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Understanding hypermobile Ehlers-Danlos syndrome, and hypermobility spectrum disorder

previously known as Ehlers-Danlos syndrome hypermobility type, and joint hypermobility syndrome

Contents page

Chapter 1 - Frequently asked questions 




Heritable Disorders of Connective Tissue 

Ehlers-Danlos syndrome 

Hypermobile Ehlers-Danlos syndrome 

Genetics and hypermobile Ehlers-Danlos syndrome 

Joint hypermobility syndrome - where does it fit into the picture? 

EDS classification history 

EDS international classification 2017 

Introducing ‘hypermobility spectrum disorder‘ 

Concerns arising from the 2017 classification

Why are people so differently affected? 

Are hEDS or HSD progressive? 


Chapter 2 - Symptoms & comorbidities 


Symptoms, manifestations, syndromes and comorbidities 

Understanding the layout of this chapter 


Symptoms Part 1 - Widespread symptoms and comorbidities

Joint hypermobility 

Recurrent musculoskeletal disorders 

Hyperextended alignment of joints 


Complex regional pain syndrome


Proprioceptive dysfunction 

Muscle stiffness and tightness 

Habitual cracking of joints 

Frequent dislocations or subluxations 




Skin involvement 

Local anaesthetic 


Cardiovascular autonomic dysfunction 

Mast cell activation syndrome 

Raynaud’s phenomenon and chilblains 


Symptoms Part 2 - Area specific symptoms and comorbidities


Ocular features 

The nose and throat 

Asthma-like symptoms

Dental features 

Chronic neck strain 

The shoulders 

The chest and ribs 

The heart 

The elbow 

The wrist and hand

The spine

The bony pelvis 

The hip 

Gastrointestinal dysfunction

Contraception, pregnancy and childbirth 

Urogynaecological symptoms 

Male urinary tract dysfunction 

Restless leg syndrome 

Disorders of the knee 

Disorders of the foot and ankle 


Chapter 3 - Diagnosis and management of hEDS and HSD


IInitial diagnosis by clinical assessment

A note to clinicians

Quick questions designed to assist GPs

The 2017 International Diagnositic Criteria for Hypermobile Ehlers-Danlos Syndrome

The Beighton Score to diagnose generalised joint hypermobility

Reaching a diagnosis

The 2017 Diagnostic Framework for Hypermobility Spectrum Disorder

Diagnosing children and adolescents

The 2023 Diagnostic Criteria for Paediatric Joint Hypermobility

Paediatric Classification Categories

Summary of investigations and management of HSD and hEDS

What might a patient expect at a rheumatology clinical assessment?

An overview of services that may be involved in patient care

Management strategy and multi-disciplinary referral

Why patients must be assessed on an individual basis


Occupational therapy

Joint protection

Splinting, bracing, taping, compression ware, and orthotics

Relief of severe pain, and using painkillers effectively


Cognitive behavioural therapy

Complementary and physical therapies

Making the most of your health care team

Dental management in hEDS and HSD

Surgical issues


Image Attribution 

The 2017 classification criteria for 8 of the 13 Ehlers-Danlos syndrome subtypes

EDS Types Chart 

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