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Health condition · Clinically reviewed

Ehlers-Danlos syndrome, 13 subtypes, one careful, multidisciplinary plan.

Not just bendy joints. A heritable connective tissue disorder that needs the right physiotherapy, the right surveillance and, in vascular EDS, the right specialist team.

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Why trust this guide

  • 01

    Clinically reviewed

    Written by our editorial team and reviewed by a registered UK clinician before publication.

  • 02

    Sourced from guidance

    Checked against the 2017 international EDS classification, NHS commissioned services and peer-reviewed sources you can see at the end.

  • 03

    Current for 2026

    Reflects modern UK practice including specialist EDS clinics, celiprolol in vascular EDS and hypermobility-aware physiotherapy.

Key facts

EDS at a glance.

The essentials, in plain English. What EDS is, which subtype matters most for you and how UK specialist care is organised.

  • What it is

    A group of heritable connective tissue disorders affecting collagen synthesis and processing, with 13 recognised subtypes in the 2017 international classification.

  • Most common form

    Hypermobile EDS (hEDS) accounts for around 90 per cent of cases, is diagnosed on clinical criteria and has no confirmed genetic marker.

  • Most serious form

    Vascular EDS (vEDS, COL3A1) carries a real risk of arterial, intestinal and uterine rupture and needs specialist surveillance.

  • Related term

    Hypermobility spectrum disorder (HSD) describes symptomatic joint hypermobility that does not meet full hEDS criteria.

  • Foundation of care

    Hypermobility-aware physiotherapy, pacing and multidisciplinary support, not a single medication or operation.

  • Where care sits

    A UK commissioned National EDS Service (adult and paediatric) plus regional specialist rheumatology, genetics and cardiology clinics.

Why this guide matters

A team plan, not a lifetime of loose joints.

EDS is not a single problem to fix, it is a system to support. The three points below shape everything else on this page.

  • Subtype changes everything

    Hypermobile, classical, vascular and the rarer forms need different tests, different specialists and different long-term plans.

  • Physiotherapy is the foundation

    A hypermobility-aware physiotherapist protects joints, calms pain and restores function far more than any single medicine.

  • Vascular EDS is different

    It needs specialist surveillance, celiprolol where tolerated and cautious anaesthetic and surgical planning.

How the diagnosis is made

From first suspicion to a subtype and a plan.

The steps a UK GP, rheumatologist or geneticist will normally follow, in order, so you know what to expect and why.

  1. 01

    Assessing

    Detailed history and family tree

    A careful look at joint problems, skin, bruising, GI and cardiovascular symptoms across you and your relatives.

  2. 02

    Assessing

    Beighton score and 2017 criteria

    The Beighton hypermobility score plus the 2017 international criteria for hEDS or the specific subtype.

  3. 03

    Assessing

    Skin and cardiovascular examination

    Skin texture, extensibility, scars and bruising, along with cardiac auscultation and pulses.

  4. 04

    Confirming

    Genetic testing where indicated

    Targeted gene panels or whole exome or genome sequencing for all subtypes other than hEDS, which remains clinical.

  5. 05

    Confirming

    Cardiology and imaging

    Echocardiogram to look at the aortic root and mitral valve, and vascular CT or MRI in suspected vascular EDS.

  6. 06

    Preparing

    Specialist EDS clinic and MDT

    Referral to the UK National EDS Service or a regional specialist team for confirmation, subtyping and coordinated care.

  7. 07

    Preparing

    Genetic counselling and family screening

    Discussion of inheritance, cascade testing for relatives and reproductive options where relevant.

Typical timeline: weeks for a working plan, months for full subtyping and MDT enrolment.

Symptoms

What EDS actually looks like.

The classic mix of joint, skin and autonomic features, plus the harder-to-spot cardiovascular, gut and mast cell overlap. And the red-flag features of vascular EDS.

  • Joint hypermobility

    Beighton score of six or more out of nine in adults, with joints that move beyond the usual range.

  • Subluxations and dislocations

    Joints that slip out of place or fully dislocate, sometimes with minimal force, and often recurrent.

  • Chronic joint and soft tissue pain

    Widespread musculoskeletal pain, tendon problems and fatigue that outlasts the initial injuries.

  • Skin changes

    Soft, velvety, mildly stretchy skin in hEDS, and hyperextensible skin with atrophic scars in classical EDS.

  • Autonomic symptoms and POTS

    Dizziness on standing, palpitations and postural orthostatic tachycardia syndrome are common companions of hEDS.

  • Gut and bladder dysfunction

    IBS-type symptoms, functional dyspepsia, gastroparesis and pelvic floor problems are frequently reported.

  • Mast cell and allergic features

    Flushing, hives, food and environmental sensitivities and features of mast cell activation in a subgroup of patients.

  • Red flag features of vascular EDS

    Thin translucent skin, easy bruising, characteristic facial features and family history of arterial or bowel rupture.

Treatment

How EDS is managed in the UK.

Hypermobility-aware physiotherapy first, multimodal pain and autonomic care next, and specialist vascular surveillance for those who need it.

  • Hypermobility-aware physiotherapy

    The mainstay of care. Graded exercise, core stability, joint stabilisation and proprioception with a specialist-informed physiotherapist.

  • Multimodal pain management

    Pacing, physiotherapy, psychology and neuromodulators such as amitriptyline, gabapentin or duloxetine through a specialist pain service.

  • Occupational therapy and adaptations

    Assessment for adaptive equipment, workplace adjustments and support with Access to Work and PIP where appropriate.

  • Cardiovascular surveillance

    Regular echocardiography for the aortic root and mitral valve, and vascular imaging in vascular EDS.

  • Celiprolol in vascular EDS

    A beta blocker with evidence for reducing arterial events in vEDS, prescribed by a specialist alongside strict activity and imaging advice.

  • POTS and autonomic care

    Increased fluid and salt, compression garments and, where needed, midodrine or fludrocortisone through a specialist POTS clinic.

  • GI, mast cell and allergy input

    Prokinetic drugs and dietary support for gut symptoms, and antihistamines, sodium cromoglicate or omalizumab for mast cell activation under immunology.

  • Psychological support

    Structured support for chronic pain, anxiety, low mood and, in some, PTSD from medical trauma, delivered as part of the MDT.

What this guide is based on

The sources behind every claim on this page.

UK commissioned service specifications, the 2017 international classification and peer-reviewed evidence, current at the time of last review.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

Your GP or specialist EDS team knows your history and can tell you which parts apply to you. If in doubt, get seen.

  • Malfait F et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet.

  • NHS England. Specialised service specification: Complex Ehlers-Danlos Syndromes Service (adult and paediatric).

  • Ehlers-Danlos Support UK. Toolkits for GPs, physiotherapists and patients.

  • Ong K-T et al. Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome. Lancet.

  • Grahame R et al. Hypermobility, the Ehlers-Danlos syndromes and chronic pain. Rheumatology.

Red flags

When EDS needs urgent attention.

Most day-to-day EDS symptoms are managed in the community. These situations are different and need urgent or specialist input.

  • Sudden severe abdominal or chest pain (vEDS)

    In known or suspected vascular EDS, treat sudden severe pain as possible arterial or intestinal rupture and go to A&E immediately.

  • Unexplained pneumothorax

    Spontaneous collapsed lung, especially in a young thin patient, can be a first presentation of vascular EDS.

  • Rapidly progressive scoliosis in a child

    Combined with hypotonia and fragile eyes, this points to kyphoscoliotic EDS and needs urgent specialist review.

  • Cauda equina or spinal cord signs

    New leg weakness, saddle numbness or bladder or bowel changes need emergency assessment, not a routine outpatient wait.

  • Pregnancy in known vascular EDS

    Pregnancy carries a materially raised risk of arterial and uterine rupture and needs specialist maternal fetal medicine planning from before conception.

  • Suicidality and severe mental health crisis

    Chronic pain, fatigue and diagnostic delay carry a real mental health burden. Any thoughts of self harm need urgent GP or crisis support.

  • Uncontrolled bleeding or bruising

    Marked bruising out of proportion to injury, or bleeding that will not stop, needs urgent haematology and EDS input.

  • Suspected mast cell anaphylaxis

    Sudden flushing, wheeze, throat swelling or collapse needs adrenaline and emergency assessment, then specialist immunology follow up.

  • Poor wound healing after surgery

    Wound breakdown, hernias or stretched scars after routine operations should trigger review for an underlying connective tissue disorder.

Living with it

A lifelong condition, with a clear rhythm.

Four things that make the biggest difference day to day. Steady movement, honest pacing, a small trusted team and a diagnosis you can hand over quickly.

A quiet reminder

Consistency beats intensity, every time.

Small, steady habits, kept up for months, do more than a heroic week that leaves you flared for a fortnight.

  1. 01 Movement

    Move often, gently, on your terms

    Little and often, guided by a hypermobility-aware physiotherapist, does more than heroic sessions that flare pain for a week.

  2. 02 Pacing

    Pace, plan and protect energy

    Alternate activity with rest, plan the week around what matters and treat pacing as a treatment, not a personality flaw.

  3. 03 Team

    Build a small trusted team

    A GP who knows you, a specialist EDS or rheumatology contact, physio, and one point of coordination for the rest.

  4. 04 Voice

    Carry your diagnosis with you

    A short written summary and, in vascular EDS, a medical alert. It saves time and, in an emergency, can save your life.

Frequently asked

Everything we get asked about EDS.

Quick answers on subtypes, diagnosis, physiotherapy and what vascular EDS means in practice.

  • What is Ehlers-Danlos syndrome?

    Ehlers-Danlos syndrome (EDS) is a group of heritable connective tissue disorders affecting how the body builds and processes collagen. The 2017 international classification recognises 13 subtypes, with hypermobile EDS the most common and vascular EDS the most serious.

  • How is EDS diagnosed?

    Diagnosis combines a detailed personal and family history, a Beighton hypermobility score and the 2017 international criteria. Genetic testing confirms most subtypes, but hypermobile EDS remains a clinical diagnosis with no confirmed gene. Cardiology, ophthalmology and vascular imaging are added when the subtype requires it.

  • Is hypermobile EDS the same as hypermobility spectrum disorder?

    No. Hypermobility spectrum disorder (HSD) describes symptomatic joint hypermobility that does not meet the full 2017 criteria for hypermobile EDS. Day to day management is very similar and both benefit from hypermobility-aware physiotherapy and multidisciplinary support.

  • Why is vascular EDS treated so differently?

    Vascular EDS is caused by faults in COL3A1 and carries a real risk of arterial dissection or rupture, intestinal perforation, uterine rupture in pregnancy and spontaneous pneumothorax. Care includes celiprolol where tolerated, annual vascular imaging, avoidance of invasive tests unless essential and specialist commissioned follow up.

  • What role does physiotherapy play?

    Physiotherapy is the mainstay of care in hypermobile EDS and HSD. A hypermobility-aware physiotherapist uses graded exercise, core stability, joint stabilisation and proprioception work. Progress is measured in months, not weeks, and consistency matters far more than intensity.

  • Can EDS be cured?

    There is no cure. Care is supportive and lifelong, aimed at protecting joints and blood vessels, treating pain, autonomic symptoms and gut problems, and supporting mental health, education and work. With the right team, most people with EDS lead full lives.

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