Diagnosing EDS

[This content is outdated. Our team is currently working on an update. Stay tuned and come back later.]

Diagnostic criteria differs depending on which type of EDS is suspected.  Criteria for the 3 most common types of EDS are described below.

Hypermobility Type:

The Hypermobility Type is the most common form of Ehlers-Danlos Syndrome. It is characterized by loose, hypermobile joints and chronic joint pain. This form of Ehlers-Danlos Syndrome was formerly called type III.

The clinical diagnosis of Hypermobility Type EDS is typically established by family history and clinical examination.

Diagnostic criteria for Hypermobility Type:

It is critical to use the current BRIGHTON DIAGNOSTIC CRITERIA for diagnosing Ehlers-Danlos Syndrome – Hypermobility Type (HEDS)/ formerly EDS III, aka Joint Hypermobility Syndrome (JHS/BJHS), Hypermobility Syndrome (HMS).

The Beighton Score is a diagnostic tool and only PART of the overall diagnostic criteria for HEDS.

The accepted criteria since 2000, the Brighton  Diagnostic Criteria has been reproduced (as published) below.

_____________________________________________________________________________________

Brighton Diagnostic Criteria:

It is the REVISED 2000 DIAGNOSTIC CRITERIA for Ehlers-Danlos Syndrome – Hypermobility Type (formerly EDS III, aka HMS, JHS, BJHS)

Ehlers-Danlos Syndrome – Hypermobility Type (HEDS)  is diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative.

Major Criteria

  • Beighton score of 4/9 or greater (either currently or historically)
  • Arthralgia for longer than 3 months in 4 or more joints

Minor Criteria

  • Beighton score of 1, 2 or 3/9 (0, 1, 2 or 3 if aged 50+)
  • Arthralgia (> 3 months) in one to three joints or back pain (> 3 months), spondylosis, spondylolysis/spondylolisthesis.
  • Dislocation/subluxation in more than one joint, or in one joint on more than one occasion.
  • Soft tissue rheumatism. > 3 lesions (e.g. epicondylitis, tenosynovitis, bursitis).
  • Marfanoid habitus (tall, slim, span/height ratio >1.03, upper: lower segment ratio less than 0.89, arachnodactyly [positive Steinberg/wrist signs].
  • Abnormal skin: striae, hyperextensibility**, thin skin, papyraceous scarring.
  • Eye signs: drooping eyelids or myopia or antimongoloid slant.
  • Varicose veins or hernia or uterine/rectal prolapse.

Click here for a description of the Brighton Diagnostic Criteria 

What is the Beighton Score?

The Beighton Score is a diagnostic tool and only PART of the overall diagnostic criteria for Ehlers-Danlos Syndrome – Hypermobility Type (HEDS)/ formerly EDS III (aka HMS, JHS, BJHS).  Diagnosis of  HEDS should be made using the Brighton Diagnostic Criteria.

The Beighton modification of the Carter & Wilkinson scoring system has been used for many years as an indicator of widespread hypermobility. A high Beighton score by itself does not mean that an individual has HEDS / formerly EDS III (aka HMS, JHS, BJHS). It simply means that the individual has widespread hypermobility.

View a video Demonstration of the Beighton Test and scoring.

Classical Type:

The Classical Type of EDS  is characterized by highly elastic**, soft, and doughy skin, as well as unusual scarring and loose joints.  This type of Ehlers-Danlos Syndrome combines the types formerly called I and II.

The clinical diagnosis of Classic Type EDS is typically established by family history and clinical examination.

Diagnostic criteria for Classical Type:

The combination of the first three major diagnostic criteria should have a high specificity for EDS, Classical Type. The presence of one or more minor criteria contributes to the diagnosis of the Classical Type of EDS, but is not sufficient to establish the diagnosis. The presence of one or more minor criteria contributes to the diagnosis of the Classical Type of EDS, but is not sufficient to establish the diagnosis.

Major Diagnostic Criteria for the Classical Type of EDS

  • Skin hyperextensibility** should be tested at a neutral site (one not subjected to mechanical forces or scarring), such as the volar surface of the forearm. It is measured by pulling up the skin until resistance is felt. In young children, hyperextensibility of the skin is difficult to assess because of abundant subcutaneous fat.
  • Widened atrophic scars (a manifestation of tissue fragility)
  • Joint hypermobility depends on age, gender, and family and ethnic backgrounds. Joint hypermobility in Classical Type EDS is general, affecting both large and small joints. It is usually noted when a child starts to walk. It should be assessed using the Beighton scale[Beighton 1988], the most widely accepted grading system for the objective semi-quantification of joint hypermobility
  • Positive Family History

Minor Diagnostic Criteria for the Classical Type of EDS

  • Smooth, velvety skin
  • Molluscoid pseudotumors: fleshy, heaped-up lesions associated with scars over pressure points such as the elbows and knees
  • Subcutaneous spheroids: small, cyst-like, hard shot-like nodules, freely movable in the subcutis over the bony prominences of the legs and arms. They occur in about one-third of affected individuals, are numerous, and feel like hard grains of rice. X-ray reveals an outer calcified layer with a translucent core. The spheroids represent subcutaneous fat globules that have lost their blood supply, becoming fibrosed and calcified.
  • Complications of joint hypermobility (sprains, dislocations,subluxations, pes planus)
  • Muscle hypotonia, delayed gross motor development
  • Easy bruising
  • Manifestations of tissue extensibility and fragility (hiatal hernia, anal prolapse in childhood, cervical insufficiency)
  • Surgical complications (postoperative hernias)

Vascular Type:

The Vascular Type of EDS is characterized by possible arterial or organ rupture as a result of spontaneous rupture of vessels or organs due to the result of even minor trauma.  The Vascular Type of EDS is the most serious form of Ehlers-Danlos Syndrome.

The Vascular Type EDS is caused by mutations in the COL3A1 gene. There is a blood test available.

Diagnostic criteria for Vascular Type:

The combination of any two of the major diagnostic criteria should have a high specificity for the Vascular Type of EDS.  The presence of one or more minor criteria supports the diagnosis of the vascular type of EDS but is not sufficient to establish the diagnosis. Biochemical testing is strongly recommended to confirm the diagnosis.

Major Diagnostic Criteria for the Vascular Type of EDS

  • Arterial rupture
  • Intestinal rupture
  • Uterine rupture during pregnancy
  • Family history of the vascular type of EDS

Minor Diagnostic Criteria for the Vascular Type of EDS

  • Thin, translucent skin (especially noticeable on the chest/abdomen)
  • Easy bruising (spontaneous or with minimal trauma)
  • Characteristic facial appearance (thin lips and philtrum, small chin, thin nose, large eyes)
  • Acrogeria (an aged appearance to the extremities, particularly the hands)
  • Hypermobility of small joints
  • Tendon/muscle rupture
  • Early-onset varicose veins
  • Arteriovenous carotid-cavernous sinus fistula
  • Pneumothorax/pneumohemothorax
  • Chronic joint subluxations/dislocations
  • Congenital dislocation of the hips
  • Talipes equinovarus (clubfoot)
  • Gingival recession

RESOURCES

In that spirit, here are a handful of supportive pieces of information that we hope will help readers who are differently abled:

SSI Child Disability Starter Kit (for children under age 18)
https://www.ssa.gov/disability/disability_starter_kits_child_eng.htm

Parenting with Disabilities: A Guide to Home Modifications
https://www.homeadvisor.com/r/parenting-with-disabilities-home-modification-guide/

10 Types of Service Dogs and What They Do
https://www.dogster.com/lifestyle/10-types-of-service-dogs-and-what-they-do

Life-changing companions: How to afford a service dog
https://www.bankrate.com/loans/personal-loans/how-to-afford-a-service-dog/

Building a Wheelchair Ramp: What you should know before tackling the project
https://abilitytools.org/blog/building-a-wheelchair-ramp-what-you-should-know-before-tackling-the-project/

Don’t Let Disabilities Get in the Way of Getting the Job
https://www.monster.com/career-advice/article/job-hunting-with-a-disability

The Ultimate Guide to Finding and Renting Housing With Disabilities
https://www.thezebra.com/renting-with-disabilities/

Transportation and Travel for People with Disabilities
https://www.medicalhomeportal.org/living-with-child/after-a-diagnosis-or-problem-is-identified/caring-for-children-with-special-health-care-needs/transportation-and-travel-for-people-with-disabilities

*Diagnostic criteria was developed by a medical advisory group in a conference (sponsored by the Ehlers-Danlos Foundation [USA] and the Ehlers-Danlos Support Group [UK]) at Villefranche in 1997[Beighton et al 1998] 

**Skin hyperextensibility is assessed at a site lacking excess  or loose skin and without evidence of prior trauma by gently  pulling until resistance is met. Extensor surfaces of joints  should not be used because of the presence of excess  skin.  An ideal location is the volar surface of the forearm, where the upper limit of normal is approximately 1-1.5 cm.

DISCLAIMER – EDSawareness.com/ChronicPainPartners.com provides general information to the Ehlers-Danlos community. The information comes from a variety of sources, and EDSawareness.com/ChronicPainPartners.com does not independently verify any of it, nor does it necessarily reflect the views and/or opinions of EDSawareness.com/ChronicPainPartners.com. Nothing on this website should be construed as medical advice or be used as a substitute for professional medical care or advice. Always consult your doctor regarding your medical treatment or self-care.

Get Webinar Announcements And Our FREE Guide

Support EDS Awareness

See all upcoming events

Upcoming and Previous Webinar Speakers

  • webinar-image

    Ross A. Hauser, MD
    10 Low-cost ways to improve symptoms of peripheral joint and spinal instabilities from hEDS, including brain fatigue, pain, POTS, MCAS and more.

    Learn More

  • webinar-image

    Bonnie Nasar RDN
    “How to Build an EDS Plate: Feel Better with Food”?

    Learn More

  • webinar-image

    Presenter: Jeannie Di Bon   MA PMA-CPT
    Title: “A Pain in the Hypermobile Neck”

    Learn More

  • webinar-image

    Presenter: Dr. Eli Penn
    Title: hEDS and the Gastrointestinal Tract

    Learn More

  • webinar-image

    Pradeep Chopra, MD
    “Management of Long Covid with EDS”

    Learn More

  • webinar-image

    Eloise Stager, LMT, CST-D
    “The Role of CranioSacral Therapy in
    Pain Management and ANS Regulation”

    Learn More

  • webinar-image

    Anne Maitland, M.D., Ph.D
    “Beyond allergies- mast cell activation diseases!”

    Learn More

  • webinar-image

    Clair Francomano MD
    “EDS and Hypermobility Spectrum Disorders – Overview of Diagnosis and Management”

    Learn More

  • webinar-image

    Sonny Mullen
    “A Guide to Medical Fundraising”

    Learn More

  • webinar-image

    Karina Sturm
    New Feature-Length Ehlers-Danlos Documentary
    “We Are Visible”

    Learn More

  • webinar-image

    Podcast Interview with Karina Strum
    ‘We are Visible’ Podcast Interview

    Learn More

  • webinar-image

    Andrea Julian
    “Starting and Maintaining a Successful Support Group”

    Learn More

  • webinar-image

    Maria Dastur, M.B.A.
    Organizing and Managing your Facebook and Social Media

    Learn More

  • webinar-image

    Alan G. Pocinki M.D.
    Sleep Disorders in EDS

    Learn More

  • webinar-image

    Ross A. Hauser, MD
    Consequences and Resolution of Joint Instabilities in H- EDS

    Learn More

  • webinar-image

    Bonnie Nasar RDN
    “Optimizing nutrition to Decrease Brain Fog, Fatigue, & Pain”

    Learn More

  • webinar-image

    Jeannie Di Bon. MA, PMA-CPT
    “How can Pilates help with my EDS Symptoms”

    Learn More

  • webinar-image

    Petra Klinge MD
    “Managing Life with Neurological Symptoms and EDS”

    Learn More

  • webinar-image

    Cathy L. Pederson, Ph.D
    “Understanding the Relationship between Chronic Illness and Suicide”

    Learn More

  • webinar-image

    Patrick Agnew DPM
    “Foot and Ankle Treatments for EDS”

    Learn More

  • webinar-image

    Susan Tran, PhD
    “Partnering with Families to Improve Functioning in Youth with EDS”

    Learn More

  • webinar-image

    Paolo A Bolognese, MD
    Chiari I Malformation, EDS, and Craniocervical Instability

    Learn More

  • webinar-image

    Lauren Stiles, JD
    “Understanding the Relationship Between Ehlers-Danlos Syndrome and Postural Orthostatic Tachycardia Syndrome”

    Learn More

  • webinar-image

    Jordan Tishler, MD
    Medical Cannabis – Pain Relief for EDS

    Learn More

  • webinar-image

    Jeannie Di Bon, MA, PMA-CPT
    “Strengthen and Control Your Hypermobile Core With Pilates”

    Learn More

  • webinar-image

    John Ferman
    Online EDS Physician CME Education

    Learn More

  • webinar-image

    Anne Maitland, MD, PhD
    “Mast Cell Activation Disorder”

    Learn More

  • webinar-image

    Joshua Milner, M.D.
    “Update on Tryptase Research”

    Learn More

  • webinar-image

    Delia Chiaramonte, MD
    “Practical Stress Management: A Mind-Body Approach”

    Learn More

  • webinar-image

    Manu Sood, MD
    Katja Kovacic, MD
    “Gastrointestinal Motility Disorders and Cyclic Vomiting”

    Learn More

  • webinar-image

    Gisela Chelimsky, MD
    “Hypermobile EDS and Gastrointestinal Issues”

    Learn More

  • webinar-image

    Pradeep Chopra, MD
    “Management of complex pain in children and adults with EDS”

    Learn More

  • webinar-image

    Pradeep Chopra, MD
    “EDS Pain Management – Connecting the DOTS… part 3”

    Learn More

  • webinar-image

    Dr. Ajoy Sarkar
    “Ehlers-Danlos Syndrome Hypermobility Type in the UK: Missed or Just Misunderstood?”

    Learn More

  • webinar-image

    Sara Williams, PhD
    “Biofeedback: Training your Body to Relax”

    Learn More

  • webinar-image

    Jan Dommerholt, DPT
    “Physical Therapy for EDS – Part 2”
    Why does it hurt all of the time?

    Learn More

  • webinar-image

    Markus-Frederik Bohn, PHD
    “Tenascin X and Ehlers-Danlos Syndrome”

    Learn More

  • webinar-image

    Dr. Marco Castori

    “Multidisciplinary Diagnostic and Management Approach to the EDS Patient”

    Learn More

  • webinar-image

    Professor Claude Hamonet and Dr. Isabelle Brock

    “A French Perspective on Ehlers Danlos”

    Learn More

  • webinar-image

    Dr. Delia Chiaramonte

    “An Integrative Approach to Pain Management”

    Learn More

  • webinar-image

    Dan Doherty — YouScript / Genelex Corp.

    “Pharmacogenetic Testing to Assess Altered Drug Metabolism”

    Learn More

  • webinar-image

    Dr. Alan Pocinki

    “Psychiatric Misdiagnoses in EDS: When is Anxiety not Anxiety?”

    Learn More

  • webinar-image

    Dr. William Ericson

    “Orthopedic Issues in the EDS Hand, Wrist and Arm”

    Learn More

  • webinar-image

    Dr. Norman Marcus

    “EDS & Pain”

    Learn More

  • webinar-image

    Dr. Lawrence Afrin

    “Mast Cell Activation Syndrome”

    Learn More

  • webinar-image

    Diane O’Leary, PhD

    “All in Your Head”: The Problem of Psychogenic Diagnosis for Ehlers-Danlos Patients

    Learn More

  • webinar-image

    John Ferman and Deanna Hamm

    “World-Wide EDS Survey Results” by EDS Awareness
    (2544 respondents from 26 countries)

    Learn More

  • webinar-image

    Sara Williams, PhD

    “Cognitive Behavioral Therapy as a Coping Skill for EDS”

    Learn More

  • webinar-image

    Dr. Anne Maitland

    “Mast Cell Activation Syndrome in EDS Patients (Part 2)”

    Learn More

  • webinar-image

    Dr. Neil Schechter

    “Chronic Pain Hypermobile Children”

    Learn More

  • webinar-image

    Karen Foulks, OTR/CHT
    Jesse Garris, Silver Ring Splint Co.

    “Anatomy and SilverRing™ Splints for Ehlers-Danlos Hands”

    Learn More

  • webinar-image

    Dr. Holly Gilmer

    “Chiari Malformation in EDS”

    Learn More

  • webinar-image

    Dr. Blair Grubb

    “Postural Orthostatic Tachycardia Syndrome (POTs)”

    Learn More

  • webinar-image

    Trish Meegan, DPT, L, ATC

    “Physical Therapy for EDS: Including how your posture and thorax/ribcage affects your PT program”

    Learn More

  • webinar-image

    Dr. Patrick Agnew

    “Soft Tissue Surgery in Collagen Disease (for EDS Feet & Ankles)”

    Learn More

  • webinar-image

    Dr. Petra Klinge

    “Tethered Cord Syndrome in Ehlers-Danlos”

    Learn More

  • webinar-image

    Kelly Clancy, OT

    “New and Emerging Manual Therapy Approaches for EDS”

    Learn More

  • webinar-image

    Dr. Clive Bridgham

    “Chronic Pain Alternatives – with focus on inflammation and nutrition”

    Learn More

  • webinar-image

    Kevin Muldowney, MSPT

    “Physical Therapy Protocol for Ehlers-Danlos Syndrome” (using the new EDS Physical Therapy Book)

    Learn More

  • webinar-image

    Dr. Mitzi Murray

    “How, Why and When: Genetic Testing in EDS for the Non-Geneticist”

    Learn More

  • webinar-image

    Ellen Lenox Smith

    “Living Life Again with Dignity Using Medical Marijuana”

    Learn More

  • webinar-image

    Dr. Joan Stoler

    “Update on Complications and Rare Forms of EDS”

    Learn More

  • webinar-image

    Jennifer Ortiz, MPT, WSC

    “Physical Therapy for Sexual Dysfunction in EDS”

    Learn More

  • webinar-image

    John Ferman

    “Support Groups for Ehlers-Danlos Syndrome”

    Learn More

  • webinar-image

    Dr. John Mitakides

    “Musculoskeletal Headaches in EDS”

    Learn More

  • webinar-image

    Dr. Patrick Agnew

    “Foot & Ankle Issues with EDS”

    Learn More

  • webinar-image

    Dr. Hal Dietz

    “Connective Tissue Disorder Research”

    Learn More

  • webinar-image

    Dr. Theoharis Theoharides

    “Mast Cell Disorders”

    Learn More

  • webinar-image

    Dr. Brad Tinkle

    “Introduction to Ehlers-Danlos Syndrome”

    Learn More

  • webinar-image

    Dr. Ronald Jaekle

    “Ehlers-Danlos and Pregnancy”

    Learn More

  • webinar-image

    Dr. Manu Sood

    “Gastrointestinal Disorders in EDS”

    Learn More

  • webinar-image

    Dr. Forest Tennant

    “Managing Centralized Intractable Pain in Ehlers-Danlos”

    Learn More

  • webinar-image

    Cynthia Allen, GCFP, STMI

    “The Feldenkrais Method”

    Learn More

  • webinar-image

    Dr. Mark E. Lavallee

    “Exercise is Medicine”

    Learn More

  • webinar-image

    John Ferman

    “Support Groups for Ehlers-Danlos Syndrome”

    Learn More

  • webinar-image

    Dr. Howard R. Epps

    “Orthopaedic Considerations in EDS”

    Learn More

  • webinar-image

    Jan Dommerholt, DPT

    “Physical Therapy for Ehlers-Danlos, Part 1”

    Learn More

  • webinar-image

    Dr. Henry Burkholder

    “Postural Orthostatic Tachycardia Syndrome (POTs) and EDS”

    Learn More

  • webinar-image

    Dr. Peter Byers

    “Vascular Ehlers-Danlos Syndrome / EDS type IV”

    Learn More

  • webinar-image

    Subinoy Das, MD, FACS, FARS

    “Sinus Care for Ehlers-Danlos Syndrome”

    Learn More

  • webinar-image

    Frank Gargano PT, DPT, OCS, CIDN, MCTA, CWT

    “Dry Needling for EDS Pain Management: Can muscle performance be improved?”

    Learn More

  • webinar-image

    Diana Lebron, MD

    “Headaches and Ehlers-Danlos Syndrome”

    Learn More

  • webinar-image

    Shweta Dhar, MD, MS, FACMG

    “Coordination of Care in Adults with EDS”

    Learn More

  • webinar-image

    Kathleen Kane, ESQ

    “Applying for Disability”

    Learn More

  • webinar-image

    Dr. Richard Barnum
    “EDS and Psychiatric Illness Misdiagnoses”

    Learn More

  • webinar-image

    Dr. Anne Maitland
    “Mast Cell Activation Syndrome” Part 1

    Learn More

  • webinar-image

    Dr. Alan Pocinki
    “Chronic Pain, Poor Sleep, Depression, and Fatigue in EDS”

    Learn More

  • webinar-image

    Dr. Derek Neilson
    “Proving the obvious: Next Steps for the Demystification
    of the Ehlers-Danlos Hypermobility Type”

    Learn More

  • webinar-image

    Dr. Peter Rowe
    “Managing Orthostatic Intolerance in EDS”

    Learn More

  • webinar-image

    Prof Joel Lamoure, RPh., DD., FASCP
    “Medical Psychiatry in Pain Management”

    Learn More

  • webinar-image

    Eric Palmer, Orthotist
    “Bracing for EDS”

    Learn More

  • webinar-image

    Dr. John Mitakides
    “TMJ, Cervical Instability and EDS”

    Learn More

  • webinar-image

    Dr. Staci Kallish
    “Cardiac Manifestations in EDS”

    Learn More

  • webinar-image

    “EDS and Pain… Connecting the Dots” Part 2

    Pradeep Chopra, MD

    Learn More

  • webinar-image

    Presenter: Kevin Muldowney, PT Click here for a link to the announcement page Video link is on this page

    Learn More

  • webinar-image

    Presenter: Michael, Healy, DPT Click here for a link to the announcement page  Video link is on this page

    Learn More

  • webinar-image

    Dr. Kenneth Goldschneider
    Pain Management for EDS

    Learn More

Current Poll

How long until you received a proper diagnosis for EDS?

View Results

Loading ... Loading ...