The 13 Ehlers-Danlos Syndrome Types: EDS’s Many Forms

This debilitating connective tissue disorder affects an estimated 10 million people in the U.S. yet has no cure. Here’s what you need to know about the 13 Ehlers-Danlos Syndrome types.

ehlers danlos syndrome types

One of the hallmark symptoms among Ehlers-Danlos Syndrome types: overly flexible and lax joints, a condition called hypermobility.

© Bahadir Yeniceri | Dreamstime.com

Ehlers-Danlos Syndrome, or EDS, is a group of 13 heritable (i.e., genetic) disorders that affect the body’s connective tissues. These tissues—found mostly in the skin, joints, and blood vessel walls—act like a glue to help provide strength and elasticity to the body’s structures, including the digestive system and essential organs. Those with EDS suffer from hypermobile (overly flexible and lax) joints, stretchy, fragile, and easily bruised and scarred skin, and poor wound-healing. All of the Ehlers-Danlos Syndrome types share these characteristics, but each form has additional symptoms (for instance, the rupture of arteries common in vascular EDS). Some EDS symptoms even can be fatal.

(To learn more about this condition, see our post Ehlers-Danlos Syndrome: “The Most Neglected Disorder in Modern Medicine.”)

Most Common Ehler-Danlos Syndrome Types

In March 2017, an international consortium of more than 90 EDS experts established new diagnostic criteria, creating 13 subtypes of Ehlers-Danlos Syndrome. The three most common forms are:

  • Hypermobile EDS (hEDS)
  • Classical EDS (cEDS)
  • Vascular EDS (vEDS)

HEDS leads the pack with an estimated 1 in 5,000 suffering from the disease. It’s followed by cEDS, which affects about 1 in 15,000, and vEDS, the most severe form of the condition, which affects about 1 in 250,000. Depending on which of the Ehlers-Danlos Syndrome types a patient has, he or she may experience symptoms in addition to those mentioned above. Read on to find out more.

1. Hypermobile EDS

Hypermobile EDS (hEDS) is the most common and least severe form of all Ehlers-Danlos Syndrome types. It represents between 80 to 90 percent of all cases of EDS, claims a paper published in the American Journal of Medical Genetics. According to the authors, “hEDS is likely the most common systemic inherited connective tissue disorder in humans, which translates to approximately 2 million in the United Kingdom, 10 million in the United States, 17 million in Europe, and 255 million affected worldwide.” With a new set of diagnostic criteria that makes it more difficult to be diagnosed with hEDS, these numbers possibly will be slightly lower in the future.

Symptoms of hEDS can present themselves at any time, but they often pop up during childhood or adolescence. Far too often, they are misjudged or ignored at that time and don’t often result in a diagnosis.

In addition to the typical EDS symptoms mentioned above, those with hEDS may also suffer from:

  • Subluxation (a partial dislocation)
  • Dislocation
  • Chronic pain in joints and bones
  • Chronic fatigue
  • Atrophic scarring (sunken or depressed scars with well-defined borders)
  • Autonomic dysfunction (problems with the control system that monitors such functions as heart rate, digestion, and respiratory rate)
  • Postural Orthostatic Tachycardia Syndrome, POTS (a condition that causes an increased heart rate along with feelings of dizziness and fainting episodes)
  • Urinary dysfunction
  • Digestive and gastrointestinal issues
  • Anxiety
  • Disturbed sleep
  • Depression
  • Mitral valve prolapse in the heart (usually very mild in hEDS)
  • Aortic root dilation (involving an enlarged blood vessel that circulates blood from the heart)
  • Uterine, rectal, or bladder prolapse
  • Varicose veins
  • Early onset osteoarthritis
  • Osteoporosis

2. Classical EDS (cEDS)

Classical EDS is the next-most-common form of the condition. This genetic disorder presents itself with extremes of hypermobile joints and stretchy and fragile skin.

Additional symptoms of cEDS include:

  • Atrophic scarring
  • Significant bruising

3. Vascular Ehlers-Danlos Syndrome (vEDS)

This is one of the most severe Ehlers-Danlos Syndrome types. Those with vEDS can suffer from weakened or ruptured arteries and blood vessels including the aorta (the heart’s largest artery) and arteries that move blood through the kidneys and spleen. These ruptures can be fatal.

Additional symptoms of vEDS include:

  • Fragile arteries, intestines, and uterus
  • Gastrointestinal perforation
  • Dissection or rupture of arteries and blood vessels
  • Frequent subluxations and dislocations

4. Kyphoscoliotic EDS (kEDS)

This rare Ehlers-Danlos type is characterized by a severe curvature of the spine, which worsens over time and can compromise a person’s breathing.

Additional symptoms of kEDS include:

  • Curvature in the upper part of the spine
  • Small cornea
  • Fragile artery walls

5. Brittle Cornea Syndrome (BCS)

Those who suffer from Brittle Cornea Syndrome experience a thinning of the cornea (the clear covering of your eye) along with other abnormalities. The cornea is extremely fragile and can rupture spontaneously, without any significant source of trauma. The result: visual loss and blindness.

Additional symptoms of BCS include:

  • Curvature of the spine

6. Spondylodysplastic EDS (spEDS)

Those with Spondylodysplastic EDS are short in stature and suffer from skeletal abnormalities such as bowed limbs.

Additional symptoms of spEDS include:

  • Bone weakness
  • Low muscle tone (hypotonia)
  • Bowed limbs
  • Flat feet
  • Eye problems
  • Mild cognitive or learning disability

7. Musculocontractural EDS (mcEDS)

This type of EDS is characterized by muscle abnormalities such as low muscle tone (hypotonia) and deformed, bent, or rigid joints (contractures).

Additional symptoms of mcEDS include:

  • Distinctive craniofacial disfigurement
  • Severe bent thumbs and fingers
  • Club feet
  • Severe spinal curvature
  • Low muscle tone
  • Wrinkled palms
  • Eye problems

8. Myopathic EDS (mEDS)

This rare form of EDS is similar to mcEDS and causes muscle atrophy or weakness.

Additional symptoms of mEDS include:

  • Bent thumbs and fingers
  • Muscle weakness
  • Low muscle tone

9. Classical-like EDS (clEDS)

Similar to cEDS, this variety of EDS is extremely rare and lacks the scarring feature of the classic form.

Additional symptoms of clEDS include:

  • Weak muscles
  • Prolapse of the vagina, uterus, or rectum
  • Foot deformities
  • Hypermobility with or without chronic dislocations (most often in the shoulder or ankle)

10. Cardiac-valvular EDS (cvEDS)

This severe form of EDS involves serious problems with one or more of the valves in the heart.

Additional symptoms of cvEDS include:

  • Severe problems with valves of the heart
  • Deformed chest
  • Hernia in the inguinal canal, which can cause contents of the abdominal cavity to protrude

11. Arthrochalasia EDS (aEDS)

This severe type of EDS, typified by bilateral hip dislocations, is extremely rare—only about 30 cases have been noted. Those who suffer from aEDS are even more hypermobile than those with hEDS. Additional symptoms of aEDS include:

  • Severe hypermobility with multiple dislocations and subluxations
  • Low muscle tone
  • Spinal curvature
  • Bilateral hip dislocations

12. Dermatosparaxis EDS (dEDS)

Another extremely rare variation among Ehlers-Danlos Syndrome types, Dermatosparaxis EDS’s main features include severe bruising, fragility, and sagging of the skin. In the very few reported cases (only about 10), patients are diagnosed by the age of 2.

Additional symptoms of dEDS include:

  • Umbilical hernia
  • Very wrinkled palms
  • Postnatal growth restriction

13. Periodontal EDS (pEDS)

This form of EDS causes dental problems such as periodontitis, often beginning in childhood or adolescence.

Additional symptoms of pEDS include:

  • Detached gingiva
  • Pretibial plaques (nodules and edema of the skin in the tibia region)
Comments
  • Thanks for your insightful article. My daughter’s partner, Sara Geurts, has type 1 and 12 and now has developed psoriasis. She hasn’t found a single doctor in Minneapolis/St Paul to treat her and has been severely financially constrained due to the medical bills. She is a well-known social media advocate for EDS and her type is quite visible. She is 28 but looks much older due to the extreme folds in her skin. Since her type is so rare do you know of any doctor or clinic that would be willing to treat her on a Pro Bono or reduced fee schedule? She has health insurance. Thanks!

    Reply

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