Ehlers-Danlos Syndrome (EDS) is a group of inherited connective tissue disorders characterized by abnormalities in collagen and other components of connective tissue. While EDS is often associated with flexible joints and stretchy skin, it is actually a complex spectrum of disorders that can affect nearly every system in the body.

The 2017 International Classification recognizes 13 distinct subtypes of EDS, each with unique genetic and clinical characteristics. Among these, Hypermobile Ehlers-Danlos Syndrome (hEDS) is the most common—and arguably the most challenging to diagnose.

The 13 Recognized Types of EDS

The current classification includes:

1. Hypermobile EDS (hEDS)
2. Classical EDS (cEDS)
3. Classical-like EDS (clEDS)
4. Vascular EDS (vEDS)
5. Cardiac-valvular EDS (cvEDS)
6. Arthrochalasia EDS (aEDS)
7. Dermatosparaxis EDS (dEDS)
8. Kyphoscoliotic EDS (kEDS)
9. Brittle Cornea Syndrome (BCS)
10. Spondylodysplastic EDS (spEDS)
11. Musculocontractural EDS (mcEDS)
12. Myopathic EDS (mEDS)
13. Periodontal EDS (pEDS)

Most of these subtypes have identifiable genetic mutations and can be confirmed through molecular testing. Hypermobile EDS remains the notable exception, as no definitive genetic marker has yet been identified for the majority of affected individuals.

Why Hypermobile EDS Deserves Special Attention

Hypermobile EDS is often misunderstood as simply “being double-jointed.” In reality, it is a systemic connective tissue disorder that can affect joints, muscles, nerves, the gastrointestinal tract, the autonomic nervous system, and more.

Individuals with hEDS may experience:

• Joint instability and recurrent dislocations
• Chronic musculoskeletal pain
• Fatigue
• Gastrointestinal dysfunction
• Headaches and migraines
• Pelvic floor dysfunction
• Dysautonomia, including Postural Orthostatic Tachycardia Syndrome (POTS)
• Easy bruising
• Soft or fragile skin
• Proprioceptive difficulties
• Sleep disturbances

The severity of symptoms varies widely. Some individuals remain relatively functional, while others experience significant disability.

The Diagnostic Challenge of hEDS

Unlike other EDS subtypes, hEDS is diagnosed clinically. Healthcare providers must evaluate:

• Generalized joint hypermobility
• Systemic manifestations of connective tissue dysfunction
• Family history
• Chronic musculoskeletal symptoms
• Exclusion of other connective tissue disorders

One of the most widely used tools in this process is the Beighton Score.

What Is the Beighton Score?

The Beighton Score is a nine-point screening system used to assess generalized joint hypermobility.

Points are awarded for:

• Passive thumb-to-forearm movement (1 point per side)
• Fifth finger extension beyond 90 degrees (1 point per side)
• Elbow hyperextension beyond 10 degrees (1 point per side)
• Knee hyperextension beyond 10 degrees (1 point per side)
• Ability to place palms flat on the floor with straight knees (1 point)

The maximum score is 9.

Higher scores suggest greater joint hypermobility and may support further evaluation for hEDS or related conditions.

The Problem: The Beighton Score Was Never Designed to Diagnose EDS

One of the most common misconceptions is that the Beighton Score can diagnose or exclude hEDS. It cannot.

The Beighton Score was originally developed as a screening tool for generalized joint hypermobility—not as a comprehensive assessment of connective tissue disease.

Despite its widespread use, the score has several important limitations.

Hypermobility Changes With Age

Many individuals with hEDS lose flexibility over time due to:

• Joint degeneration
• Chronic pain
• Muscle tightness
• Arthritis
• Previous injuries
• Surgical interventions

A person who scored 8 or 9 as a teenager may score only 2 or 3 in adulthood while still experiencing significant connective tissue dysfunction.

It Ignores Many Commonly Hypermobile Joints

The Beighton assessment focuses on only a handful of joints.

It does not evaluate:

• Shoulders
• Hips
• Ankles
• Toes
• Jaw
• Cervical spine
• Ribs
• Sacroiliac joints

Many patients experience instability in these areas despite having a low Beighton Score.

It May Miss Previously Hypermobile Individuals

Some patients learn to restrict movement because of pain or repeated injuries.

Others undergo physical therapy, surgery, or develop compensatory muscle guarding that limits measurable hypermobility.

As a result, a current Beighton assessment may not accurately reflect lifetime joint mobility.

It Does Not Measure Symptoms

The Beighton Score assesses mobility—not disease burden.

A patient with a high score may have minimal symptoms, while another patient with a lower score may experience:

• Chronic pain
• Frequent dislocations
• Fatigue
• Dysautonomia
• Gastrointestinal complications
• Significant functional limitations

The score alone cannot determine severity.

Sex, Ethnicity, and Genetics Influence Scores

Research suggests that baseline joint flexibility varies across populations, ages, and sexes.

Using a universal cutoff may fail to capture the diversity of how hypermobility presents in different individuals.

Looking Beyond the Numbers

Recognizing these limitations, the 2017 hEDS diagnostic criteria incorporated additional measures beyond the Beighton Score.

Clinicians are encouraged to consider:

• Historical hypermobility
• Family history
• Systemic connective tissue features
• Chronic pain
• Joint instability
• Recurrent injuries
• Functional impairment

Patient history is often just as important as the physical examination.

Questions such as:

• “Could you do the splits as a child?”
• “Did your joints frequently dislocate?”
• “Were you considered unusually flexible growing up?”

may provide critical diagnostic information that a current Beighton assessment cannot capture.

The Future of hEDS Diagnosis

Researchers continue to investigate the genetic and biological mechanisms underlying hEDS. As scientific understanding evolves, future diagnostic approaches may include:

• Genetic markers
• Biomarker testing
• Advanced connective tissue imaging
• More comprehensive hypermobility assessment tools

Many experts believe that reliance on a single joint mobility score does not adequately reflect the complexity of hypermobile EDS.

Conclusion

The Beighton Score remains a useful screening tool, but it should never be viewed as the sole determinant of whether a person does or does not have hypermobile Ehlers-Danlos Syndrome.

Hypermobile EDS is a multisystem connective tissue disorder that extends far beyond joint flexibility. Accurate diagnosis requires a comprehensive evaluation that considers a patient’s history, symptoms, family background, and overall clinical presentation.

As awareness grows, healthcare professionals are increasingly recognizing that a low Beighton Score does not necessarily mean the absence of hEDS—and that listening to the patient’s full story is often the most valuable diagnostic tool of all.