Living with Ehlers-Danlos Syndrome, Type III – Hypermobile

Types of Ehlers-Danlos Syndromes

Click any syndrome to jump to its full list of symptoms:

Classical EDS (cEDS)

  • extremely stretchy, smooth skin that is fragile and bruises easily;
  • wide, flat or depressed scars; and
  • joints that stretch further than they should (hypermobility).

Also frequently seen are:

  • calcified bruises over pressure points, such as the elbow, and
  • lipomas (fatty cysts that appear as lumps on forearms and shins).

Also possible in infants are:

  • severely low muscle tone or Floppy Baby Syndrome (FBS); and
  • delayed motor development.
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Classical-like EDS (clEDS)

  • extremely stretchy and velvety skin with no wide, flat or depressed scarring;
  • generalized joint hypermobility (GJH) with or without repeated dislocations (usually in shoulders and ankles); and
  • easy bruising, along with bruises larger than 3mm in diameter.
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EDS3 or Hypermobile EDS

  • large (knee, elbow) and small (fingers, toes) joints stretching beyond the normal range, which may lead to repeated joint dislocations and subluxations (partial dislocation).

Also likely:

  • soft, smooth, and velvety skin with easy bruising; and
  • chronic pain of the muscles and/or bones.

Although not listed as criteria on the diagnostic worksheet (created in 2017), other symptoms are nationally recognized as common to people with EDS3. Such symptoms include:

  • problems with the autonomic nervous system, responsible for regulating body functions and the fight-or-flight response, such as:
    • tachycardia (rapid heartbeat);
    • chest pain;
    • shortness of breath;
    • temperature intolerance;
    • difficulty swallowing;
    • excessive thirst;
    • light-headedness upon changing positions too quickly, specifically standing quickly from a lying down position; and
    • fainting;
  • at least seven different types headaches, which become constant;
  • mitral valve prolapse;
  • organ prolapse;
  • teeth overcrowding;
  • piezogenic papules on the heel when standing (painful bumps of fat)
  • chronic disorders, such as:
    • pain;
    • fatigue;
    • nausea;
    • neck pain; and
    • anxiety;
  • fibromyalgia;
  • insomnia;
  • muscle weakness;
  • severe muscle spasms;
  • early onset osteoarthritis;
  • degenerative joint disease;
  • degenerative disc disease;
  • debilitating pain;
  • craniocervical instability (CCI, sometimes called “bobblehead”);
  • bowel disorders, especially indigestion and irritable bowel syndrome; and
  • psychological impairment and mood problems.

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Cardiac-valvular EDS (cvEDS)

  • severe progressive heart valve problems (aortic valve, mitral valve);
  • skin problems, such as:
    • extremely stretchy skin;
    • wide, flat or depressed scars;
    • thin, fragile skin;
    • easy bruising; and
    • overly stretchy joints, in general or restricted to small joints.
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Vascular EDS (vEDS)

  • thin, translucent skin that is extremely fragile and bruises easily;
  • arteries and certain organs, such as the intestines and uterus, are also fragile and prone to rupture;

Typical characteristics include:

  • short build;
  • thin scalp hair; and
  • large eyes, a thin nose, and virtually no earlobes;
  • small joints, such as fingers and toes, are generally too stretchy.

Also common are:

  • club foot;
  • tendon and/or muscle rupture;
  • acrogeria (premature aging of the skin of the hands and feet);
  • early onset varicose veins;
  • pneumothorax (collapse of a lung);
  • recession of the gums; and
  • a decreased amount of fat under the skin.
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Arthrochalasia EDS (aEDS)

  • severe joint stretchiness; and
  • congenital hip dislocation.

Also common are:

  • fragile, stretchy skin with easy bruising;
  • low muscle tone or Floppy Baby Syndrome (FBS);
  • kyphoscoliosis (hunchback curvature of the spine with a left/right curvature of the spine); and
  • low bone density.
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Dermatosparaxis EDS (dEDS)

  • extremely fragile skin leading to severe bruising and scarring;
  • saggy, redundant skin, especially on the face; and
  • hernias.
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Kyphoscoliosis EDS (kEDS)

  • severely low muscle tone (Floppy Baby Syndrome) at birth;
  • delayed motor development;
  • progressive scoliosis (curvature of the spine), present from birth; and
  • fragility of the tough outer coat of the eye that covers the eyeball except for the cornea.

The following may also be present:

  • easy bruising;
  • fragile arteries that are prone to rupture;
  • unusually small corneas; and
  • low bone density.

Also possible are:

  • long, slender fingers (arachnodactyly);
  • unusually long limbs; and
  • a sunken chest (pectus excavatum) or protruding chest (pectus carinatum).
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Brittle Cornea Syndrome (BCS)

  • thin cornea;
  • early onset progressive corneal changes that result in a spherical, slightly enlarged eye; and
  • blue outer coat of the eye that covers the eyeball except for the cornea.
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Spondylodysplastic EDS (spEDS)

  • short build (progressive in childhood);
  • low muscle tone, ranging from severe congenital (Floppy Baby Syndrome) to mild later-onset; and
  • bowing of limbs.
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Musculocontractural EDS (mcEDS)

  • congenital multiple contractures (loss of joint motion);
  • clubfoot;
  • characteristic craniofacial features, which are evident at birth or in early infancy; and
  • skin features, such as extreme stretchiness, easy bruising, fragility with wide, flat or depressed scars, and increased wrinkling of the palms.
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Myopathic EDS (mEDS)

  • congenital low muscle tone or Floppy Baby Syndrome (FBS);
  • muscle atrophy, that improves with age;
  • loss of joint motion in the knee, hip and elbow; and
  • ankles, wrists, feet and hands that stretch more than they should.
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Periodontal EDS (pEDS)

  • severe and inflexible inflammation of the tissue around the teeth, often causing shrinkage of the gums and loosening of the teeth during childhood or adolescence;
  • lack of attached gums, pretibial plaques; and
  • family history of a first-degree relative who meets clinical criteria.
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