Regarding HSD vs hEDS

This page and topic are currently somewhat controversial, and have been ever since the inception (invention) of the Hypermobility Spectrum Disorders in 2017 by those claiming the power to decide these things.

These powers chose a stereotyped and limited subset of criteria and signs to come up with the diagnostic criteria for hypermobile type EDS (hEDS) designed to support research into the underlying gene or genes, rather than to aid patient care and quality of life. hEDS is considered the most common type of EDS by far, but prevalence rates are disputed as more robust epidemiology is lacking.

graphic of an iceberg with labels of EDS above the water line, and HSD below
See the rest of the iceberg!

The current ( as of March 2024 still) 2017 hEDS diagnostic criteria skew toward thinner white bodies with more apparent Marfanoid habitus. They are also biased toward people who do not have additional auto-immune diseases (which we frequently do), as this added condition is then literally held against the patient, dismissing their recurrent joint dislocations or frank instability (Feature C, Criterion 2) in Criterion 3.

And they further discriminate by requiring patients to have biological family available to help make the diagnosis in those with co-occurring autoimmune diseases. Not everyone has access to their families of origin, either because they were adopted, abused or abandoned, and some may never. Nor are all biological families cooperative. And many of our kin are actually homeless, lacking any access to proper medical care.

There really is no clean, clear distinction between hEDS and HSD yet. HSD has truly just been the newest “catch-all bin”, or diagnosis of exclusion – to hEDS. Which itself was a prior catch-all bin or diagnosis of exclusion of all other forms of Ehlers-Danlos syndromes and similar Connective Tissue Diseases/Disorders (CTDs) like Marfan Syndrome, Stickler Syndrome, Osteogenesis Imperfecta and Loeys-Dietz Syndrome. These all share similar issues of hypermobility and weak connective tissues and many attendant issues (sequalae, or knock-on effects of weak tissues), but from different underlying molecular causes than ascribed to the forms of EDS.

In other words, you are supposed to thoroughly rule out not just all other rare forms of EDS (of which there are now 13 official, and 14 unofficial and counting), but also all other similar appearing CTDs (as mentioned above) in order to diagnose hypermobile type EDS. (Not always done.)

But if you run the hypermobile EDS criteria checklist, and someone doesn’t quite pass despite having many signs and suffering (else why are they at a doctor’s office), and you have thoroughly ruled out all other possibilities mentioned above, and they still have pain and issues with their tissues and signs not caused by an autoimmune disease, then, you can diagnose someone with a form of the Hypermobility Spectrum Disorders as a final resort.

There are no actual criteria for the HSDs yet though this is under discussion again in 2023. Nor is there an ICD-10 code yet.

The following are articles that either help describe, diagnose or recognize or define either hypermobile type EDS (hEDS) or the Hypermobility Spectrum Disorders. As well as describe how they differ, or if they really do. And will include the latest research into hEDS and or HSD.

See also The RCCX Theory (yes, technically a hypothesis yet) on a possible genetic complex behind hEDS/HSD. (I gave this its own page as its such a complex topic that has no bench research backing it yet either.) Updated May 18, 2024

Hypotheses on causes of hEDS or HSD and how they may differ or not

Discussions of Prevalence of hEDS or HSD