Types of EDS

The 2017 international classification describes 13 types of EDS. The most common is hypermobile EDS. For each one, you will find here the signs, follow-up, genes and mode of inheritance.

13recognised types

12confirmed by genetic testing

2017classification

2017 international classification

The 13 types of EDS and their features

Dominant inheritance: one carrier parent is enough (a 1 in 2 risk for each child). Recessive: both parents are healthy carriers, meaning they have the gene without being ill (a 1 in 4 risk). The condition can also appear for the first time in a family.

Common
Rare
Very rare (a few dozen to a few hundred cases described)

hEDS Common

Hypermobile

Generalised hypermobility (joints that are too flexible), dislocations (a joint comes out of place) and subluxations (partial dislocation), chronic pain, severe fatigue. Often accompanied by digestive problems and dysautonomia (dysregulation of pulse, blood pressure or digestion).

Diagnosis: the only type with no genetic test. It is based on the 2017 clinical criteria.

Gene(s): unknown Inheritance: dominant

PDF sheet (in French) ↗

cEDS Rare

Classical

Very stretchy, soft skin, wide thin scars (“cigarette paper” scars), hypermobility, easy bruising.

Care: wounds heal poorly. Suitable stitches and protection for the knees and shins.

Gene(s): COL5A1, COL5A2 Inheritance: dominant

PDF sheet (in French) ↗

vEDS Rare

Vascular

Thin, translucent skin, visible veins, very easy bruising. Hypermobility mainly of the small joints.

Watch out for: risk of rupture of the arteries, intestine or uterus. Specialist follow-up in the FAVA-Multi centres ↗ and an emergency card.

Gene(s): COL3A1 Inheritance: dominant

PDF sheet (in French) ↗

clEDS Very rare

Classical-like

Resembles the classical type: velvety, stretchy skin, hypermobility, bruising, but without atrophic scars (sunken, thin scars).

Cause: the absence of tenascin-X, a connective tissue protein.

Gene(s): TNXB Inheritance: recessive

PDF sheet (in French) ↗

cvEDS Very rare

Cardiac-valvular

Progressive, severe damage to the heart valves, with hypermobility and fragile skin.

Follow-up: regular cardiology check-ups (heart ultrasound).

Gene(s): COL1A2 Inheritance: recessive

PDF sheet (in French) ↗

aEDS Very rare

Arthrochalasia

Congenital (present at birth) dislocation of both hips, severe hypermobility with repeated dislocations.

Diagnosis: often spotted in the first days of life.

Gene(s): COL1A1, COL1A2 Inheritance: dominant

PDF sheet (in French) ↗

dEDS Very rare

Dermatosparaxis

Extreme skin fragility, loose and excess skin, characteristic facial features.

Day to day: the skin can tear at the slightest knock, so it needs protecting.

Gene(s): ADAMTS2 Inheritance: recessive

PDF sheet (in French) ↗

kEDS Very rare

Kyphoscoliotic

Very hypotonic baby (muscles with no tone), early scoliosis (spine curved to the side) and kyphosis (rounded back) that get worse, hypermobility.

Follow-up: orthopaedic and respiratory, sometimes eye or artery involvement.

Gene(s): PLOD1, FKBP14 Inheritance: recessive

PDF sheet (in French) ↗

BCS Very rare

Brittle cornea syndrome

Very thin cornea (the clear surface of the eye), keratoconus (cornea bulging into a cone), bluish whites of the eyes, severe short-sightedness, hypermobility.

Watch out for: risk of corneal rupture. Protective glasses and eye follow-up.

Gene(s): ZNF469, PRDM5 Inheritance: recessive

PDF sheet (in French) ↗

spEDS Very rare

Spondylodysplastic

Short stature, muscle hypotonia (muscles with no tone), bowed limbs, skeletal abnormalities.

Follow-up: growth and orthopaedic, from childhood.

Gene(s): B4GALT7, B3GALT6, SLC39A13 Inheritance: recessive

PDF sheet (in French) ↗

mcEDS Very rare

Musculocontractural

Joint contractures (joints stuck in a bent position) from birth: thumbs, clubfoot (feet turned inwards), distinctive facial features, fragile skin.

Watch out for: risk of large bruises and organ damage.

Gene(s): CHST14, DSE Inheritance: recessive

PDF sheet (in French) ↗

mEDS Very rare

Myopathic

Muscle weakness from birth that often improves with age, stiffness of the large joints, hypermobile fingers.

Diagnosis: can be mistaken for a myopathy (muscle disease): genetic testing makes the difference.

Gene(s): COL12A1 Inheritance: dominant or recessive

PDF sheet (in French) ↗

pEDS Very rare

Periodontal

Severe gum inflammation from childhood, early tooth loss, brown patches on the shins.

Follow-up: very frequent dental check-ups.

Gene(s): C1R, C1S Inheritance: dominant

PDF sheet (in French) ↗

Since 2017, new forms have been described (for example a second classical-like type linked to the AEBP1 gene), and research continues to find the gene or genes behind hypermobile EDS.

New criteria expected on 1 December 2026. An international group of experts brought together by The Ehlers-Danlos Society will publish that day a new classification of all types of EDS and of hypermobility spectrum disorders, replacing the 2017 one. Care recommendations will follow in March 2027. This page will be updated as soon as they are published. If you already have a diagnosis, there is no need to worry or to rush into new steps: your doctor or your centre will tell you whether this change affects you.

The 13 n’Cie fact sheets in PDF

A detailed, printable sheet for each type, to show your doctor.

See the 13 sheets

Sources and resources

2017 international classification Reference classification of Ehlers-Danlos syndromes ↗ The Ehlers-Danlos Society Diagnostic criteria and overview of the different types of EDS ↗ Orphanet The reference portal for rare diseases: Ehlers-Danlos syndromes ↗ SED Gastroparésie N’Cie The EDS reference and competence centres →

Medical information

The content of this page is provided for information only. It does not replace a consultation, a diagnosis or personalised advice from a healthcare professional.

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