Ehlers-Danlos syndromes (EDS)

Connective tissue holds the body together. Rich in collagen, it gives strength, support and stretch to the skin, the ligaments and tendons, the blood vessels and the organs inside the body. EDS is the collective name for a set of inherited disorders of that tissue. In each, a change in a gene for collagen, or for a protein closely tied to it, makes the tissue looser or more easily damaged than usual, and that one common thread explains why the features vary so much from type to type.

EDS is usually inherited, though it can also start for the first time in someone with no family history of it. Some types follow a dominant pattern, in which each child of an affected parent has a one in two chance of inheriting it; others follow a recessive pattern, in which a child is affected only on inheriting a changed gene from each parent. The thirteen types set out in the 2017 international classification differ in which tissues they mainly affect, how they are inherited and how they are diagnosed, so EDS is better seen as a family of linked conditions than as one condition.

Thirteen types are set out in the international classification agreed in 2017. Most people with EDS have the hypermobile type, and the other twelve types are all rare. Each of those twelve has an identified gene, which means genetic testing can usually confirm it.

• Hypermobile EDS (hEDS), much the commonest, where flexible joints, instability and pain are the main features, and where no gene test exists yet
• Classical EDS (cEDS), in which the skin stretches a great deal, tears easily and heals into broad scars, and the joints are hypermobile too
• Classical-like EDS (clEDS), close to classical EDS in its soft, stretchy skin, but caused by a different gene and without the abnormal scars
• Cardiac-valvular EDS (cvEDS), where problems with the heart valves, which progress over time, are found alongside the skin and joint features
• Vascular EDS (vEDS), in which the arteries and hollow organs are fragile and at risk of a serious tear, and which is often regarded as the gravest of them
• Arthrochalasia EDS (aEDS), with severe hypermobility of the whole body and a strong tendency to dislocate, the hips included from birth
• Dermatosparaxis EDS (dEDS), in which the skin is soft, sagging and extremely fragile
• Kyphoscoliotic EDS (kEDS), marked by a spine that curves from early childhood, weak muscle tone and pronounced hypermobility
• Brittle cornea syndrome (BCS), in which the cornea, the clear front window of the eye, is thin, fragile and easily damaged, along with other features of the connective tissue
• Spondylodysplastic EDS (spEDS), where short stature, low muscle tone and changes in the way the bones grow come with hypermobility
• Musculocontractural EDS (mcEDS), with distinctive facial features, joints fixed in a bent position and fragile skin
• Myopathic EDS (mEDS), with low muscle tone, weak muscles and joints fixed in a bent position
• Periodontal EDS (pEDS), in which serious gum disease begins young and the teeth lose their hold in the jaw

Of all the types, hypermobile EDS is the one rheumatologists meet most, and the one most likely to bring someone to this practice. Its core is generalised joint hypermobility, with many joints moving beyond the usual range, but in hEDS symptoms come with it: joints that feel loose or partly slip out, pain that is long-lasting, easy bruising, and often tiredness, digestive trouble or light-headedness as well.

No gene has been linked to hEDS, so no blood test can show it. The diagnosis is clinical, made against criteria agreed in 2017 that combine three elements: generalised joint hypermobility, scored with a quick nine-point check known as the Beighton score; two or more of three further groups of features, one of them a family history of it; and ruling out anything else that might explain the picture. Applying the criteria takes a thorough history and examination, not one test. A new international framework for diagnosing every type of EDS, and hypermobility spectrum disorder, is due to be published on 1 December 2026 and will replace the 2017 criteria.

Seeing hEDS as part of a range helps. Simple joint hypermobility, common and often harmless, sits at one end. When flexible joints do bring symptoms but fall short of the full hEDS criteria, the label is hypermobility spectrum disorder (HSD). Treatment for HSD and for hEDS is largely the same, so the distinction helps understanding more than it changes care. The joint hypermobility syndromes guide looks at the whole range in broader terms, and this page goes into EDS in depth.

Vascular EDS needs a few plain words to itself. It is rare. In those who have it, the walls of arteries and of hollow organs, the bowel and the womb among them, are weaker than normal and can, uncommonly, tear with little warning. For that reason it is often regarded as the gravest type, and it is cared for in a different way from the others.

Recognising it is what counts in practice. Anyone who may have vascular EDS, because of their own features or because a relative had an artery or an organ tear early in life, needs referral to a clinical genetics service; there, a genetic test can settle the question, and suitable monitoring and specialist care can be set up. A known diagnosis changes how fresh symptoms are weighed and allows a plan to be worked out in advance. Carrying proof of it, a medical alert bracelet for example, tells the staff at A&E about it even if you cannot. The obstetric team needs to hear of it as soon as a pregnancy is confirmed, since pregnancy raises the risk of a tear, and a group of antibiotics called fluoroquinolones, ciprofloxacin among them, is generally avoided. Vascular EDS is rare among people assessed for hypermobility and joint pain, but it stays on the list so that the rare person with it is not missed. With vascular EDS, diagnosed or suspected, new pain in the chest, the abdomen or the side, sudden severe pain in the back, and severe or continuous pain in the head or the neck are 999 or A&E emergencies, as the first list under When to seek help says, where the other warning signs of a tear stand too.

The route to a diagnosis differs from type to type. The twelve rarer types each have a known gene, so genetic testing can usually confirm them, through a clinical genetics service once the features point that way. Hypermobile EDS works differently: as there is no gene to test, it is identified clinically, from generalised joint hypermobility measured on the Beighton score, the further features the criteria list, a family history among them, and a careful search for any other explanation.

At a rheumatology appointment, the doctor takes a full history and examines you, checking how the joints move and behave, looking at the skin, and watching for any feature that suggests a particular type. Point-of-care ultrasound is part of the consultation with Dr Borukhson, so where particular joints or soft tissues hurt, the tendons and other structures around them can be looked at directly and checked for inflammation; the ultrasound clinic page says more. If a rarer or graver type seems possible, you are referred on to clinical genetics for testing and any specialist monitoring.

Hypermobile EDS has no cure, and physiotherapy is at the centre of managing it. The groundwork is an exercise programme fitted to you and built up in stages, strengthening the muscles that hold and steady the joints, so that the joints sit more firmly and painful partial dislocations become rarer. For people with hypermobility, exercise can also improve balance and ease long-term pain.

Several other strands sit around it. Advice on protecting the joints helps you use them in ways that strain them less in everyday tasks. Pacing, the balance of activity and rest, matters because doing too much on a good day often brings on a flare. Simple measures come first for pain; when it persists or disturbs sleep and mood, a wider approach follows, on the lines set out in the fibromyalgia guide. Problems in the soft tissues, such as tendinopathy, turn up commonly, as do back and neck pain, and the same plan takes them in. Since hEDS may also cause tiredness and symptoms away from the joints, other specialists are sometimes brought in, and any medicine is picked carefully and kept under review. You and Dr Borukhson settle the plan together.

Day-to-day habits add a lot to what physiotherapy achieves, with the goal of joints that are strong and steady but never overloaded.

• Keep active, gently and often. Swimming, cycling, Pilates and other low-impact exercise strengthen the muscles that support the joints without jarring them, and moving between physio sessions helps the progress stick.
• Protect your joints. Do not push joints as far as they will go, even when stretching, and take regular breaks from tasks you repeat.
• Spread your energy. Mix activity with rest, and stop short of exhaustion, all the more if tiredness is one of your symptoms.
• Think about the small things. Shoes that support your feet take strain off, a physiotherapist can help with your posture and balance, and fragile or slow-healing skin needs looking after.
• Care for sleep and mood. Long-term pain often brings poor sleep and low mood, which need help of their own; neither means the symptoms are in your head.

With the pattern understood and a plan that fits, many people find their symptoms grow more predictable and easier to manage.

Most symptoms of hEDS are for a routine review. The few that are not sit in the two lists below, the most serious first, each with its route; the paragraph after the lists deals with the others.

Treat any of these as a 999 or A&E emergency: call 999 and do not drive yourself to A&E.

• Severe illness with suspected sepsis: a high fever with shaking, confusion, rapid breathing, or feeling dramatically worse over hours
• With a fever or feeling unwell, a rash that does not fade when you press it, a stiff neck with eyes that cannot bear bright light, or skin, lips or tongue turning blue, grey, pale or blotchy, since these can be signs of meningitis or sepsis; on black or brown skin the rash and the change of colour may be easier to see on the palms of the hands or the soles of the feet
• A joint you cannot put weight on or move, or that has moved out of place, or, after a fall or injury, very bad pain or any tingling or numbness
• After an injury, a crack heard as it happened, or skin around it that has turned blue, grey or cold, since these can be signs of a broken bone
• Chest pain that is crushing, tight or like a pressure, that spreads to an arm, the jaw, the neck, the stomach or the back, that comes with breathlessness, sweating, feeling sick or feeling light-headed, or that came on suddenly and does not go away
• Breathlessness that is severe, with gasping, choking or no breath to get words out; or coughing up blood
• Palpitations happening now that do not stop, or that come with chest pain, breathlessness, feeling faint or fainting, since a heart whose muscle or rhythm is affected has to be checked at once
• With vascular EDS, diagnosed or suspected: new pain in the chest, the abdomen or the side; sudden severe pain in the back; severe or continuous pain in the head or the neck; bleeding that starts suddenly, vomiting blood among it; a large bruise that keeps growing; a limb that swells for no clear reason; breathlessness or difficulty breathing or speaking; dizziness or passing out; weakness of an arm or a leg; any change in your sight; an eye that is red, painful and bulging; or a pulsing sound in the head, since in vascular EDS an artery, or a hollow organ such as the bowel, can tear
• A cut that will not stop bleeding or that spurts bright red blood, a wound that is very large, deep or gaping open, a bad cut on the face or the palm of the hand, something stuck in a cut, or loss of feeling or movement near a wound, since fragile skin and blood vessels can make a wound hard to close, and an open wound in EDS needs closing as soon as it can be
• A faint after which you cannot be woken within a minute, do not fully recover, or have trouble speaking or moving, or a faint with chest pain, a pounding, fluttering or irregular heartbeat, a seizure or a serious injury, or one that happened during exercise or while lying down, since these can mean a problem with the heart or the brain
• Sudden weakness or numbness on one side, a drooping face or difficulty speaking, a sudden severe headache, sudden confusion or a seizure, or sudden loss of vision, any of which can be a stroke, even if it stops after a short time
• An eye that suddenly and completely loses its sight
• Lips, mouth, throat or tongue that suddenly swell, a throat that feels tight, very fast breathing or a struggle to breathe or to swallow, skin, tongue or lips turning blue, grey or pale (on black or brown skin this may be easier to see on the palms of the hands or the soles of the feet), becoming suddenly very confused, drowsy or dizzy, or fainting and not being able to be woken, since this can be a severe allergic reaction, anaphylaxis, which can follow a dose of any medicine

Any of these needs an assessment that is urgent, on the same day, from your GP or NHS 111:

• A joint that is hot and swollen, with a fever or feeling unwell, since an infected joint has to be ruled out at once, at A&E or through NHS 111
• A joint that has become hot and very swollen suddenly, or is worsening quickly, even without a fever, since an infected joint does not always announce itself with one
• Joint pain with a fever, or feeling hot, cold or shivery, or generally unwell, since an infection in or around a joint has to be found the same day
• New chest pain, new palpitations, or breathlessness that is new or getting worse, with none of the signs in the first list, since a cause in the heart or the lungs is looked for that day; palpitations happening now that do not stop, or that come with chest pain, breathlessness, feeling faint or fainting, belong to the first list, and so, with vascular EDS, diagnosed or suspected, does any new chest pain or breathlessness; in pregnancy, breathlessness that comes on suddenly, or when you lie down, or with dizziness, is for your midwife or maternity unit straight away
• A cut longer than about 5 cm, a wound with soil or body fluids in it or still dirty after cleaning, or a bite from a person or a wild or stray animal, since skin affected by EDS can heal slowly, and a wound that may become infected needs treating that day
• A cut or other wound that is red, swollen and getting more painful or is weeping pus, skin around a cut that turns painful, hot and swollen, or a cut that comes with a high temperature or feeling generally unwell, since a wound can become infected and the infection can spread into the skin as cellulitis, whose redness may be harder to see on black or brown skin; with a fever or feeling unwell, short of the signs that follow, at A&E or through NHS 111; with a very high temperature or feeling hot, cold or shivery, a fast heartbeat or fast breathing, purple patches on the skin, which may be less obvious on black or brown skin, feeling dizzy or faint, confusion, or skin that is cold, clammy or pale, an infected wound or the skin around it is a 999 or A&E emergency, as are the signs of sepsis in the first list
• After an injury, swelling or bruising that is large or still getting worse, or a kneecap that has slipped out of place for the first time and gone back by itself, since a break, a torn ligament or damage around the joint has to be ruled out; NHS 111 can say where to go, and an urgent treatment centre can sometimes X-ray it; a joint or limb left at an odd angle or out of shape after the injury, a knee that swells up very quickly or gave a pop or a snap, or a shoulder that is painful, bruised or swollen after it slipped out of place, is a 999 or A&E emergency

After an injury, a joint or limb left at an odd angle or out of shape, a knee that swells up very quickly or gave a pop or a snap, or a shoulder that is painful, bruised or swollen after it slipped out of place, is a 999 or A&E emergency. With none of the signs in the first list, a single faint is for a GP appointment, so that its cause can be checked, and fainting that keeps happening is for a GP within days, since the cause has to be found; one possible cause, more likely with hypermobility, is a problem with how the body controls the heart rate and blood pressure on standing up. Book a routine review if joints slip out of place, fully or partly, and go back again in the way you know, or keep feeling unstable; if pain is steadily getting worse; or if new symptoms appear beyond the joints, such as marked tiredness or digestive trouble. If you would like a family history of EDS, or of an artery or organ that tore at a young age, looked into, ask your GP, who can refer you to a genetics service. No referral is needed to be seen here; next-working-day appointments can almost always be arranged, and same-day appointments are sometimes possible. If you cannot tell how urgent something is, asking is never a mistake.

EDS is widely misunderstood, and hypermobile EDS in particular is easy to miss, both because no test exists for it and because its features turn up all over the body. A clear assessment can sort out whether your symptoms match hEDS, hypermobility spectrum disorder or simple hypermobility, spot the uncommon cases that call for genetic testing or a specialist referral, and lay out a practical plan with physiotherapy at its centre. Understanding what is going on brings reassurance and a way forward, and where hypermobility causes symptoms, that understanding is often the first real step to feeling steadier and more in control.

What is EDS, or Ehlers-Danlos syndrome?

EDS stands for the Ehlers-Danlos syndromes, a family of inherited conditions in which connective tissue, which holds up the skin, the joints, the blood vessels and the organs inside the body, is put together differently. Collagen, or a protein that partners it, is altered, and the tissue ends up looser or easier to damage than usual. Thirteen types are recognised today, and they behave in quite different ways: hypermobile EDS, much the commonest, centres on the joints, whereas the rare types mainly concern the skin, the eyes, the bones or the blood vessels.

Does being double-jointed mean I have hypermobile EDS?

Not on its own. Plenty of people have joints that bend further than most and never have a problem with them. Hypermobile EDS (hEDS) is diagnosed against agreed criteria: widespread bendiness, joined by enough of a set of further features, which can include a family history of it, and nothing that explains it better. Between the two sits hypermobility spectrum disorder, in which flexible joints are causing symptoms without all the hEDS criteria being met. The broader picture is in the joint hypermobility syndromes guide, and this page goes deeper into EDS.

Is there a blood test for EDS?

For most types there is. Each type apart from hypermobile EDS has an identified gene, and a genetic test on a blood sample, usually organised by a clinical genetics service, can make the diagnosis certain. Hypermobile EDS is the exception: there is still no gene to test for, so the diagnosis is clinical, built from how the joints move, the other features found, the family history and excluding other conditions.

Which type of EDS is the most serious, and why?

Vascular EDS is rare, yet it can weaken the walls of arteries and of certain hollow organs, so that, uncommonly, one of them tears. That is the reason for specialist care and a clear plan, with referral to clinical genetics to confirm the diagnosis in anyone who may have it. There is no need for alarm, but recognising it matters, because the diagnosis alters how a new symptom is weighed and how care is organised. With vascular EDS, diagnosed or suspected, new pain in the chest, the abdomen or the side, sudden severe pain in the back, severe or continuous pain in the head or the neck, and bleeding that starts suddenly are 999 or A&E emergencies, as are the other warning signs of a tear in the first list under When to seek help. Call 999 and do not drive yourself to A&E.

What treatment helps hypermobile EDS?

Nothing cures it, and physiotherapy leads the care. The centre of it is a programme, increased step by step, that builds the muscles holding the joints steady, alongside advice on joint protection, pacing and pain. As hEDS can bring tiredness and symptoms outside the joints, other professionals sometimes join in. Exercise can be a main part of managing hEDS, because stronger muscles give loose joints more support.