{"id":5634,"date":"2025-12-10T11:53:41","date_gmt":"2025-12-10T11:53:41","guid":{"rendered":"https:\/\/regenerated.health\/eds-symptoms\/"},"modified":"2026-07-28T10:02:41","modified_gmt":"2026-07-28T10:02:41","slug":"eds-symptoms","status":"publish","type":"post","link":"https:\/\/regenerated.com\/blog\/eds-symptoms\/","title":{"rendered":"Ehlers-Danlos Syndrome Symptoms: What to Watch For"},"content":{"rendered":"\n<div class=\"at-a-glance\">\n<h2>At a Glance<\/h2>\n<ul>\n<li>Hypermobile EDS (hEDS) is the most common subtype, affecting an estimated 1 in 500 to 1 in 5,000 people<\/li>\n<li>Joint hypermobility alone doesn&#8217;t equal EDS. The diagnosis requires systemic involvement across multiple organ systems<\/li>\n<li>Common symptoms include chronic joint pain, frequent subluxations, skin fragility, severe fatigue, and GI dysfunction<\/li>\n<li>There is significant overlap with POTS (postural orthostatic tachycardia syndrome) and mast cell activation syndrome<\/li>\n<li>Average time to diagnosis is 10-12 years, partly because symptoms are often dismissed as anxiety or &#8220;just being flexible&#8221;<\/li>\n<\/ul>\n<\/div>\n\n<h2>What Is Ehlers-Danlos Syndrome?<\/h2>\n\n<p>Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders caused by defects in collagen or the proteins that interact with collagen. Since collagen is the most abundant protein in the human body, found in skin, joints, blood vessels, organs, and the GI tract, problems with it can cause symptoms almost everywhere [1].<\/p>\n\n<p>There are 13 recognized subtypes of EDS, but three account for the vast majority of diagnoses:<\/p>\n\n<ul>\n<li><strong>Hypermobile EDS (hEDS):<\/strong> The most common type. Primarily affects joints and soft tissue. No identified genetic mutation yet, which makes genetic testing impossible for this subtype.<\/li>\n<li><strong>Classical EDS (cEDS):<\/strong> Marked by extremely stretchy, fragile skin that bruises easily and heals with characteristic &#8220;cigarette paper&#8221; scars. Caused by mutations in COL5A1 or COL5A2 genes.<\/li>\n<li><strong>Vascular EDS (vEDS):<\/strong> The most dangerous subtype. Affects blood vessels and organs, raising the risk of arterial rupture and organ perforation. Caused by COL3A1 mutations. Estimated prevalence is 1 in 50,000 to 1 in 200,000 [2].<\/li>\n<\/ul>\n\n<p>This article focuses primarily on hEDS because it&#8217;s what most patients and practitioners will encounter. But knowing the other subtypes matters because some carry serious medical risks that require different management.<\/p>\n\n<h2>Joint Hypermobility: The Hallmark Symptom<\/h2>\n\n<h3>The Beighton Score<\/h3>\n\n<p>The Beighton score is a 9-point screening tool used to assess generalized joint hypermobility. You get points for:<\/p>\n\n<ol>\n<li>Passively extending each pinky finger beyond 90 degrees (1 point per hand)<\/li>\n<li>Bending each thumb to touch the forearm (1 point per hand)<\/li>\n<li>Hyperextending each elbow beyond 10 degrees (1 point per arm)<\/li>\n<li>Hyperextending each knee beyond 10 degrees (1 point per leg)<\/li>\n<li>Placing palms flat on the floor with knees straight (1 point)<\/li>\n<\/ol>\n\n<p>A score of 5 or higher in adults (4 or higher if over age 50) suggests generalized hypermobility [3]. But here&#8217;s the critical point: a high Beighton score alone does not mean you have EDS. Roughly 10-20% of the general population is hypermobile. Most of these people will never develop problems from it.<\/p>\n\n<h3>When Flexibility Becomes a Problem<\/h3>\n\n<p>The line between &#8220;benign&#8221; hypermobility and EDS gets crossed when joint looseness starts causing actual damage. In EDS, hypermobility leads to:<\/p>\n\n<ul>\n<li><strong>Chronic joint pain:<\/strong> Not the occasional ache after exercise. This is daily, persistent pain that worsens with activity and doesn&#8217;t respond well to standard treatments. It often starts in childhood or adolescence.<\/li>\n<li><strong>Recurrent subluxations and dislocations:<\/strong> Joints slip partially or fully out of place during normal activities. Shoulders, kneecaps, fingers, and jaws are frequent offenders. Some patients report subluxations from sneezing, reaching for something on a shelf, or rolling over in bed.<\/li>\n<li><strong>Joint instability:<\/strong> A constant feeling that joints are &#8220;loose&#8221; or might give out. Many patients describe their joints as feeling like they&#8217;re held together by rubber bands instead of ligaments.<\/li>\n<li><strong>Early osteoarthritis:<\/strong> Unstable joints wear down cartilage faster. It&#8217;s not unusual to see degenerative changes in the 20s and 30s in EDS patients [4].<\/li>\n<\/ul>\n\n<h2>Skin Symptoms<\/h2>\n\n<p>Skin involvement varies by subtype, but even in hEDS, you&#8217;ll often see some of the following:<\/p>\n\n<ul>\n<li><strong>Hyperextensibility:<\/strong> Skin stretches beyond what&#8217;s normal for the body site. In classical EDS, skin can be pulled several centimeters away from the body. In hEDS, it may be subtler but still noticeable.<\/li>\n<li><strong>Easy bruising:<\/strong> Bruises appear with minimal or no recalled trauma. Some patients wake up with new bruises regularly.<\/li>\n<li><strong>Delayed wound healing:<\/strong> Cuts and surgical incisions take longer to close and are more prone to widening over time.<\/li>\n<li><strong>Atrophic scarring:<\/strong> Scars that are thin, widened, and often described as looking like &#8220;tissue paper.&#8221; Most prominent in classical EDS.<\/li>\n<li><strong>Soft, velvety skin texture:<\/strong> Described by clinicians as having a &#8220;doughy&#8221; quality. This is actually a clinical finding that can help point toward an EDS diagnosis.<\/li>\n<li><strong>Stretch marks (striae):<\/strong> Appearing at a young age and in unusual locations, not just from rapid weight change or pregnancy.<\/li>\n<\/ul>\n\n<h2>Fatigue: The Symptom Nobody Expects<\/h2>\n\n<p>If you ask EDS patients what symptom affects their quality of life the most, many won&#8217;t say joint pain. They&#8217;ll say fatigue.<\/p>\n\n<p>The fatigue in EDS is not ordinary tiredness. It&#8217;s a bone-deep exhaustion that doesn&#8217;t fully resolve with rest or sleep. Research shows that fatigue severity in hEDS is comparable to what&#8217;s seen in chronic fatigue syndrome and multiple sclerosis [5]. Several factors contribute:<\/p>\n\n<ul>\n<li><strong>Muscle overwork:<\/strong> When ligaments can&#8217;t stabilize joints properly, muscles have to work overtime to compensate. This creates constant low-grade muscle fatigue.<\/li>\n<li><strong>Poor sleep quality:<\/strong> Joint pain, difficulty finding comfortable positions, and frequent waking all disrupt sleep architecture.<\/li>\n<li><strong>Autonomic dysfunction:<\/strong> Many EDS patients have dysautonomia (especially POTS), which causes blood pressure and heart rate instability. The body expends enormous energy just trying to maintain basic cardiovascular regulation.<\/li>\n<li><strong>Chronic pain itself:<\/strong> Pain is neurologically exhausting. The central nervous system processing required to manage constant pain signals drains energy reserves.<\/li>\n<li><strong>Mast cell activation:<\/strong> A significant subset of EDS patients have concurrent mast cell activation syndrome (MCAS), which causes inflammatory mediator release and its own fatigue burden.<\/li>\n<\/ul>\n\n<h2>Dysautonomia and Cardiovascular Symptoms<\/h2>\n\n<p>Up to 80% of hEDS patients meet criteria for some form of dysautonomia, and POTS is the most common presentation [6]. The connection makes anatomical sense: if your blood vessel walls contain faulty collagen, they&#8217;re more compliant (stretchy) than they should be. Blood pools in the legs and abdomen when you stand up, your heart rate spikes to compensate, and you feel dizzy, lightheaded, or like you might pass out.<\/p>\n\n<p>Common dysautonomia symptoms in EDS include:<\/p>\n\n<ul>\n<li>Dizziness or lightheadedness when standing<\/li>\n<li>Heart palpitations and racing heart rate<\/li>\n<li>Exercise intolerance that seems out of proportion to fitness level<\/li>\n<li>Heat intolerance and excessive sweating (or inability to sweat normally)<\/li>\n<li>Difficulty regulating body temperature<\/li>\n<li>Brain fog and difficulty concentrating<\/li>\n<li>Pre-syncope (feeling like you&#8217;re about to faint) or actual fainting<\/li>\n<\/ul>\n\n<p>If you&#8217;ve been told you have anxiety but your &#8220;panic attacks&#8221; happen when you stand up, start with a tilt table test or a simple active stand test before accepting a psychiatric diagnosis. Learn more in our guide to <a href=\"\/blog\/eds-and-pots\">EDS and POTS overlap<\/a>.<\/p>\n\n<h2>GI Symptoms: The Gut Connection<\/h2>\n\n<p>Gastrointestinal problems are reported by 50-75% of EDS patients, yet this is one of the most under-recognized parts of the condition. The GI tract is essentially a tube made of connective tissue and smooth muscle, so it makes sense that collagen defects would affect gut function [7].<\/p>\n\n<p>Common GI manifestations include:<\/p>\n\n<ul>\n<li><strong>Gastroparesis (delayed stomach emptying):<\/strong> Food sits in the stomach longer than normal, causing nausea, early fullness, bloating, and sometimes vomiting. Some EDS patients are misdiagnosed with eating disorders because of their inability to eat normal portions.<\/li>\n<li><strong>Functional dyspepsia:<\/strong> Chronic upper abdominal pain, burning, or discomfort without an identifiable structural cause on endoscopy.<\/li>\n<li><strong>IBS-like symptoms:<\/strong> Alternating constipation and diarrhea, abdominal cramping, and bloating. Many EDS patients carry an IBS diagnosis for years before the underlying connective tissue disorder is identified.<\/li>\n<li><strong>Gastroesophageal reflux (GERD):<\/strong> A lax lower esophageal sphincter allows acid to reflux more easily. Some EDS patients also have hiatal hernias at young ages.<\/li>\n<li><strong>Rectal prolapse and pelvic floor dysfunction:<\/strong> Lax connective tissue in the pelvic floor can lead to organ prolapse, difficulty with bowel movements, and urinary issues.<\/li>\n<\/ul>\n\n<p>If you&#8217;re being treated for IBS that isn&#8217;t responding to standard therapy, and you also have joint flexibility or chronic pain, bring up the possibility of an underlying connective tissue disorder with your gastroenterologist.<\/p>\n\n<h2>Other Symptoms You Might Not Connect to EDS<\/h2>\n\n<p>EDS is genuinely a whole-body condition. Symptoms that patients and doctors frequently overlook include:<\/p>\n\n<ul>\n<li><strong>TMJ dysfunction:<\/strong> The temporomandibular joint is particularly susceptible to instability in EDS. Jaw clicking, locking, and pain are common. Some patients can sublux their jaw while yawning or eating.<\/li>\n<li><strong>Dental crowding and high-arched palate:<\/strong> Connective tissue abnormalities affect craniofacial development.<\/li>\n<li><strong>Myopia (nearsightedness):<\/strong> The sclera (white of the eye) is made of collagen. Lax scleral tissue can change the shape of the eye.<\/li>\n<li><strong>Chronic headaches:<\/strong> A combination of cervical instability, TMJ dysfunction, and muscle tension. Some EDS patients also develop intracranial hypertension or CSF leak headaches.<\/li>\n<li><strong>Easy muscle tears and tendinopathy:<\/strong> Tendons and muscles are more injury-prone.<\/li>\n<li><strong>Anxiety and panic attacks:<\/strong> While there may be a true neurological component, many patients diagnosed with anxiety actually have unrecognized POTS or mast cell activation. The physical symptoms (racing heart, breathlessness, dizziness) get mislabeled [8].<\/li>\n<li><strong>Proprioception deficits:<\/strong> Reduced awareness of where joints are in space. This contributes to clumsiness, frequent tripping, and difficulty with balance.<\/li>\n<\/ul>\n\n<h2>The Other EDS Subtypes: A Brief Overview<\/h2>\n\n<p>While hEDS dominates the conversation, other subtypes carry their own distinct symptom profiles:<\/p>\n\n<ul>\n<li><strong>Classical EDS:<\/strong> Extreme skin extensibility, severe scarring, generalized hypermobility. The skin involvement is what sets it apart from hEDS.<\/li>\n<li><strong>Vascular EDS:<\/strong> Thin, translucent skin (veins visible through the skin), characteristic facial features, and risk of spontaneous arterial or organ rupture. This subtype requires specialized monitoring because of life-threatening complications. Median life expectancy was historically around 50 years, though improving with surveillance [2].<\/li>\n<li><strong>Kyphoscoliotic EDS:<\/strong> Severe muscle weakness at birth, progressive scoliosis, and ocular fragility.<\/li>\n<li><strong>Arthrochalasia and dermatosparaxis EDS:<\/strong> Extremely rare subtypes with severe skin or joint involvement.<\/li>\n<\/ul>\n\n<p>If you have signs of classical or vascular EDS, genetic testing is available and recommended. These subtypes have identified gene mutations and confirmed genetic tests.<\/p>\n\n<h2>Why Diagnosis Takes So Long<\/h2>\n\n<p>The average time from symptom onset to EDS diagnosis is 10 to 12 years. Some patients wait two decades or more. This delay isn&#8217;t just frustrating; it&#8217;s harmful. Years of improper treatment, unnecessary surgeries, and psychological damage from being told &#8220;nothing is wrong&#8221; take a real toll [9].<\/p>\n\n<p>Several factors contribute to the diagnostic delay:<\/p>\n\n<ul>\n<li><strong>Symptom fragmentation:<\/strong> Patients see a rheumatologist for joint pain, a gastroenterologist for GI symptoms, a cardiologist for palpitations, a dermatologist for bruising, and a psychiatrist for &#8220;anxiety.&#8221; No single specialist sees the full picture.<\/li>\n<li><strong>Medical education gaps:<\/strong> EDS gets about five minutes of coverage in most medical school curricula. Many physicians have never diagnosed it and may not recognize it.<\/li>\n<li><strong>The &#8220;you look fine&#8221; problem:<\/strong> EDS patients typically don&#8217;t look sick. They&#8217;re often young women (the most common demographic) who appear healthy and active. Invisible illness is easy to dismiss.<\/li>\n<li><strong>Diagnostic criteria complexity:<\/strong> The 2017 international criteria for hEDS are rigorous and involve a three-part checklist that many clinicians aren&#8217;t familiar with. Unlike other subtypes, there&#8217;s no blood test or genetic test for hEDS [3].<\/li>\n<li><strong>Normalization of symptoms:<\/strong> Patients who&#8217;ve been hypermobile since childhood often don&#8217;t realize their experience is abnormal. They assume everyone&#8217;s joints hurt. They think dislocating a shoulder during gym class is just bad luck.<\/li>\n<\/ul>\n\n<h3>The Diagnostic Criteria Debate<\/h3>\n\n<p>The 2017 criteria for hEDS were intended to create more consistent diagnosis, but they&#8217;ve generated controversy. Some clinicians argue the criteria are too restrictive, missing patients with significant symptoms who fall just short of meeting threshold. Others say they&#8217;re necessary to prevent over-diagnosis [10].<\/p>\n\n<p>The reality is that many patients fall into a gray zone called &#8220;hypermobility spectrum disorder&#8221; (HSD). HSD shares many symptoms with hEDS but doesn&#8217;t meet the full 2017 criteria. Importantly, HSD can be just as disabling as hEDS and requires the same treatment approach. Whether you technically meet hEDS criteria or fall under HSD shouldn&#8217;t change how your symptoms are managed.<\/p>\n\n<h2>When to Seek Evaluation<\/h2>\n\n<p>Consider pursuing an EDS evaluation if you have a combination of:<\/p>\n\n<ul>\n<li>Generalized joint hypermobility (Beighton score of 5+ or a history of flexibility)<\/li>\n<li>Chronic musculoskeletal pain that started before age 30<\/li>\n<li>Recurrent joint subluxations or dislocations<\/li>\n<li>Unusual skin features (hyperextensibility, easy bruising, atrophic scars)<\/li>\n<li>A first-degree relative with confirmed or suspected EDS<\/li>\n<li>Unexplained fatigue, GI dysfunction, or dysautonomia symptoms<\/li>\n<li>Multiple seemingly unrelated diagnoses across different specialties<\/li>\n<\/ul>\n\n<p>The best starting points for evaluation are a geneticist with connective tissue expertise or a rheumatologist familiar with EDS. The Ehlers-Danlos Society maintains a provider directory that can help you find someone experienced with these conditions.<\/p>\n\n<h2>Related Reading<\/h2>\n\n<ul>\n<li><a href=\"\/blog\/eds-guide\/\">Ehlers-Danlos Syndrome: The Complete Guide<\/a><\/li>\n<li><a href=\"\/blog\/eds-treatment\">EDS Treatment: Managing Hypermobility and Pain<\/a><\/li>\n<li><a href=\"\/blog\/eds-and-pots\">EDS and POTS: Why These Two Conditions Often Overlap<\/a><\/li>\n<li><a href=\"\/blog\/dysautonomia-symptoms\">Dysautonomia Symptoms: Recognizing Autonomic Dysfunction<\/a><\/li>\n<li><a href=\"\/blog\/chronic-fatigue-treatment\">Chronic Fatigue Treatment: Evidence-Based Approaches<\/a><\/li>\n<\/ul>\n\n<h2>References<\/h2>\n\n<ol>\n<li>Malfait F, Francomano C, Byers P, et al. The 2017 international classification of the Ehlers-Danlos syndromes. <em>Am J Med Genet C Semin Med Genet.<\/em> 2017;175(1):8-26. doi:10.1002\/ajmg.c.31552<\/li>\n<li>Pepin M, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. <em>N Engl J Med.<\/em> 2000;342(10):673-680. doi:10.1056\/NEJM200003093421001<\/li>\n<li>Castori M, Tinkle B, Levy H, et al. A framework for the classification of joint hypermobility and related conditions. <em>Am J Med Genet C Semin Med Genet.<\/em> 2017;175(1):148-157. doi:10.1002\/ajmg.c.31539<\/li>\n<li>Rombaut L, Malfait F, De Paepe A, et al. Impairment and impact of pain in female patients with Ehlers-Danlos syndrome: a comparative study with fibromyalgia and rheumatoid arthritis. <em>Arthritis Rheum.<\/em> 2011;63(7):1979-1987. doi:10.1002\/art.30337<\/li>\n<li>Voermans NC, Knoop H, Bleijenberg G, van Engelen BG. Fatigue is a frequent and clinically relevant problem in Ehlers-Danlos syndrome. <em>Semin Arthritis Rheum.<\/em> 2010;40(3):267-274. doi:10.1016\/j.semarthrit.2009.08.003<\/li>\n<li>De Wandele I, Rombaut L, Ber Lennart, et al. Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers-Danlos syndrome. <em>Semin Arthritis Rheum.<\/em> 2014;44(1):93-100. doi:10.1016\/j.semarthrit.2013.12.006<\/li>\n<li>Fikree A, Chelimsky G, Collins H, et al. Gastrointestinal involvement in the Ehlers-Danlos syndromes. <em>Am J Med Genet C Semin Med Genet.<\/em> 2017;175(1):181-187. doi:10.1002\/ajmg.c.31546<\/li>\n<li>Bulbena A, Baeza-Velasco C, Bulbena-Cabre A, et al. Psychiatric and psychological aspects in the Ehlers-Danlos syndromes. <em>Am J Med Genet C Semin Med Genet.<\/em> 2017;175(1):237-245. doi:10.1002\/ajmg.c.31544<\/li>\n<li>Demmler JC, Atkinson MD, Mayberry JF, et al. Diagnosed prevalence of Ehlers-Danlos syndrome and hypermobility spectrum disorder in Wales, UK: a national electronic cohort study and case-control comparison. <em>BMJ Open.<\/em> 2019;9(11):e031365. doi:10.1136\/bmjopen-2019-031365<\/li>\n<li>Tinkle B, Castori M, Berglund B, et al. Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome type III and Ehlers-Danlos syndrome hypermobility type): clinical description and natural history. <em>Am J Med Genet C Semin Med Genet.<\/em> 2017;175(1):48-69. doi:10.1002\/ajmg.c.31538<\/li>\n<\/ol>\n","protected":false},"excerpt":{"rendered":"<p>Ehlers-Danlos syndrome is far more than being &#8220;double-jointed.&#8221; Learn how to recognize the full spectrum of EDS symptoms, from joint instability and skin changes to fatigue and GI dysfunction, and why diagnosis often takes over a decade.<\/p>\n","protected":false},"author":1,"featured_media":6217,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_kad_post_transparent":"","_kad_post_title":"","_kad_post_layout":"","_kad_post_sidebar_id":"","_kad_post_content_style":"","_kad_post_vertical_padding":"","_kad_post_feature":"","_kad_post_feature_position":"","_kad_post_header":false,"_kad_post_footer":false,"_kad_post_classname":"","_regenerated_references":"","footnotes":""},"categories":[26],"tags":[],"class_list":["post-5634","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-connective-tissue-eds"],"_links":{"self":[{"href":"https:\/\/regenerated.com\/blog\/wp-json\/wp\/v2\/posts\/5634","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/regenerated.com\/blog\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/regenerated.com\/blog\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/regenerated.com\/blog\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/regenerated.com\/blog\/wp-json\/wp\/v2\/comments?post=5634"}],"version-history":[{"count":2,"href":"https:\/\/regenerated.com\/blog\/wp-json\/wp\/v2\/posts\/5634\/revisions"}],"predecessor-version":[{"id":6900,"href":"https:\/\/regenerated.com\/blog\/wp-json\/wp\/v2\/posts\/5634\/revisions\/6900"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/regenerated.com\/blog\/wp-json\/wp\/v2\/media\/6217"}],"wp:attachment":[{"href":"https:\/\/regenerated.com\/blog\/wp-json\/wp\/v2\/media?parent=5634"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/regenerated.com\/blog\/wp-json\/wp\/v2\/categories?post=5634"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/regenerated.com\/blog\/wp-json\/wp\/v2\/tags?post=5634"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}