Medically reviewed by Assoc. Prof. Dr. Alan Hakim, MD MA FRCP · June 2026 · Source: ehlers-danlos.com
The Ehlers-Danlos syndromes (EDS) are a group of 13 heritable connective tissue disorders caused by genetic changes that affect the structure, production, or processing of connective tissue. Connective tissue is found throughout the body — in skin, muscles, tendons, ligaments, blood vessels, organs, gums, and eyes. Because connective tissue is so widespread, EDS can affect many different body systems simultaneously and can look very different from one person to the next.
Three features are common across all types of EDS: joint hypermobility, skin hyperextensibility, and tissue fragility — though not every individual will have all three. Each of the 13 types has its own genetic causes, diagnostic criteria, and clinical features.
13
Distinct types of EDS, each with unique features and diagnostic criteria
~90%
Of all EDS cases are hypermobile EDS — the most common type
10–12 yrs
Average time from presenting with concerns to diagnosis, across EDS and HSD
Source: ehlers-danlos.com/what-is-eds/
Each type has its own genetic causes, diagnostic criteria, and clinical features. Twelve of the 13 types have identified genetic causes and can be confirmed by genetic testing. The one exception is hypermobile EDS (hEDS) — the most common type and the only one that currently has no identified genetic cause or confirmable genetic test.
Hypermobile EDS (hEDS) accounts for approximately 90% of all EDS cases. It is the most frequently misdiagnosed type and the only type that cannot currently be confirmed by genetic testing.
| Type | Abbrev. | Key Features |
|---|---|---|
| Hypermobile EDS | hEDS | Most common (~90% of cases). Joint hypermobility, instability, chronic pain. No genetic test available. Clinical diagnosis only. |
| Classical EDS | cEDS | Stretchy, fragile skin with characteristic scarring. Joint hypermobility. COL5A1/COL5A2 gene mutations. |
| Classical-like EDS | clEDS | Similar to classical EDS without atrophic scarring. TNXB mutations. |
| Cardiac-valvular EDS | cvEDS | Severe progressive cardiac valvular disease. Rare. COL1A2 mutations. |
| Vascular EDS | vEDS | Risk of arterial, intestinal, and uterine rupture. Life-threatening. COL3A1 mutations. |
| Kyphoscoliotic EDS | kEDS | Progressive scoliosis from birth, muscle weakness, fragile eyes. PLOD1/FKBP14 mutations . |
| Arthrochalasia EDS | aEDS | Severe generalized joint hypermobility, congenital hip dislocation. COL1A1/COL1A2 mutations. |
| Dermatosparaxis EDS | dEDS | Extreme skin fragility, sagging skin, hernias. ADAMTS2 mutations. |
| Brittle Cornea Syndrome | BCS | Progressive corneal thinning, blue sclerae, hearing impairment. ZNF469/PRDM5 mutations. |
| Spondylodysplastic EDS | spEDS | Short stature, bowing of limbs, skeletal dysplasia. B4GALT7/B3GALT6/SLC39A13 mutations. |
| Musculocontractural EDS | mcEDS | Congenital contractures, distinctive facial features. CHST14/DSE mutations. |
| Myopathic EDS | mEDS | Muscle weakness or contractures from birth. COL12A1 mutations. |
| Periodontal EDS | pEDS | Severe, treatment-resistant periodontitis, gum fragility. C1R/C1S mutations. |
Source: ehlers-danlos.com/types/
Because EDS affects connective tissue throughout the body, symptoms can involve multiple systems simultaneously. The specific symptoms depend on the type of EDS. Across all types, some degree of the following is typically present.
Musculoskeletal
Skin
Autonomic
Other Associated Conditions
Source: www.ehlers-danlos.com/heds/ · ehlers-danlos.com/types/
EDS is a group of heritable connective tissue disorders, meaning it is caused by genetic changes typically passed down through families. For 12 of the 13 types, specific gene mutations have been identified and can be confirmed through genetic testing.
Genetic inheritance
Some types of EDS are autosomal dominant, meaning only one copy of the altered gene is needed to cause the disorder — an affected person has approximately a 50% chance of passing it on to each child. But most types are autosomal recessive, requiring two copies of the altered gene.
Hypermobile EDS: cause currently unknown
The cause of hypermobile EDS has not yet been identified. Research is ongoing. Although hEDS appears to follow an autosomal dominant inheritance pattern based on family histories, no causative gene has been confirmed. This is the primary reason hEDS cannot currently be diagnosed by a genetic test and requires a clinical evaluation.
Source: https://www.ehlers-danlos.com/types/ · ehlers-danlos.com/types/
Diagnosis of EDS depends on the type. For most types, a combination of clinical evaluation and genetic testing is used. For hypermobile EDS (hEDS), diagnosis is clinical only — there is currently no laboratory test available.
Clinical evaluation
A clinical evaluation typically includes a detailed medical and family history, a physical examination, and assessment of joint mobility, skin features, and other clinical signs. The clinician compares findings against the diagnostic criteria for each type.
Genetic testing
Genetic testing can confirm 12 of the 13 types of EDS. Testing is most appropriate when clinical findings raise concern for a rare or vascular type. Interpretation is most reliable when performed by a clinician with specialized training in connective tissue disorders or genetics.
Classification timeline
EDS Classification Milestones
Source: ehlers-danlos.com/diagnosis/ · ehlers-danlos.com/road-to-2026/
EDS occurs across all racial, ethnic, and geographic groups. The true prevalence is not fully known and is likely underestimated due to underdiagnosis and misdiagnosis.
EDS affects people of all genders, races, and ethnicities. A perceived excess of female patients is documented, which the Ehlers-Danlos Society notes may be related to the influence of sex hormones on joint hypermobility.
Source: ehlers-danlos.com/prevalence/
For information about the most common type — hypermobile EDS — or about Hypermobility Spectrum Disorder, see the resources below.
Medically reviewed by
Assoc. Prof. Dr. Alan Hakim, MD MA FRCP
Medicine & Rheumatology. Biobank and DICE Registry Principal Investigator and Medical & Scientific Board Member, The Ehlers-Danlos Society · Member of the Steering Committee of The International Consortium on the Ehlers-Danlos Syndromes and Hypermobility Spectrum Disorders
Last reviewed: June 2026 · All clinical content sourced from ehlers-danlos.com
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