Ehlers-Danlos syndrome (EDS) is a group of inherited conditions that weaken your body’s connective tissue, the material that holds together your skin, joints, blood vessels, and organs. There are 13 recognized types, and the most common, hypermobile EDS, affects roughly 1 in 3,100 to 5,000 people. All forms share a root cause: genetic changes that disrupt collagen, the protein that gives connective tissue its strength and structure.
How Collagen Goes Wrong
Collagen is the most abundant protein in your body. It works like scaffolding, providing firmness and flexibility to skin, tendons, ligaments, blood vessel walls, and the lining of organs. In EDS, genetic mutations either prevent collagen from forming correctly or interfere with the proteins responsible for processing and folding it into usable shape. The result is connective tissue that stretches too easily, tears more readily, and fails to provide normal support.
Because connective tissue is everywhere, the effects ripple across multiple body systems. Joints lose stability. Skin becomes fragile or unusually stretchy. Blood vessels and organs may become vulnerable to injury. Which symptoms dominate depends on which type of collagen is affected and where in the body it’s most needed.
The 13 Types of EDS
A 2017 international classification expanded the number of recognized EDS subtypes to 13. Most are extremely rare. The ones you’re most likely to encounter are:
- Hypermobile EDS (hEDS): By far the most common, accounting for about 90% of all EDS cases. Joint hypermobility, chronic pain, and frequent dislocations or subluxations are hallmarks.
- Classical EDS (cEDS): Marked by very stretchy, fragile skin that bruises easily and heals with wide, papery scars. Joint hypermobility is also present.
- Vascular EDS (vEDS): The most dangerous form. It affects blood vessel and organ walls, creating a risk of spontaneous arterial rupture or organ perforation. The median lifespan for people with vEDS is 48 years, with most deaths caused by vascular complications.
The remaining ten types, including kyphoscoliotic, dermatosparaxis, cardiac-valvular, and periodontal EDS, are rare enough that many clinicians will never see a case. Each has distinct features, from severe spinal curvature to extremely fragile skin to gum disease with early tooth loss.
Genetics and Inheritance
EDS is caused by mutations in genes that either build collagen or help process it. For classical EDS, the mutations typically sit in genes responsible for type V collagen. Vascular EDS involves type III collagen, which is concentrated in blood vessel walls and hollow organs, explaining why that subtype carries such serious cardiovascular risk.
Most EDS subtypes follow an autosomal dominant pattern, meaning you only need one copy of the mutated gene (from one parent) to develop the condition. Some rarer forms are autosomal recessive, requiring a copy from both parents. Hypermobile EDS clearly runs in families, but despite being the most common type, its specific genetic cause has not yet been identified. This makes genetic testing unavailable for hEDS, which has significant implications for diagnosis.
How EDS Is Diagnosed
For most subtypes, a blood sample can confirm the diagnosis through genetic testing. Vascular, classical, and other rarer forms each have identified gene mutations that a lab can look for. Hypermobile EDS is the exception. Because no genetic marker has been found, hEDS is diagnosed entirely through clinical evaluation.
A key part of that evaluation is the Beighton score, a nine-point scale that measures joint flexibility. You receive one point for each of the following you can do: bend each pinky finger back past 90 degrees, touch each thumb to your forearm, hyperextend each elbow, hyperextend each knee, and place your palms flat on the floor without bending your knees. A score of four or more, combined with joint pain lasting at least three months, suggests generalized hypermobility. From there, clinicians look at additional criteria including skin involvement, family history, and musculoskeletal complications to distinguish hEDS from other causes of flexible joints.
Diagnosis often takes years. Many people with hEDS are told their pain is unexplained or psychological before a specialist connects the dots between joint instability, skin findings, and systemic symptoms.
What Daily Life Feels Like
The experience varies widely by type and severity, but chronic pain is nearly universal. Joints that move beyond their normal range are prone to partial dislocations (subluxations) during ordinary activities like reaching overhead, sleeping in certain positions, or walking on uneven ground. Over time, repeated joint injuries lead to early osteoarthritis.
Fatigue is another constant companion, often disproportionate to activity level. Skin may bruise from minor contact, heal slowly, or scar abnormally. Some people find that wounds split open easily or that adhesive bandages tear their skin. For those with vascular EDS, even routine activities carry a background risk of arterial or organ rupture, which can occur without warning or obvious trauma.
Conditions That Often Overlap
EDS rarely travels alone. Two conditions appear alongside it frequently enough that clinicians familiar with EDS actively screen for them.
The first is postural orthostatic tachycardia syndrome (POTS), a dysfunction of the autonomic nervous system that causes your heart rate to spike when you stand up. Symptoms include dizziness, brain fog, and fainting. The second is mast cell activation syndrome (MCAS), where immune cells release histamine and other chemicals inappropriately, causing flushing, hives, GI distress, and allergic-type reactions without a clear allergen. In one study, 66% of patients already diagnosed with both POTS and EDS reported symptoms consistent with MCAS, suggesting a strong overlap among all three conditions.
The biological connection between these conditions is still being worked out, but if you have EDS and experience unexplained episodes of rapid heart rate, flushing, or GI symptoms, these overlapping conditions are worth investigating.
Managing EDS
There is no cure for EDS, and no treatment that fixes the underlying collagen defect. Management focuses on protecting joints, building strength, and reducing pain.
Physical therapy is the cornerstone. The goal is to strengthen the muscles surrounding hypermobile joints so they provide the stability that loose ligaments cannot. Low-impact exercises that emphasize controlled movement work best. Swimming, cycling, Pilates, and targeted resistance training are common recommendations. The standard advice is to start low and go slow: begin with few repetitions, light resistance, and short sessions, then increase gradually. Rushing the process tends to trigger flares.
Activities to avoid depend on your subtype. For hypermobile EDS, high-impact sports (running, jumping), contact sports, and anything that emphasizes extreme flexibility can worsen joint instability. People with classical EDS need to be cautious about friction and skin trauma. Those with vascular EDS should stick to low-intensity activity and avoid anything that causes sharp spikes in blood pressure or heavy straining.
Braces and splints can stabilize problem joints during activities that would otherwise cause subluxations. Ring splints for fingers, ankle braces, and wrist supports are common tools. Pain management often involves a combination of physical strategies, since many people with EDS find that standard pain medications offer limited relief over the long term.
Vascular EDS Requires Special Caution
Vascular EDS deserves its own mention because it carries risks the other types do not. The defective collagen in vEDS concentrates in arterial walls and the walls of hollow organs like the intestines and uterus. Spontaneous arterial dissections, intestinal perforations, and organ ruptures can occur at any age, often without warning. These events are medical emergencies.
People with vEDS typically have thinner, more translucent skin (veins are often visible through the skin on the chest and abdomen), and they bruise extensively. Pregnancy carries elevated risk due to the possibility of uterine or arterial rupture. The median survival of 48 years reflects how serious the vascular complications can be, though individual outcomes vary and surveillance protocols can help catch developing problems earlier.