When Else to Suspect Ehlers-Danlos Syndrome

Beighton 9 pt scoring illustration
Bendy signs of hypermobility

Author’s note March 16, 2017: Since the brand new updated criteria and nosology for all forms of the now plural Ehlers-Danlos Syndromes (13 of them) and the brand new category of Hypermobility Spectrum Disorders JUST came out 24 hours ago, please bear with me as I get my site updated to reflect this.

Accordingly, please take the following with a grain of salt, and know that it is intended primarily to target those with the less rare form of EDS, hypermobile EDS or “hEDS” only now, as well as the new forms of the Hypermobility Spectrum Disorders primarily. 

Prior to March 15, 2017 we spoke of six main forms of EDS only, and some additional hypermobility syndromes (HMS, JHS, BJHS) which seemed to share a lot of overlap and confusing everyone. These have all been folded into either the newly sharpened EDS criteria, or the new categories of Hypermobility Spectrum Disorders for those who don’t meet the tightened Beighton criteria for hEDS as of March 15, 2017 for any reason but exhibit a lot of the following issues just the same, indicating some kind of systemic connective tissue disorder, after all clear ones have been excluded. (E.g. OI, Marfan, Stickler, etc.)

The more rare forms of the Ehlers-Danlos syndromes are enough easier to suspect and diagnose due to their severity that they have much less trouble doing so. Please consult The Ehlers-Danlos Society for more information on all forms (March 2017).

Original post pre-March 2017:

I wrote about my top five ways to suspect hypermobile Ehlers-Danlos Syndrome a couple of years ago. These included:

  1. Fibromyalgia
  2. IBS
  3. Chronic Pain
  4. Anxiety, depression and Autism Spectrum 
  5. Allergies and sensitivities or MCAS (a form of MCAD)

But I keep finding so many additional common signs and symptoms that people exhibit, that I felt them worthy of another post so am hatching this one. Though let me be clear, many of these are actually common comorbidities still, and none of them is truly diagnostic alone. Just part of the trail of breadcrumbs I hope you find leading you to suspect and look further into the EDS (and now HSD, March 2017).

However, rather than expounding on the signs I’ll just list them with a few links where able to outside sources about them. Again, because this is a systemic condition the myriad ways it manifests are rather mind-boggling in number and ultimately limitless: they affect the whole body in some way or another. Again this seems to be what makes it so hard for doctors to identify the condition prompting some wise person to quip:


If you can’t connect the issues, think connective tissues – author unknown*


*If you know the author, please tell me so I can give proper credit! And no it’s not Dr. Heidi Collins – she said she doesn’t know either. She just quoted them too! (She says so in her 2014 talk of the same title at the EDNF Conference.)

The thing is, doctors have only been told about or possibly only remember the grossest signs of the rarest types (of EDS and all HDCTs) for the most part, so many of us with less hypermobile or gross types keep getting missed still. Why I’m writing! Patreon support logo

Anyway, on with the unofficial and incomplete list. And if you find yourself checking off more than not, I would highly suspect you!

  • Postive Gorlin’s sign (ability to touch tip of tongue to nose, yes I think this case has EDS, natch)
  • Flying Bird Hand sign
  • W-sitting (don’t do it!)
  • Reverse Namaste or Namaskar sign (able to form the “Namaste” prayer pose with hands behind your back)
  • Standing on the sides of your feet as your ankles roll easily in, or out – pronation, anti-pronation
  • Severe myopia (near-sightedness) and or astigmatism
  • Nystagmus or repetitive uncontrolled eye movements (magnesium may help)
  • Chronic Blepharitis  or eyelid infections (likely from Selective IgA deficiency, part of commonly comorbid CVID)
  • Weak, crowded or cracked teeth (low Vitamin D contributes to the latter)
  • Peg teeth, or abnormal shaped teeth or microdontia
  • Receding gums that easily bleed even with good hygiene (Vitamin C can help this)
  • Sensitive teeth – this can come on as your ligaments loosen. (Mine tightened up and the sensitivity resolved with Vitamin C therapy yay.)
  • TMJ and all forms of jaw deformities, but especially high palate & crowding
  • Stretchy skin, ability to flip eyelids up (and freak out younger siblings)
  • Contortionist ability, or extreme flexibility in gymnastics or acrobatics
  • Weak ankles, trouble wearing high heel shoes, strong pronation or anti-pronation
  • Clumsiness and poor proprioception (sense of body location in space)
  • Weak knees, loose patellas, hyper-extended knees
  • Restless Leg Syndrome – some find magnesium to help ease this (not all)
  • One side much weaker or more injury-prone than the other, not always your dominant side either
  • Thin skin that tears easily and any scars that widen or grow or move
  • Fallen or falling arches, or any related issues (neuromas, plantar fasciitis)
  • Easy bruising
  • Keratosis pilaris – tiny red bumps that show in patches on upper arms, buttocks and more
  • Easy bleeding, trouble suturing, poor healing post surgery
  • “Dodgy” easily blown veins, often require ports for infusions
  • Any amount of CSF leaks anywhere along the dura, but especially out the nose or ears (yes, this happens! Suspect leaks if your headaches worsen the longer you are upright)
  • Deviated septum
  • Crossed eyes or wandering eyes from lax eye ligaments
  • Bulging veins, varicose veins (especially early onset)
  • Venous insufficiency
  • Herniations or prolapses of any and all kinds including hemorrhoids
  • Easy or frequent fainting or dizzy spells (likely from comorbid POTS and dysautonomia)
  • Raynaud’s phenomenon or syndrome, livedo reticularis (purple “mottling” of skin) and all other forms of dysautonomia
  • Chronic constipation with quick flips to diarrhea (IBS) – already mentioned in first post
  • Lots of allergies of any kind
  • Chemical sensitivity, multiple chemical sensitivity (likely really a form of MCAD)
  • Insomnia and adrenal fatigue
  • Sleep apnea, snoring, even in “thin” patients (weight doesn’t help, but we have weak laryngeal tissues), not to be confused with CNS apnea making you forget to breathe even in the daytime.
  • Chronic headaches of almost any kind, including migraines
  • Chronic idiopathic (unknown origin) nausea
  • Bad handwriting, lax hand writing, trouble holding pens or pencils properly, need for pencil “grip”
  • Chronic or recurrent infections, get sick easily or often fighting candida and viruses (likely from CVID or any other variations of primary immunodeficiency)
  • Chronic or frequent heart palpitations or fluttering, tachycardia (PVCs and POTS are common in us)
  • Chronic or frequent or multiple instances of tendonitis (aka tendinitis)
  • Bursitis, chondromalacia of any kind (loss of cartilage between bones of a joint, any joint, though usually knees aka chondromalacia patella)
  • Bone spurs (why this is true I don’t know, but they’re common in us)
  • Spinal stenosis (narrowing of the spinal canal between the vertebrae, impinging your spinal cord sometimes)
  • Easy flushing red (from MCAD)
  • Trouble swallowing from floppy laryngeal tissues and CCI and mild/occult Chiari
  • Hearing trouble from weak inner ear bones, weak tendons, or sensory processing disorder
  • Sensory Processing Disorder of any kind (including lack of pain in some lucky cases)
  • Poor temperature control (run hot or cold easily, usually cold) goes with dysautonomia
  • Chronic fatigue (may be misdiagnosed as Chronic Fatigue Syndrome aka ME / CFIDS / SEID)
  • Fibromyalgia (already mentioned, and very very common, usually an under-diagnosis for HEDS, may be worsened by comorbid occult MCAD of any kind too)
  • EMF sensitivity – and tendency to “blow” nearby electronics
  • Ganglion cysts, Baker’s cysts
  • Petechiae
  • Peizogenic papules
  • Lipomas and lipedema causing unavoidable weight gain most often in women
  • Osgood-Schlatter’s disease (knobby knees from rapid growth in childhood)
  • Leaky gut syndrome, dysbiosis
  • Hypochlorhydria or low stomach acid (unsure fully why, but it’s common and lends to dysbiosis and SIBO) – possibly from high cortisol and/or thyroid imbalance
  • SIBO – Small Intestinal Bacterial Overgrowth (likely aggravated by weak ileo-cecal valves in us allowing more to “back up” than should plus low stomach acid as mentioned)
  • Keratoconus (droopy or thin “pointy” corneas, hard to fit soft contacts)
  • Sensitive eyes, trouble wearing contacts
  • Weakness, mitochondrial disorders and disease
  • Seizures, hypnic jerks, myoclonus and actual epilepsy
  • PTSD, anxiety and panic disorders
  • Depression and mixed mood disorders of all kinds
  • Stretch marks or striae in any gender, not just after pregnancy in women
  • Pelvic floor dysfunction, prolapses (uterine or rectal, bladder, any)
  • Ptosis (drooping of anything, but most noticeably saggy breasts in women)
  • Drooping upper eyelid or eyebrow sometimes requiring an upper eye brow “tuck”
  • Sciatica
  • Claw finger or hammer toe or claw toe
  • Early onset arthritis of any kind, as well as any other auto-immune diseases (very common in us, likely secondary to leaky gut and chronic inflammation)
  • Paradoxic reactions to medications, strong or strange reactions, need for increased novocaine or lidocaine
  • Vocal cord dysfunction
  • Trouble swallowing (can have both neurologic and allergy-type drivers)

I’m stopping here for now, but will periodically add to this list. Many of these were already listed on the common comorbidities page, but I’m trying to add signs that in themselves are not full comorbidities, but just signs we find common in EDS and HSD patients. Alas, I’ve caved and ended up adding/sharing a couple that are driven by one of our most common comorbidities I find: MCAD.

And I will re-iterate that though many patients are quite flexible or “bendy”, and some even extremely so,  NOT ALL are very or even at all “bendy” or flexible! So do not consider yourself exempt just because you are not, sorry. Many of us stiffen with age and early onset arthritis, and some, like Dr. Jaime Bravo a leading rheumatologist in Chile with EDS himself told me never are in 2012.

Note March 16, 2017: They/you may now be getting re-classified as having a form of the new Hypermobility Spectrum Disorders instead of a form of EDS accordingly, but that remains to be seen. Check with your doctor using the new criteria guidelines from The Ehlers-Danlos Society please!

87 Comments

    Add a Comment

    Your email address will not be published. Required fields are marked *

    This site uses Akismet to reduce spam. Learn how your comment data is processed.