There are 13 types of Ehlers-Danlos syndrome, and most are rare, according to the NHS. They differ by gene, by whether skin, joints or blood vessels are affected most, and by risk. Vascular EDS can cause blood vessels and organs to split open, leading to life-threatening bleeding. Only a clinician can confirm your type.
Think of connective tissue as the stitching that holds a garment together. The type tells you which seams are weak. It doesn’t tell you how the person wearing the garment feels on a Tuesday afternoon, and I care about that second question as much as the first.
How many types of EDS are there?
There are 13 recognized types. Cleveland Clinic says healthcare providers classify EDS into 13 types based on where it affects you and the symptoms you experience. The NHS adds that most of those 13 are rare.
The number comes from the 2017 international classification. The EDS Clinic describes that framework as integrating molecular insights and clinical criteria to better reflect the diverse presentations of EDS. Before 2017 the types were grouped differently, which is why older articles and older diagnoses use names you may not recognize now.
All of them involve connective tissue, the material that supports skin, joints, blood vessels and organs. According to Cleveland Clinic, EDS makes your body produce weak collagen, so your connective tissue can’t support you as it should. How that shows up depends heavily on the type.
What are the three main types of EDS?
No official classification names three main types. When people say it, they usually mean hypermobile, classical and vascular, because those tend to be described first. Cleveland Clinic says five of the 13 are the most common, so "three" is shorthand and not a medical category.
What matters more is what each type risks. Hypermobile EDS mostly affects joints. Classical EDS affects skin more. Vascular EDS affects blood vessels and organs.
How do the 13 types of EDS compare?
Five types have a clear one-line difference you can compare side by side.
| Type | Abbreviation | What stands out | Gene involved |
|---|---|---|---|
| Hypermobile | hEDS | Joint hypermobility is the central feature. A clinician diagnoses it from history and exam. | No gene identified |
| Classical | cEDS | Stretchy skin, atrophic scarring, generalized joint hypermobility | COL5A1 and COL5A2 |
| Classical-like | clEDS | Hypermobility and stretchy skin, but without the tendency for atrophic scarring | TNXB |
| Cardiac-valvular | cvEDS | Severe heart valve disease alongside joint and skin features | COL1A2 |
| Vascular | vEDS | Arterial rupture risk, thin translucent skin | COL3A1 |
The EDS Clinic supplies the genes and features for classical, cardiac-valvular and vascular EDS. DermNet supplies the classical-like gene and notes that the gene behind hEDS is unknown. Sequencing.com adds the point about scarring in classical-like EDS.
The hEDS row is the odd one out. With no identified gene, no blood test confirms it. The other four rows each have a named gene.
DermNet also lists COL1A1 and COL1A2 for arthrochalasia EDS (aEDS) and ADAMTS2 for dermatosparaxis EDS (dEDS).
Telling those apart takes gene testing and a genetics service, not a web page.
What makes hypermobile EDS different from the rest?
Hypermobile EDS is diagnosed by a clinician from your history and a physical exam, because no gene has been found for it. The NHS notes that classical EDS is less common than hypermobile EDS, and that joint hypermobility on its own is relatively common, affecting around 1 in 30 people.
That gap between common hypermobility and an actual diagnosis explains a lot of the confusion. Plenty of hypermobile people never get an EDS diagnosis, and only a specialist evaluation can tell the difference. We cover the diagnostic side in Current Advancements in Diagnosing Ehlers-Danlos Syndrome and the care side in Hypermobile EDS (hEDS) Effective Treatments.
Why does vascular EDS get separate attention?
Because of what it can do to blood vessels. The EDS Clinic describes it as associated with COL3A1 mutations, defined by arterial rupture risk, thin translucent skin, and facial characteristics such as a pinched nose.
The NHS says this type affects the blood vessels and internal organs, which can cause them to split open and lead to life-threatening bleeding. DermNet adds that the signs can be subtle or absent in some people, which makes vEDS hard to diagnose.
So a suspicion of a rare type shouldn’t stay a guess. The NHS says that if there’s a possibility you have one of the rare types, the GP can refer you to a local genetics service for an assessment.
Can you inherit EDS, and is it always inherited?
Not always. The NHS explains that, depending on the type, the faulty gene may have been inherited from one parent or both. Sometimes the gene change isn’t inherited and occurs in the person for the first time.
DermNet reports that 50% of patients diagnosed with vEDS have a de novo mutation, meaning neither parent is affected. A clean family history doesn’t rule a type out, and your diagnosis doesn’t automatically point to a parent.
Why should a subtype only be named once it’s confirmed?
Because the types overlap, and the stakes differ sharply between them. A symptom list that fits hEDS can also fit other connective tissue conditions, and the risks of one type aren’t the risks of another.
It also protects you from a quieter problem. Reading a list of 13 types, it’s easy to match your symptoms to one and decide that’s you. A list can’t examine you. A subtype should come from a clinician who has.
What is changing in the EDS classification?
The diagnostic criteria are being revised. Sequencing.com, a genetic testing company, says an update is coming in December 2026, the first for EDS and hypermobility spectrum disorders since 2017.
Nobody can say yet what the new criteria will contain, so be careful with any claim about them. If you were diagnosed under the 2017 criteria, keep your records and ask your clinician at your next visit whether anything needs revisiting.
What mistakes do people make when reading about EDS types?
The most common one is picking a subtype from a symptom list. It feels like progress, but a wrong label can send you to the wrong specialist, or reassure you about a risk that deserves a real check.
Another is treating hypermobility and EDS as the same thing. Hypermobility is common and often causes no trouble. EDS is a diagnosis, and a clinician has to make it.
People also assume a negative genetic test rules out EDS. For hEDS there’s no gene to test for, so the route is a clinical evaluation. For the rarer types, a genetics service can tell you which test fits.
And many assume the type tells the whole story. It doesn’t. Two people with the same type can have very different days, so the type is where care starts, and your own pattern still has to be heard in full.
Where does the nervous system fit in?
EDS is a connective tissue condition, yet a subtype name doesn’t explain every symptom you live with. At The Spero Clinic, we look at how the central and autonomic nervous system is working alongside your diagnosis.
That doesn’t replace a genetics evaluation or medical care. It’s a different question, asked in addition. If you’d like to see how we think about it, read What Is Ehlers-Danlos Syndrome (EDS)? and Understanding Ehlers-Danlos Syndrome (EDS). My book on treating EDS and POTS covers it at length.
FAQ
What are the three main types of EDS?
No official list names three. People usually mean hypermobile, classical and vascular. Cleveland Clinic says five of the 13 types are the most common, and only a clinician can confirm which one is yours.
What do people with hypermobile EDS look like?
There’s no single look. Hypermobile EDS is diagnosed from history and a physical exam, and many hypermobile people never receive the diagnosis. Appearance can’t confirm a type, so a clinician’s evaluation is the only reliable guide.
Which parent do you inherit EDS from?
It depends on the type. The NHS says the faulty gene may come from one parent or both, and sometimes it occurs for the first time in the person. A genetics service can tell you which pattern applies to your type.
One thing to do today
Write down the exact wording of your diagnosis, who gave it, and the date. If a clinician said "hypermobility," "EDS," or a specific type, copy it word for word, then add your symptoms in the order they appeared. Bring the page to your next appointment and ask whether a genetics referral makes sense for you.
This article is general education. It doesn’t replace evaluation by a qualified clinician or emergency care.
