Understanding Ehlers-Danlos Syndrome

What Is EDS?

Ehlers-Danlos syndromes are a group of inherited connective tissue disorders. Connective tissue is the "scaffolding" of the body, giving structure and strength to skin, joints, blood vessels, and organs. In people with EDS, this tissue is more fragile or stretchy than usual, and that affects far more than the joints.

EDS is not one condition but a family of them, which is part of why it's so often misunderstood and missed.

Hypermobility vs. EDS vs. HSD

Joint hypermobility simply means joints move beyond the typical range. Plenty of people have it without any problems, and it's common in dancers, gymnasts, and children.

When hypermobility comes with pain, instability, fatigue, or other body-wide symptoms, it may fall under one of two diagnoses:

• Hypermobile EDS (hEDS): diagnosed when a person meets specific criteria set in 2017, including generalized joint hypermobility (often measured with the Beighton score), systemic signs of connective tissue involvement, a family history, and complications like chronic pain or recurrent dislocations, while other conditions are ruled out.

• Hypermobility Spectrum Disorders (HSD): a diagnosis for people who have symptomatic hypermobility but don't fully meet the hEDS criteria. HSD can be just as disabling and deserves the same care. It is not a "lesser" diagnosis.

The 13 Subtypes of EDS

The 2017 international classification recognizes 13 subtypes. Twelve have an identified genetic cause that can be confirmed with testing. The exception is hEDS, which is the most common type and has no known genetic marker yet, though research continues.

• Hypermobile (hEDS): joint instability, chronic pain, and fatigue, often alongside other body-wide symptoms

• Classical (cEDS): very stretchy, fragile skin, atrophic scarring, and joint hypermobility

• Classical-like (clEDS): resembles classical EDS but with different genetic causes and without the characteristic scarring

• Cardiac-valvular (cvEDS): severe heart valve problems alongside skin and joint features

• Vascular (vEDS): thin, translucent skin, easy bruising, and a risk of arterial and organ rupture

• Arthrochalasia (aEDS): severe hypermobility and congenital hip dislocation

• Dermatosparaxis (dEDS): extremely fragile, sagging skin

• Kyphoscoliotic (kEDS): progressive spinal curvature, muscle weakness, and fragile eyes

• Brittle cornea syndrome (BCS): thin corneas that are prone to rupture

• Spondylodysplastic (spEDS): short stature, muscle weakness, and bone changes

• Musculocontractural (mcEDS): joint contractures and distinctive facial features

• Myopathic (mEDS): muscle weakness that often improves with age

• Periodontal (pEDS): severe gum disease and early tooth loss

Because the subtypes differ so much in their risks, an accurate diagnosis matters. Vascular EDS in particular carries serious, potentially life-threatening complications that need specialized monitoring.

Issues People with EDS May Face

Joint problems. Frequent subluxations (partial dislocations), full dislocations, sprains, and early-onset arthritis are common. Joints may "slip" during everyday activities like turning over in bed or opening a jar.

Chronic pain and fatigue. Pain can be widespread and persistent, caused by joint instability, muscle strain from overcompensating, and nerve sensitivity. Fatigue is often profound and isn't relieved by rest alone.

Dysautonomia and POTS. Many people with hEDS also have postural orthostatic tachycardia syndrome (POTS), where standing causes a rapid heart rate, dizziness, brain fog, or fainting.

Digestive issues. Nausea, reflux, slow stomach emptying, constipation, and bloating are widely reported.

Skin and healing. Skin may bruise easily, tear, scar widely, or heal slowly, depending on the subtype.

Other overlapping conditions. Mast cell activation issues, migraines, temporomandibular joint (TMJ) problems, pelvic floor dysfunction, and sleep difficulties are often seen alongside EDS. The links between them are still being studied.

Delayed diagnosis. Many people wait years, sometimes decades, for a diagnosis. Symptoms get dismissed as anxiety, "growing pains," or being out of shape, especially in women. Because EDS touches so many body systems, patients often see a string of specialists who each treat one piece of the puzzle.

Emotional toll. Living with an invisible, chronic condition, along with the experience of not being believed, can take a real toll on mental health. Anxiety, depression, and isolation are common.

Living Well with EDS

There is no cure, but good management can make a meaningful difference:

• Physical therapy with a clinician who understands hypermobility, focusing on gentle strengthening and joint stability rather than stretching

• Pacing activity to avoid boom-and-bust cycles

• Supports like braces, splints, and mobility aids when needed

• Targeted treatment for related conditions such as POTS, GI problems, and pain

• Mental health support and connection with others in the EDS community

• A coordinated care team, ideally including a geneticist or a doctor familiar with EDS

The Bottom Line

EDS is complex, varied, and frequently overlooked, but awareness is growing, and so is research. If you suspect you or a loved one might have EDS, talk to a doctor, and consider asking for a referral to a geneticist or a specialist familiar with connective tissue disorders. You know your body, and your symptoms are worth taking seriously.

Sources and Further Reading

• Malfait F, et al. "The 2017 International Classification of the Ehlers-Danlos Syndromes." American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 2017; 175(1): 8-26. Abstract

• "Ehlers-Danlos Syndrome: Immunologic contrasts and connective tissue comparisons." PubMed Central. Read here

• "Ehlers Danlos Syndrome." StatPearls, NCBI Bookshelf. Read here

• The Ehlers-Danlos Society: EDS types and the 2017 International Classification

The EDS classification is under review by the International Consortium on Ehlers-Danlos Syndromes and Hypermobility Spectrum Disorders, so check the Society's website for the latest information.