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Hi folks.. iwas doing some research.. and couldnt quite find the answer that i was looking for..I was wondering.. do all forms of EDS involve hypermobile joints? superflexible bendy parts?

Can you have a form of eds and not be flexible is waht i guess i am asking???

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Possibly, yes.

Dr. Grubb told me he thinks I have Joint Hypermobility Syndrome without significant joint involvement. My skin and blood vessels do fit the diagnosis, even though my joints mostly don't. BUT the possibility of such a form of JHS has NOT been formally studied, so it's still in the theory stage.

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The short answer would be no, not all types of EDS involve hypermobile joints. You should look at the EDNF website for diagnositic criteria. http://www.ednf.org/abouteds/index.php?opt...3&Itemid=31

text below is from the above page, but they have more detailed info on each disorder elsewhere on their site.



"There are six major types of EDS. The different types of EDS are classified according to the signs and symptoms that are manifested. Each type of EDS is a distinct disorder that "runs true" in a family. This means that an individual with Vascular Type EDS will not have a child with Classical Type EDS. More detailed information can be found in our Medical Professionals Section.

Hypermobility (Formerly EDS Type III)

Joint hypermobility is the dominant clinical manifestation. Generalized joint hypermobility that affects large (elbows, knees)and small (fingers and toes) joints is evident in the Hypermobility Type. Recurring joint subluxations and dislocations are common occurrences. Certain joints, such as the shoulder, patella, and temporomandibular joint dislocate frequently. The skin involvement (hyperextensibility and/or smooth velvety skin) as well as bruising tendencies in the Hypermobility Type are present but variable in severity.

Chronic joint and limb pain is a common complaint amongst individuals with the Hypermobility Type. Skeletal X-rays are normal. Musculoskeletal pain is early onset, chronic and may be debilitating. The anatomical distribution is wide and tender points can sometimes be elicited.

To date, no distinctive biochemical collagen finding has been identified by researchers. The Hypermobility Type of EDS is inherited in an autosomal dominant manner.

Classical (Formerly EDS Types I & II)

Marked skin hyperextensibility (stretchy) with widened atrophic scars and joint hypermobility are found in the Classical Type of EDS. The skin manifestations range in severity from mild to severe expression. The skin is smooth and velvety with the evidence of tissue fragility and easy bruisability. Examples of tissue extensibility and fragility include hiatal hernia, anal prolapse in childhood and cervical insufficiency. Hernias may be a post-operative complication. Scars are found mostly over pressure points such as the knees, elbows, forehead and chin. Molluscoid pseudo tumors (calcified hematomas) associated with scars are frequently found over pressure points such as the elbows, and spheroids (fat containing cysts) are usually found the on the forearms and shins.

Complications of joint hypermobility include sprains, dislocations/subluxations and pes planus (flat foot) to name a few. Recurrent joint subluxations are common in the shoulder, patella and temporomandibular joints. Muscle hypotonia and delayed gross motor development may also be evident.

Clinical Testing - Abnormal electrophoretic mobility of the proa1(V) or proa2(V) chains of collagen type V has been detected in several but not all families with the Classical Type. The Classical Type of EDS is inherited in an autosomal dominant manner.

Vascular (Formerly EDS Type IV)

This type is generally regarded as the most serious form of EDS due to the possibility of arterial or organ rupture. The skin is usually thin and translucent with veins being seen through the skin. This is most apparent over the chest and abdomen. There are certain facial characteristics present in some affected individuals. These manifestations include large eyes, thin nose, lobeless ears, short stature and thin scalp hair. Also evident is a decrease in subcutaneous tissue, particularly in the face and extremities. Minor trauma can lead to extensive bruising.

Arterial/intestinal/uterine fragility or rupture commonly arise in this type of EDS. Spontaneous arterial rupture has a peak incidence in the third or fourth decade of life, but may occur earlier. Midsize arteries are commonly involved. Arterial rupture is the most common cause of sudden death. Acute diffuse or localized abdominal or flank pain is a common presentation of arterial or intestinal rupture. Life expectancy is shortened with a majority of individuals living only into their forties. Pregnancies maybe complicated by intra-partum uterine rupture and pre- and postpartum arterial bleeding.

Joint hypermobility is usually limited to the digits. Tendon and muscle rupture can occur. Talipes equinovarus (clubfoot) is frequently seen at birth. Other manifestations that may be found in the Vascular Type include: acrogeria (premature aging of the skin of the hands and feet); early onset varicose veins; arteriovenousfistula (an opening between an artery and vein), carotid-cavernousfistula; pneumothorax (collapse of a lung) /pneumohemothorax (collapse of a lung with a collection of air or gas and blood); gingivalrecession and complications during and after surgery (i.e. wound dehiscence).

The Vascular Type of EDS is caused by structural defects in the proa1(III) chain of collagen type III encodes by COL3A1. This type of EDS is inherited in an autosomal dominant manner. A skin biopsy can diagnose this type of EDS.

Kyphoscoliosis (Formerly EDS Type VI)

Generalized joint laxity and severe muscle hypotonia (weak muscle tone) at birth are seen in this type of EDS. The muscular hypotonia can be very pronounced and leads to delayed gross motor development. Individuals with the Kyphoscoliosis Type present with Scoliosis at birth that is progressive. The phenotype is most often severe, frequently resulting in the loss of ambulation in the second or third decade. Scleral fragility may lead to rupture of the ocular globe after minor trauma.

Tissue fragility including atrophic scars and easy bruising may be seen in the Kyphoscoliosis Type. Spontaneous arterial rupture can occur. Other findings may include: marfanoid habitus (Marfan like features); micro cornea (abnormally small cornea); and radiologically considerable osteopenia (diminished amount of bone tissue).

Kyphoscoliosis Type EDS is the result of a deficiency of lysylhydroxylase (PLOD), which is a collagen-modifying enzyme. This type of EDS is inherited in an autosomal recessive manner. Kyphoscoliosis Type can be diagnosed through a urine test.

Arthrochalasia (Formerly EDS Type VII A&;)

Congenital hip dislocation has been present in all biochemically proven individuals with this type of EDS. Severe generalized joint hypermobility with recurrent subluxations are seen in individuals with this type of EDS. Other manifestations of this type may include: skin hyperextensibility with easy bruising; tissue fragility including atrophic scars; muscle hypotonia; Kyphoscoliosis and radiologically mild osteopenia.

The Arthrochalasia Type is caused by mutations leading to deficient processing of the amino-terminal end of proa1(I) [type A] or proa2(I)[type B] chains of collagen type I. It is inherited in an autosomal dominant manner. A skin biopsy can also diagnose this type of EDS.

Dermatosparaxis (Formerly EDS Type VIIC)

Individuals with Dermatosparaxis Type EDS have severe skin fragility and substantial bruising. Wound healing is not impaired and the scars are not atrophic. The skin texture is soft and doughy. Sagging, redundant skin is evident. The redundancy of facial skin results in an appearance resembling cutis laxa. Large hernias (umbilical, inguinal) may also be seen. The number of patients reported with this type of EDS is small.

Dermatosparaxis Type EDS is caused by a deficiency of procollagenI N-terminal peptidase. It is inherited in an autosomal recessive manner. A skin biopsy can diagnose this type of EDS.


The current EDS type V (X-linked) has been described in a single family. It is a rare variant and the molecular basis of which remains unknown.

The current EDS type VIII is similar to the Classical Type except that in addition it presents with periodontal friability. This is a rare type of EDS. The existence of this syndrome as an autonomous entity is uncertain.

The EDS type IX was previously redefined as "Occipital Horn syndrome", an X-linked recessive condition allelic to Menkes syndrome. This was previously removed from the EDS classification.

The current EDS type X has been described in only one family.

The EDS type XI termed "Familial Joint Hypermobility syndrome" was previously removed from the EDS classification. Its relationship to the EDS is not yet defined."

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We're pretty sure I have eds. My joints are unstable (subluxate, get injured easily, etc.), and some are hyperflexible, but many are not. Dr. Lavallee said I was one of the least flexible eds people he'd ever seen. My fingers don't bend in the ways they look for to diagnose eds, but still subluxate all the time. So my collagen is stiff but weak???

It's best to think of it as a syndrome. I think there are probably lots of variations, and until the genetics are better understood, it's all clinical diagnosis.

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  • 2 weeks later...

So, to get a diagnosis of EDS you would be evaluated clinically only? I thought my POTS specialist told me that one would be evaluated by a geneticist?

I am increasingly wondering if I have EDS. I mentioned this to my specialist and he said he does not suspect I have it. I do not think I have hypermobile joints--I have never had a dislocation or a sprain--but I was born with my hip dislocated. I do have arthritic knees I think. I have serious stretch marks after pregnancy (I did have a 10 lb baby). I had placental abruption at delivery. My skin is soft and I am fair and tall. My daughter is advanced in all areas except gross motor coordination and she did not walk until 18 months. I have a sister with POTS too.

Should I/we be evaluated and if so, what would I do with that information?


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No, it's not usually just a clinical evaluation--although for some folks it might be. Some of the genetically known types include a genetic screening. A geneticist could also help confirm or refute a diagnosis. Some types involve also skin an tissue biopsies.


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I had a clinical diagnosis from a geneticist-- they can only biopsy for vascular , 50% of classical, and perhaps some of the rarer types. I did have a negative biopsy, but that only ruled out vascular and one other mutation. They don't yet know much about all the types of collagen mutations. But a geneticist should still be able to diagnose.

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