How rare is vascular eds. Each type is caused by pathogenic genetic variant...
How rare is vascular eds. Each type is caused by pathogenic genetic variants that prevent connective tissue from functioning properly. It is characterized by serious complications such as spontaneous arterial dissections, aneurysms, ruptures, and gastrointestinal perforations, which begin in early adulthood. Find expert insights and prevention tips. The current EDS classification distinguishes 13 We would like to show you a description here but the site won’t allow us. The diagnosis of vEDS is established in a proband by identification of a heterozygous Sep 19, 2025 · Vascular Ehlers-Danlos is another form of EDS, which fortunately is relatively rare. We delineate a novel form of EDS with vascular features Jul 30, 2020 · The Ehlers–Danlos syndromes are a group of genetically heterogeneous connective tissue disorders with a wide range of clinical manifestations. Vascular Ehlers-Danlos syndrome People who have vascular Ehlers-Danlos syndrome often share distinctive facial features of a thin nose, thin upper lip, small earlobes and prominent eyes. Twelve types of EDS have known genetic causes, and some types are associated with multiple different genes. Ehlers-Danlos syndrome (EDS) is a group of genetic disorders that affect the connective tissues. Common symptoms include thin, translucent skin; easy bruising; characteristic facial appearance; and fragile arteries, muscles and internal organs. Ehlers-Danlos syndrome type IV, also known as the vascular type of Ehlers-Danlos syndrome (EDS), is an inherited connective tissue disorder defined by characteristic facial features (acrogeria) in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive Ehlers–Danlos syndromes (EDS) are a group of heritable connective tissue disorders (HCTDs) characterized by a variable degree of skin hyperextensibility, joint hypermobility and tissue fragility. 4 days ago · The pattern is similar in vascular EDS: a study of 177 people with vEDS found that roughly 51% had no family history, pointing to a de novo mutation. Vascular EDS (vEDS) is an inherited connective tissue disorder caused by Aortic root dilation affects 15-20% of adults with hEDS, where the aorta's base enlarges mildly (usually non-progressive), more noticeable in men and with age; vascular EDS carries graver artery risks. People with this subtype of EDS have very fragile arteries and The prevalence of the Ehlers-Danlos syndromes (EDS) and hypermobility spectrum disorders (HSD) Our community may read different commentaries about prevalence figures for EDS and HSD. Vascular EDS is usually caused by a Apr 15, 2019 · Vascular Ehlers-Danlos syndrome (vEDS) is a rare genetic connective tissue disorder secondary to pathogenic variants within the COL3A1 gene, resulting in exceptional arterial and organ fragility and premature death. Managing and monitoring What is Ehlers-Danlos Syndrome? The Ehlers-Danlos syndromes (EDS) are a group of varied, heritable connective tissue disorders affecting the quality of collagen and other connective tissues in the body. [8] Symptoms often include loose joints, joint pain, stretchy, velvety skin, and abnormal scar formation. D. Oct 9, 2025 · Learn about Ehlers Danlos Syndrome, including its symptoms, causes, risk factors, and available treatment options. In rare cases (2 to 3%), a parent carries the mutation in only some of their cells, a phenomenon called mosaicism, which can make family history misleading. While extremely rare, it’s also especially severe. Patients with these defects frequently present at a young age with spontaneous arterial complications involving the The Center for Vascular Ehlers-Danlos Syndrome Research Director: Hal Dietz, M. Jan 9, 2025 · The Ehlers-Danlos syndromes (EDS) are rare inherited conditions. Nov 6, 2024 · Outlook (Prognosis) People with EDS generally have a normal life span. We would like to show you a description here but the site won’t allow us. Ehlers-Danlos syndrome is a group of disorders that affect connective tissues supporting the skin, bones, blood vessels, and many other organs and tissues. This group of genetic connective tissue conditions is commonly characterized by unstable, hypermobile joints, loose, “stretchy” skin, and fragile tissues. Ehlers-Danlos syndrome (EDS) most often presents with the classic symptoms of skin hyperelasticity, hypermobility of joints, atrophic scarring, and fragility of blood vessels. Background The Ehlers-Danlos syndromes (EDS) are heritable disorders of connective tissue (HDCT), reclassified in the 2017 nosology into 13 subtypes. Most feature joint hypermobility. This rare condition is caused by pathogenic variants in the COL3A1 gene and affects the digestive system, the uterus, the skin, and the Abstract Ehlers-Danlos Syndrome (EDS) is a family of multisystemic hereditary connective tissue disorders now comprised of 13 recognized subtypes, classical, classical-like, cardiac-valvular, vascular, hypermobile, arthrochlasia, dermosparaxis, kyphoscoliotic, brittle cornea syndrome, spondylodysplastic, musculocontractural, myopathic, and periodontal, as designated by the most recent 2017 Ehlers-Danlos syndrome is a diverse connective tissue condition that has thirteen different subtypes. However, some subtypes, such as Vascular Ehlers-Danlos and Kyphoscoliotic Ehlers-Danlos, are prone to life-threatening arterial Apr 16, 2024 · BACKGROUND: Vascular Ehlers-Danlos syndrome (vEDS) is a rare connective tissue disorder with a high risk for arterial, bowel, and uterine rupture, caused by heterozygous pathogenic variants in COL3A1. Unlike other EDS subtypes primarily characterized by joint hypermobility and skin elasticity, vEDS is distinguished by its impact on the vascular system, posing a significant risk of life-threatening complications. They also have thin, translucent skin that bruises very easily. Is Ehlers-Danlos syndrome really so rare? Hypermobile EDS challenges the perception of EDS as a rare condition due to its estimated prevalence of up to 1 in 500 individuals. Most types of EDS affect joints and skin. These are all considered rare. Find out about the symptoms, causes and treatments. Aug 25, 2022 · A more severe form of the disorder, called vascular Ehlers-Danlos syndrome, can cause the walls of your blood vessels, intestines or uterus to rupture. However, many other subtypes, such as classical or vascular EDS, remain rare, with prevalence rates significantly lower than 1 in 10,000. Vascular EDS is usually caused by a A comprehensive, evidence-based guide to hypermobility and Ehlers-Danlos syndrome. Mar 4, 2024 · Ehlers–Danlos syndromes (EDS) are a group of connective tissue disorders caused by mutations in collagen and collagen-interacting genes. Sep 1, 2022 · Abstract Vascular Ehlers-Danlos syndrome (vEDS) is a rare genetic disorder clinically characterized by vascular, intestinal and uterine fragility and caused by heterozygous pathogenic variants in the COL3A1 gene. Sep 17, 2025 · Ehlers-Danlos syndrome is a group of rare genetic disorders. Ehlers-Danlos syndrome is also referred to as EDS or Ehlers-Danlos disease, and is a largely inherited disease. Spondylodysplastic EDS (spEDS) Vascular EDS (vEDS) #TogetherWeDazzle Sign up to The Ehlers-Danlos Society mailing list Click here to sign up Donate Vascular Ehlers-Danlos syndrome is also referred to as vEDS, Ehlers-Danlos syndrome type IV, Sack-Barabas syndrome, and the arterial form of Ehlers-Danlos syndrome. In fair-skinned people, the underlying blood vessels are very visible through the skin. In children with marked Learn about Vascular Ehlers-Danlos Syndrome (vEDS) and it's signs and symptoms. The Ehlers-Danlos syndromes are a group of heritable connective tissue disorders. Covers diagnosis, symptoms, comorbidities, exercise, nutrition, and daily management. Vascular complications, though rare in classical EDS, can be life-threatening, and this necessitates one to look for vascular associations in Ehlers-Danlos syndrome (EDS) is a genetic connective tissue disorder that affects how collagen is made and structured in the body. Rarer Types, Such as Vascular EDS, Have More Resources Today Vascular Ehlers-Danlos Syndrome (vEDS) is a rare subtype of EDS that affects the blood vessels and organs, leading to a higher risk of severe and life-threatening complications. See Subtypes of Ehlers–Danlos syndrome What causes Ehlers–Danlos syndrome? The Ehlers–Danlos syndromes comprise a clinically and genetically heterogeneous group of heritable connective tissue disorders, which are characterized by joint hypermobility, skin hyperextensibilit These mostly rare and inherited conditions number 13 types, with hypermobile, classical, and vascular EDS being the most common. Managing and monitoring In rare cases, genetic testing does not confirm the diagnosis and a skin biopsy to look at the collagen fibres with an electron microscope may help clarify whether vascular EDS is the correct diagnosis. What causes Ehlers-Danlos syndrome? Nov 19, 2015 · The vascular type is rarer, affecting 1 in 250,000 people. Most children with EDS have hypermobile joints that are prone to full or partial dislocation. The aim of this cohort study is to provide further insights into the natural history of vEDS and describe genotype-phenotype correlations in a Dutch multicenter cohort to optimize patient care Mar 29, 2023 · The UK National Diagnostic Service for Ehlers-Danlos Syndromes (EDS) was established in 2009 for the rare types of EDS. Alan Hakim, Consultant Rheumatologist, Acute Physician, and Director of Education for The Ehlers-Danlos Society. The only published clinical trial to date demonstrated the benefit of celiprolol on arterial morbimortality. We hope the following is a helpful summary. Intelligence is normal. Identification of a disease-causing variant may assist with diagnosis, prognosis, clinical management, familial screening, and genetic counseling for various forms of Ehlers-Danlos syndrome and related conditions. Vascular EDS (vEDS): Marked by vascular and organ fragility. Dec 27, 2024 · Vascular Ehlers-Danlos syndrome (vEDS, OMIM #130050) is a rare autosomal dominant connective tissue disorder characterised by arterial and intestinal fragility and severe tissue friability, with What is vascular Ehlers-Danlos Syndrome? Vascular Ehlers-Danlos Syndrome (vEDS) is a rare disorder that weakens the body’s connective tissue. Vascular Ehlers Danlos syndrome (vEDS) is a rare disorder, estimated to affect between 1 in 50,000 and 1 in 200,000 people. Dermatosparaxis EDS is an example of an extremely rare type of EDS, with only a few cases recorded in medical literature. Sep 1, 2025 · "This is my daughter Ella travelling back from holidays wearing her sunflower lanyard from Annabelle's Challenge Vascular EDS Charity🌻 She was diagnosed with Vascular EDS last year, a rare genetic condition that can be life-threatening. These people have a high risk for sudden death. Apr 14, 2022 · Vascular Ehlers-Danlos is a more severe form of the disorder that affects blood vessels. Connective tissues are proteins, such as collagen, that provide elasticity and support to the joints, blood vessels, and skin. Learn more about EDS at Boston Children’s. The other types of EDS are very rare (less than 10%). Prevalence in the Population EDS and Marfan Syndrome are both rare conditions, though their exact prevalence varies: EDS affects 1 in 5,000 to 1 in 20,000 people globally. Feb 13, 2020 · Other types of EDS are very rare, with only a few cases reported worldwide. It causes elastic skin, unusual joint movement, and hyperfragility in many tissues. By pursuing the most innovative research, educating the medical community, general public and affected individuals, and providing support to patients, families, and caregivers, we can charge forward and improve the outcomes for those living with VEDS. Ehlers-Danlos syndrome affects tissues that support blood vessels, bones, skin, and other organs. Vascular EDS (vEDS), due to deleterious variants in COL3A1, is one of the most commo … Vascular Ehlers-Danlos syndrome (VEDS) is a genetic condition that makes the arteries and hollow organs prone to tear, due to a mutation in the COL3A1 gene. There is no cure for EDS, but symptoms are managed Key Takeaways Ehlers-Danlos syndrome encompasses 13 distinct types affecting connective tissue throughout the body Hypermobility EDS is just one type—other forms can involve skin fragility, vascular complications, and organ dysfunction EDS affects multiple body systems including cardiovascular, gastrointestinal, and neurological functions Early diagnosis and multidisciplinary care Ehlers-Danlos syndromes (EDS) are a group of rare inherited conditions that affect connective tissue. It can affect your skin, joints, muscles, blood vessels, organs and bones. It is inherited in an autosomal dominant way which means that if a person has vEDS there is a 50% (1 in 2) chance that the condition will passed on to a child in each pregnancy. Dr. The endothelial tissue that lines the blood vessels, intestines, and uterus is prone to easily rupturing. It gets its strength from a protein called collagen. Spontaneous coronary artery dissection (SCAD) is a cause of acute myocardial infarction predominantly affecting adult women. Explore symptoms, inheritance, genetics of this condition. Dec 27, 2024 · Vascular Ehlers-Danlos syndrome (vEDS, OMIM #130050) is a rare autosomal dominant connective tissue disorder characterised by arterial and intestinal fragility and severe tissue friability, with Disease information Ehlers-Danlos Syndrome (EDS) is a group of genetic disorders that affect the connective tissues. Feb 1, 2026 · The Marfan Foundation drives research, education, and support – and builds community – to improve outcomes, save lives, and empower all people to thrive who are living with Marfan, Loeys-Dietz, Vascular Ehlers-Danlos syndromes and related genetic aortic and vascular conditions. Apr 15, 2019 · Of all hereditary connective tissue diseases, vascular Ehlers-Danlos Syndrome (vEDS) is pre-eminently the most feared—mainly due to the unpredictability in the occurrence of potentially serious/fatal vascular events such as arterial dissection or rupture. Main differential diagnosis are other forms of Ehlers-Danlos syndromes, notably classical EDS (including COL1A1 variants leading to substitutions of arginine to cysteine residues in the triple helical domain), kyphoscoliotic EDS and periodontal EDS. The new framework will be published on December 1, 2026, in the internationally renowned journal, the American Journal of Medical Genetics, a leading global publication for genetic and Apr 15, 2021 · Hypermobile Ehlers-Danlos syndrome (EDS) and hypermobility spectrum disorders are the most common symptomatic joint hypermobility conditions seen in clinical practice. Those with the rare vascular type of EDS are at greater risk for rupture of a major organ or blood vessel. Feb 23, 2016 · Vascular Ehlers-Danlos syndrome (EDS) is a relatively rare genetic syndrome that occurs owing to disorders in the metabolism of fibrillary collagen. A 14th subtype was identified in 2018. Vascular EDS is rarer, occurring in 1 in 50,000 to 200,000. What is the mildest form of EDS? Hypermobility EDS (hEDS) is often considered the mildest form in terms of life-threatening complications, as it does not typically involve significant vascular or organ fragility. 🤍 Because collagen is a major building block of ligaments, tendons, skin, and other tissues, changes in its quality can lead to increased joint laxity and hypermobility. ” states Assoc. There are 13 types of this condition, with vascular EDS being type IV. These tissues are also known as connective tissues. Mar 29, 2023 · The UK National Diagnostic Service for Ehlers-Danlos Syndromes (EDS) was established in 2009 for the rare types of EDS. In the case of vascular EDS, there is an autosomal dominant inheritance pattern, resulting from mutations in the COL3A1 gene. Vascular Ehlers-Danlos syndrome is an inherited connective tissue disorder that is caused by defects in a protein called collagen. [1][4] The existing 6 days ago · What are the main causes of reduced life expectancy in people with EDS? The primary reason for reduced life expectancy in individuals with certain types of Ehlers-Danlos Syndromes, most notably Vascular EDS (vEDS), is the increased risk of life-threatening complications due to fragile connective tissues. The condition is characterized by aneurysms (a bulge in an artery), rupture of the bowel, and rupture of the womb during pregnancy. Other rare connective tissue disorders are: Loeys-Dietz syndromes, polycystic kidney disease, and Marfan syndrome. classic EDS). Sep 19, 2021 · “Aortic and other arterial dissection and rupture is a major complication and a cause of death in vascular EDS and other rare types of EDS. Apr 12, 2024 · Depending upon the specific subtype present, EDS can follow autosomal dominant or autosomal recessive inheritance Only certain rare types of EDS include a predisposition for severe cardiovascular issues (such as vascular EDS); other types predominantly alter the skin and joints (e. Jan 1, 2021 · Ehlers-Danlos Syndrome (EDS) is a family of multisystemic hereditary connective tissue disorders now comprised of 13 recognized subtypes, classical, classical-like, cardiac-valvular, vascular, hypermobile, arthrochlasia, dermosparaxis, kyphoscoliotic, brittle cornea syndrome, spondylodysplastic, musculocontractural, myopathic, and periodontal, as designated by the most recent 2017 Hypermobile EDS is the most common subtype of EDS (MIM 130020), followed by classical EDS (MIM 130000) and vascular EDS (MIM 130050). Know more about symptoms, causes, risk factors, subtypes, prognosis, treatment, diagnosis We would like to show you a description here but the site won’t allow us. Focused on understanding the molecular pathophysiology of the vascular form of Ehlers-Danlos syndrome (vascular EDS) with the aim of providing informed management of these patients as well as developing new forms of therapy. The different EDS rare types are Classical, Vascular, Arthrochalasia, Dermatosparaxis, Cardiac valvular, Kyphoscoliotic, Classical-like type 1, Classical-like type 2, Myopathic, Musculocontractural, Spondylodysplastic, Periodontal EDS and Brittle Cornea Syndrome. Normally, collagen keeps the tissue elastic and able to stretch without tearing. Vascular Ehlers-Danlos Syndrome (vEDS) is a rare and severe subtype of Ehlers-Danlos Syndrome (EDS), a group of inherited disorders afecing connective tissue. It is caused by a gene mutation affecting a major protein, which causes weakness in vessel walls and hollow organs. hEDS is the most common subtype, though estimates suggest 1 in 3,000 to 5,000 people may be affected. [1] These may be noticed at birth or in early childhood. Written by a GP. Oct 8, 2024 · Ehlers Danlos syndrome (EDS) is a rare condition affecting connective tissue that supports skin, joints, blood vessels, and internal organs. This Primer discusses the epidemiology, mechanisms Vascular Ehlers-Danlos syndrome (vEDS) is a rare and severe connective tissue disorder caused by mutations in the collagen type III alpha I chain ( COL3A1) gene. However, EDS can also have uncommon presentations which are much more Ehlers Danlos Syndrome is a rare disorder. Vascular EDS (vEDS) is an inherited connective tissue disorder caused by See Targeted Genes and Methodology Details for Ehlers-Danlos Syndrome Gene Panel and Method Description for additional details. Genetic testing for rare types of Ehlers-Danlos syndrome The Ehlers-Danlos Syndromes (EDS) national diagnostic service, also known as the Complex EDS service, is a specialist service for you and your family if you have or are suspected to have a rare type of EDS. Ehlers-Danlos syndrome (EDS) is a group of inherited disorders marked by extremely loose joints, very stretchy (hyperelastic) skin that bruises easily, and easily damaged blood vessels. Type III collagen is a protein which is found throughout the body including skin, blood vessels and hollow organs (such as Vascular Ehlers-Danlos Syndrome (vEDS) is a rare and severe subtype of Ehlers-Danlos Syndrome (EDS), a group of inherited disorders affecting connective tissue. Thankfully she isn’t showing any symptoms and I said she didn’t have to wear the lanyard but she Ehlers–Danlos syndromes (EDS) are a group of 13 genetic connective tissue disorders. vEDS is caused by mutations in the COL3A1 gene. Each type of EDS has its own genetic causes and unique symptoms. Methods Whole exome sequencing (WES) was undertaken on 174 EDS patients recruited from a national diagnostic service for complex EDS and a specialist clinic for hEDS Vascular Ehlers-Danlos syndrome is also referred to as vEDS, Ehlers-Danlos syndrome type IV, Sack-Barabas syndrome, and the arterial form of Ehlers-Danlos syndrome. Ehlers-Danlos syndromes (EDS) are a group of connective tissue disorders associated with skin, ligament, blood vessel and organ abnormalities. Apr 10, 2022 · What is vascular Ehlers-Danlos syndrome? Ehlers-Danlos syndrome (EDS) refers to a group of genetic disorders that mainly affect your body's connective tissues, such as ligaments, tendons, and cartilage. Mar 11, 2015 · Vascular Ehlers–Danlos syndrome (vEDS) is a rare and severe autosomal dominant disorder caused by variants at the COL3A1 gene. There's no cure for EDS, but symptoms can often be treated and managed. Also, be sure your privacy is respected and data collection complies with appropriate laws. Jan 5, 2026 · Ehlers-Danlos syndrome (EDS) is a connective tissue disorder that causes joint hypermobility, skin hyperextensibility, and tissue fragility. Collagen helps support joints, skin, and internal organs. Mar 3, 2025 · Yet, EDS often leads to disabling or life-threatening multisystemic comorbidities, such as scoliosis, cardiac dysautonomia, vascular dissection or aneurysms, and pneumothorax, which overlap across different subtypes and with other HCTDs. Characterised by joint hypermobility and skin findings, there are usually additional features (called Red Flags) in other parts of the body. Vascular Ehlers-Danlos syndrome (vEDS), previously named type-IV EDS, is a rare genetic disorder of connective tissue, accounting for 5% of all EDS cases (1, 2). Prof. Most types of EDS are rare, but the hypermobile EDS type is thought to be the most common. Ehlers-Danlos Syndrome (EDS) is a group of genetic connective tissue disorders that affect how the body’s collagen works. [3] Complications may include aortic dissection, joint dislocations, scoliosis, chronic pain, or early osteoarthritis. Hypermobile EDS is a multi-system disorder which can affect health in various ways and can cause multiple symptoms. The vascular subtype of Ehlers Danlos Syndrome (vEDS) is a rare connective tissue disorder characterized by spontaneous arterial, bowel or organ rupture. A proportion of SCAD cases are associated with rare heritable connective tissue disorders. EDS is a group of inherited disorders where skin, joints, blood vessels and hollow organs can be affected as a result of underlying defects in connective tissue. Unlike other EDS subtypes primarily characterized by joint hypermobility and skin elasticity, vEDS is distinguished by its impact on the vascular system, posing a significant risk of life We would like to show you a description here but the site won’t allow us. Vascular EDS is reported to have a prevalence of 1 in 100,000 individuals, and cardiac-valvular EDS a prevalence of less than 1 in a million people. A Major Update Is Coming to EDS and HSD Diagnosis For the first time since 2017, the diagnostic criteria for all types of Ehlers-Danlos syndromes (EDS) and hypermobility spectrum disorders (HSD) will be updated. Figures may be presented in different ways, but they mean the Vascular EDS (vEDS) is a rare type of EDS. These mostly rare and inherited conditions number 13 types, with hypermobile, classical, and vascular EDS being the most common. Abstract Ehlers-Danlos syndrome type IV, the vascular type of Ehlers-Danlos syndromes (EDS), is an inherited connective tissue disorder defined by characteristic facial features (acrogeria) in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive and uterine complications, which are rarely, if at all Jun 22, 2023 · Considered a rare and severe disorder, vascular Ehlers-Danlos is characterized by fragile, thin skin and blood vessels. Skin hyperextensibility, joint hypermobility and widened atrophic scars are characteristic of classical EDS. Because vascular Ehlers-Danlos syndrome can have serious potential complications in pregnancy, you may want to talk to a genetic counselor before starting a family. The Ehlers Danlos syndromes (EDS) are a clinically and genetically heterogeneous group of heritable connective – tissue disorders (HCTDs) characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Ehlers-Danlos syndrome (EDS) is a genetic condition that weakens your connective tissue. g. Connective tissue is like a glue that supports different parts of the body, like the skin, eyes, and heart. Cardiac-valvular EDS (rare) involves severe progressive valve damage needing surgery. . These remain the most widely recognized and studied types of EDS. Clinical characteristics and course of disease of 215 molecularly Abstract Vascular Ehlers-Danlos syndrome (vEDS) is a rare genetic disorder clinically characterized by vascular, intestinal and uterine fragility and caused by heterozygous pathogenic variants in the COL3A1 gene. There are several genetically defined types of EDS including classical, vascular, kyphoscoliotic, arthrochalasic and dermatosparactic EDS. Navigate the body map to learn more about the condition. Mutations in this gene effect the production of type III collagen. Vascular EDS is caused by a genetic alteration in a gene called COL3A1. 9. EDS can cause joints to be overly flexible or unstable, frequent pain, fatigue, and a higher risk of injury—often making everyday movement more challenging. Read more on connective tissue diseases. What makes a condition “rare”? A condition is considered rare if it affects less than 1 in 2000 people. Weakened Vascular Ehlers-Danlos syndrome (vEDS), previously named type-IV EDS, is a rare genetic disorder of connective tissue, accounting for 5% of all EDS cases (1, 2). It is generally considered the most severe form of Ehlers-Danlos syndrome (EDS). The VEDS Movement mission is to save lives and improve the quality of life of individuals with Vascular Ehlers-Danlos Syndrome (VEDS). These defects affect the soft connective tissues resulting in abnormalities in the skin, joints, hollow organs, and blood vessels. The genetic basis for hypermobile Ehlers-Danlos syndrome (hEDS) remains unknown. How common is EDS? Vascular Ehlers-Danlos Syndrome (vEDS, previously Ehlers-Danlos Type IV) is a rare genetic disorder with an estimated prevalence of 1/50,000 to 1/200,000 (1). It can weaken the aorta, which is the large artery that carries blood away from your heart, as well as the arteries in the neck and abdomen. In some cases, vascular Ehlers-Danlos can weaken the walls of the large intestine or uterus, too. mvwgxpaxkcwjgmemolcumscrlemzklwtoujszcmnwhdkjuxaisprnfrdio