Classical-like skin features without atrophic scarring; TNXB-related (tenascin-X deficiency) |
| Last checked | 2026-09-16 |
Records about clEDS
Pain and joint instability lead the symptoms reported at a German EDS clinic
findingevidence: probableRauterberg and colleagues found that pain was the complaint most often reported to severely affect daily activities at a German EDS clinic. Their 2025 paper surveyed 99 adults attending University Hospital Cologne, including 80 with hEDS or HSD, 16 with classical EDS, and three with classical-like EDS. Of the 98 who answered the daily-activity question, 59 reported musculoskeletal pain (60.2%), 38 joint instability (38.8%), 36 other orthopedic problems (36.7%), 35 digestive complaints (35.7%), and 32 fatigue (32.7%). Respondents could name more than one complaint. These figures describe one specialist clinic.
Fatigue severely affected daily activities for one-third of a German clinic sample
findingevidence: probableRauterberg and colleagues found that 32 of 98 adults at a German EDS clinic reported fatigue severely affecting daily activities (32.7%). The 2025 study included people with hEDS, HSD, classical EDS, and classical-like EDS. Within the hEDS/HSD group, 27 of 80 respondents reported this level of fatigue (33.8%). Fatigue is not one of the 2017 hEDS diagnostic criteria.
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
- Older hypermobility-type studies use different diagnostic criteria
- 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