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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, respectively.

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 Classification 2017 

Introducing ‘hypermobility spectrum disorder‘ 

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


Initial diagnosis by clinical assessment 

A note to clinicians

Quick questions designed to assist GPs

Diagnostic criteria

2017 International Criteria for hypermobile Ehlers-Danlos syndrome

The Beighton Score to diagnose generalised joint hypermobility

Reaching a diagnosis

Summary of investigations and management of HSD and hEDS

The 2017 Classification Criteria for 8 of the 13 Ehlers-Danlos syndrome subtypes

What might a patient expect at a rheumatology clinical assessement

An overview of services who may be involved in patient care 

Management strategy and multi-disciplinary referral 


Occupational therapy 

Joint protection 

Splinting, bracing, taping, compression ware, and orthotics

Relief of severe pain, and using pain killers effectively

Self management 

Cognitive behavioural therapy 

Complementary and physical therapies 

Making the most of your health care team 

Dental management in hEDS and HSD

Surgical issues 

Bibliography, and image attribution


EDS Types Chart

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