Eds vascular. [1] These may be noticed at birth or in early childhood.

Eds vascular Vascular complications, though rare in classical EDS, can be life-threatening, and this necessitates one to look for vascular associations in Vascular Ehlers–Danlos syndrome (vEDS) is a rare autosomal dominant connective tissue disease resulting from pathogenic variants in the collagen type III alpha 1 chain (COL3A1) gene, encoding type III procollagen. ” Articles about celebrities who reveal they have EDS do not usually Ehlers-Danlos Syndrome (EDS) refers to a group of disorders that affect the body’s connective tissue, including skin, tendons, ligaments and blood vessel walls. Ehlers-Danlos Syndrome (EDS) is a group of inherited connective tissue disorders that impact the body’s collagen structure and function. 27, 2022, by Marisa Wexler, MS Fact-checked by José Lopes, PhD Ehlers-Danlos syndrome (EDS) is the name given to a group of related genetic diseases characterized by weakness in the connective tissue that makes up joints and holds tissues together. Connective tissue holds all the body’s cells, organs, and tissue together. Joint hypermobility is largely limited to the digits, and skin hyperextensibility is minimal or Sep 13, 2022 · This article discusses Vascular EDS, highlighting its predisposition to blood vessel and bowel rupture and physical characteristics Vascular Ehlers-Danlos syndrome is an inherited connective tissue disorder that is caused by defects in a protein called collagen. The VEDS Movement mission is to save lives and improve the quality of life of individuals with Vascular Ehlers-Danlos Syndrome (VEDS). Some people have characteristic facial features, thin skin, and tissue fragility Treatment and management recommendations for those with Vascular Ehlers-Danlos Syndrome, or VEDS, including circumstances to avoid and medications. Vascular Ehlers-Danlos Syndrome is a rare and severe form of Ehlers-Danlos Syndrome, affecting the connective tissues that provide structure and strength to the body's blood vessels and organs. Skin hyperextensibility, joint hypermobility and widened atrophic scars are characteristic of classical EDS. It's estimated to impact 1 in 90,000 people. [1] These may be noticed at birth or in early childhood. The UK National Diagnostic Service for Ehlers-Danlos Syndromes (EDS) was established in 2009 for the rare types of EDS. 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. vascular EDS, meaning that it affects blood vessels prominently). Apr 15, 2025 · Ehler’s-Danlos syndrome (EDS) is a group of 13 internationally classified and genetically diverse inherited disorders that affect your skin, joints, and blood vessel walls and other organs (Rashed et al. A ruptured organ can cause severe internal bleeding, organ failure, or even death. Individuals with EDS may experience abnormalities in their blood vessels, increasing the risk of aneurysms and other cardiovascular issues. The review was conducted Nov 23, 2012 · OBJECTIVE. These characteristics create a distinctive facial profile that can assist clinicians in identifying vascular EDS. Learn about diagnosis, symptoms and treatment from Froedtert & MCW specialists. ⁠ Rebecca recently collaborated with the Colorado Avalanche ice hockey team with a special edition jersey. Learn about EDS and available genetic testing options, based on your type of EDS. Associated tissue fragility affects multiple organ systems, increas … More about Vascular EDS (vEDS) Vascular Ehlers-Danlos Syndrome (vEDS) is a more severe form of EDS. Disease Overview 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 and uterine complications, which Vascular Ehlers-Danlos (vEDS) impacts arteries and internal organs making them extremely fragile. Hypermobile EDS, affecting as many as 1 in 500 people, is relatively common. EDS is a group of hereditary connective tissue disorders characterized by joint hypermobility, skin fragility, and vascular complications. 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 What can be mistaken for Ehlers-Danlos? ‍ Conditions that can be mistaken for EDS include Marfan syndrome, Loeys-Dietz syndrome, joint hypermobility syndrome, and other connective tissue disorders due to overlapping symptoms. The main cause of this disease is the mutation in the COL3A1 gene, which disturbs the proper production of type III collagen. He and his team at the Center provide care for the numerous complications of EDS: joint pain, headaches, POTS, GI problems, and mast cell activation syndrome. People with vascular EDS have a significantly increased risk of developing an aortic aneurysm, dissection and/or rupture, rupture of certain organs, and other health concerns. , 2011). As many tissues can be involved, the underlying molecular defect can manifest itself in many organs and with varying Vascular Ehlers-Danlos syndrome (VEDS) is a genetic condition that is caused by a change (mutation) in the gene for collagen type 3 (the COL3A1 gene). Proper Find a doctor knowledgeable about Vascular Ehlers-Danlos Syndrome (VEDS) through The Marfan Foundation's Help & Resource Center, or download a directory. People are often diagnosed when they have easy and frequent bruising that is not explained by other causes, spontaneous bowel perforations or arterial tears, or because other family members are afected. How rare is Ehlers-Danlos syndrome? The rarity of Ehlers-Danlos syndrome depends on the subtype. Ehlers-Danlos syndrome (EDS) id a rare genetic disorder affecting connective tissue. Feb 15, 2018 · Vascular Ehlers-Danlos syndrome (EDS) or type IV Ehlers-Danlos syndrome (EDS 4) is the most malignant form of Ehlers-Danlos syndrome. Learn about the symptoms, diagnosis, and treatment. Bowel rupture is uncommon in early childhood, has been described in late childhood, and continues to be a risk into adulthood. Vascular Ehlers-Danlos Syndrome is a rare and life-threatening condition characterized by vascular and soft tissue fragility. Vascular Ehlers-Danlos Syndrome Vascular Ehlers-Danlos syndrome (VEDS) is a condition that is quite variable. Two other systems existed before this one. Sep 2, 1999 · Vascular Ehlers-Danlos syndrome (vEDS) should be suspected in individuals with any one of the following major diagnostic criteria or several minor diagnostic criteria, particularly in those younger than age 40 years. Medications for Vascular Ehlers-Danlos Syndrome (vEDS) Many individuals with EDS, especially those with vascular EDS (vEDS), are at risk of cardiovascular complications such as high blood pressure, arterial rupture, or aneurysms. One vEDS patient has been reported with autosomal recessive mode of inheritance [5]. The symptoms listed here may not affect everyone with cvEDS, and people with cvEDS may have other symptoms that are not listed on this page. These include vascular events, described in detail below, and rupture of hollow organs such as bowel and the uterus (in pregnant women). Our depth of experience translates into expert, comprehensive care for you. This meta-analysis assesses the prevalence of vascular complications in among patients with EDS. Outside of vascular EDS, most other EDS subtypes generally lack specific facial markers. [1][4] The existing Ehlers-Danlos syndrome is a group of disorders that affect connective tissues supporting the skin, bones, blood vessels, and many other organs and tissues. GI Issues in EDS: A Comprehensive Guide I. 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. , 2024). Definition 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. The complications of vEDS can be life-threatening and include aneurysm, dissection, and rupture of the arteries and rupture of orga s. What is Ehlers-Danlos syndrome physical appearance? The physical appearance of Ehlers-Danlos Syndrome varies widely by subtype. Significant advancements in genetic testing have contributed to understanding of the cause, management, and treatment of vascular connective tissue disorders, which include Ehlers-Danlos syndrome, Marfan syndrome, and Loeys-Dietz syndrome. Key results A pathogenic or likely pathogenic DNA variant associated with vascular Ehlers-Danlos syndrome (vascular EDS) was found in the COL3A1 gene. Aug 14, 2025 · Ehlers-Danlos syndrome comprises a heterogeneous group of collagen disorders (hereditary connective tissue disease). conducted one of the most detailed examinations of inflammatory biomarkers in vEDS. On the other hand, rarer forms like vascular or dermatosparaxis EDS occur in fewer than 1 in 100,000 individuals, meeting the criteria for rare diseases. It is caused by a gene mutation affecting a major protein, which causes weakness in vessel walls and hollow organs. Ehlers-Danlos syndromes (EDS) are a group of connective tissue disorders associated with skin, ligament, blood vessel and organ abnormalities. Vascular dissection or rupture, gastrointestinal perforation, or organ 4 days ago · Ehlers-Danlos syndrome (EDS) is a heterogeneous group of inherited connective tissue disorders characterized by hyperelastic skin, hypermobile joints, and vascular and other tissue fragility the 2017 classification describes 13 distinct EDS subtypes (Malfait, 2017) inheritance pattern varies by EDS subtype mutations in at least 20 genes have been identified (e. Although the nervous system is not considered a primary target of the Discover comprehensive insights into Ehlers-Danlos syndrome blood testing, including connective tissue disorder analysis, collagen defect examination, and HEDS screening, as well as related assessments for hypermobility and vascular EDS, providing essential diagnostic information for accurate diagnosis and management. Transmission is autosomal dominant. Associated Diagnosis of Vascular Ehlers-Danlos syndrome (VEDS) is based on careful assessment of medical and family history, physical examination, and genetic testing. Vascular Ehlers-Danlos syndrome is an inherited connective tissue disorder that is caused by defects in a protein called collagen. [3] Complications may include aortic dissection, joint dislocations, scoliosis, chronic pain, or early osteoarthritis. Patients with vEDS present with Vascular Ehlers-Danlos Syndrome (vEDS) is a rare and severe subtype of Ehlers-Danlos Syndrome (EDS), a group of inherited disorders afecing connective tissue. Abstract The term “Ehlers-Danlos syndrome” (EDS) groups together an increasing number of heritable connective tissue disorders mainly featuring joint hypermobility and related complications, dermal dysplasia with abnormal skin texture and repair, and variable range of the hollow organ and vascular dysfunctions. This page is intended to provide information about symptoms that may occur in individuals with cvEDS and Jun 22, 2023 · Explore the research on Ehlers-Danlos syndrome and the genetic mutations that cause some of the subtypes of the disorder. Vanderbilt Health offers medical and support services with easy access locations throughout Middle Tennessee and surrounding regions. Saperstein has diagnosed and treated over 500 patients with EDS. , COL5A1 or COL5A2 mutations You could win an amazing prize pack, courtesy of Rebecca Yarros! Rebecca is a #1 New York Times Bestselling Author, and her Fourth Wing fantasy book series follows the story of Violet Sorrengail, who has Ehlers-Danlos syndrome (EDS), a diagnosis Rebecca shares. Characterized by articular hypermobility, skin exten-sibility and tissue fragility, individuals with EDS have a defect in their connec-tive tissue. Please verify the address directly with the provider. Overview of Life Expectancy with EDS Life expectancy in Ehlers-Danlos Syndrome (EDS) varies significantly depending on the specific subtype. Common symptoms include thin, translucent skin; easy bruising; characteristic facial appearance; and fragile arteries, muscles and internal organs. Currently, all 13 recognized types of EDS that are inherited in an autosomal dominant or autosomal recessive pattern (Zschocke et al. For most types, such as hypermobile EDS (hEDS) and classical EDS (cEDS), life expectancy is generally normal, with patients facing chronic symptoms like pain, joint instability, and skin fragility rather than life-threatening complications. They were named Oct 30, 2019 · Vascular EDS Last updated Jan. Discover your risk. There is an increased risk of coronary artery dissection, and as a result, myocardial infarction (heart Vascular Type Medical Resource Guide is copyright 2009 Ehlers-Danlos National Foundation. What are the symptoms found in those affected by Vascular Ehlers-Danlos syndrome, or VEDS? The following are symptoms found in people with Vascular Ehlers-Danlos Syndrome (VEDS): Cardiovascular and arterial characteristics There is no increased risk of valvular or structural cardiac defects. Here, I focus Sep 7, 2025 · Ehlers-Danlos syndrome is a group of genetic connective tissue disorders classified in 13 types. There has been debate regarding the safety of performing elective procedures in patients with vascular manifestations associated with Ehlers-Danlos syndrome (EDS). 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 Vascular Ehlers-Danlos syndrome (VEDS) is one of the different subtypes of Ehlers-Danlos syndrome (EDS). Epidemiology The combined prevalence for all types of Ehlers-Danlos syndrome is estimated to be at least 1 of every 5000 individ Spondylodysplastic EDS (spEDS) Vascular EDS (vEDS) #TogetherWeDazzle Sign up to The Ehlers-Danlos Society mailing list Click here to sign up Donate Ehlers-Danlos Syndrome Specialists in Florida Ehlers-Danlos Syndrome (EDS) is increasingly recognized within the medical community as a genetic disorder that significantly impacts the body's connective tissues, which are crucial for providing support and elasticity to the skin, joints, and vascular structures. What Is Vascular Ehlers-Danlos Syndrome? Ehlers-Danlos syndrome (EDS) is a heterogeneous group of inherited con-nective tissue disorders. The purpose of this study was to review the surgical management and clinical outcomes Jul 16, 2025 · A new classification system for EDS was released in 2017 (1). Dr. Ehlers–Danlos syndromes (EDS) are a group of 13 genetic connective tissue disorders. Vascular Ehlers-Danlos syndrome predisposes young adults to a high risk of digestive tract events which can be life-threatening. People with this condition have very fragile tissues and are at high risk for severe bleeding and internal injuries. With vascular Ehlers-Danlos syndrome, this protein is collagen III, and the specific gene is COL3A1. These can include joint hypermobility, stretchy skin and tissue fragility. Bowel rupture ultimately affects about 25–30% of individuals but rarely leads to death. We follow patients with diagnoses of VEDS in our Connective Tissue Disorder Program here at Lurie Children’s. This form is often accompanied by neurovascular complications secondary to vessel dissections and/or aneurysms. The radiologic, surgical, and genetic databases at a How to Use: Explore the features of cvEDS by selecting different body parts from the menu. Vascular Ehlers-Danlos syndrome (VEDS) is one of the different subtypes of Ehlers-Danlos syndrome (EDS). This new system names 13 different subtypes of EDS. The outcome depends on the severity of the injury and how quickly it’s treated. Learn about Vascular Ehlers-Danlos Syndrome (vEDS) and it's signs and symptoms. Consequently, individuals with EDS often experience symptoms such as joint hypermobility, skin Feb 1, 2014 · Vascular Ehlers–Danlos syndrome (vEDS) is an autosomal dominant disease that affects the arteries, bowels, uterus, and skin. g. Vascular Ehlers Danlos syndrome (vEDS) is a rare disorder, estimated to affect between 1 in 50,000 and 1 in 200,000 people. Patients with this syndrome often have typical What are symptoms of Vascular EDS? Vascular Ehlers-Danlos syndrome is the most severe form of EDS with symptoms that can be life-threatening. , 2022; Zschocke et al. Ehlers-Danlos syndrome (EDS) is a disorder that affects the body's connective tissues. If a patient presents with signs of chest, abdominal pain, Sep 1, 2024 · Ehlers-Danlos Syndrome (EDS) is a genetic disorder affecting connective tissue, causing joint hypermobility & skin elasticity. MATERIALS AND METHODS. Ehlers-Danlos Syndrome (EDS) presents a unique set of challenges in the field of anesthesia. Vascular EDS (vEDS) is an inherited connective tissue disorder caused by Vascular EDS and Systemic Inflammation Though genetically distinct from hEDS, vascular EDS (vEDS) offers additional insight into the role of inflammation in EDS more broadly. It is generally considered the most severe form of Ehlers-Danlos syndrome (EDS). Introduction Ehlers-Danlos Syndrome (EDS) is a group of heritable connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Explore symptoms, inheritance, genetics of this condition. Affected individuals can have spontaneous rupture of hollow organs, such as the bowels or gravid uterus, along with arterial dissections and ruptures that lead to premature death. We would like to show you a description here but the site won’t allow us. May 22, 2020 · It’s Ehlers-Danlos Syndrome (EDS) awareness month! We want to take this opportunity to share crucial differences between the most common form of EDS and Vascular Ehlers-Danlos Syndrome (VEDS). It may cause the walls of your blood vessels, intestines, or uterus to rupture. Despite its growing recognition and the frequency with which it is diagnosed, there Vascular Ehlers-Danlos syndrome (vEDS) is an uncommon genetic disorder that is considered to be the most severe form of Ehlers-Danlos Syndrome (EDS). The information in this article is based on the experience and expertise of the UK's EDS National Diagnostic Service. Our care teams have advanced training and extensive experience diagnosing and treating Vascular Ehlers-Danlos. Sep 2, 1999 · Vascular Ehlers-Danlos syndrome (vEDS) is characterized by arterial, intestinal, and/or uterine fragility; thin, translucent skin; easy bruising; characteristic facial appearance (thin vermilion of the lips, micrognathia, narrow nose, prominent eyes); and an aged appearance to the extremities, particularly the hands. About Vascular Ehlers-Danlos syndrome As with other connective tissue disorders, Ehlers-Danlos syndrome involves a mutated gene that affects proteins that make up connective tissue in your child’s body. What is the pinch test for Ehlers-Danlos syndrome skin? 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 Map results may not be 100% accurate. Our purpose was to review the imaging findings in a cohort of patients with a diagnosis of vascular EDS. Learn about Vascular Ehlers-Danlos Syndrome (VEDS), how it affects the body and who is affected from The VEDS Movement, a division of The Marfan Foundation. What is the pinch test for Ehlers-Danlos syndrome skin? Vascular Type EDS is considered the most serious form of EDS due to the possibility of arterial or organ rupture. Vascular-type Ehlers-Danlos syndrome (EDS) is a rare inherited connective tissue disorder caused by a mutation in type III procollagen. Morissette et al. [1][4] The existing Vascular Compression Syndromes (VCS), Postural Orthostatic Tachycardia Syndrome (POTS), and Ehlers-Danlos Syndrome (EDS) represent complex medical conditions that frequently coexist, impacting blood flow, nerve function, and the quality of life for those affected. It is inherited in an autosomal dominant way which means that if a person has vEDS there is a 50% […] Vascular EDS Vascular Ehlers Danlos syndrome (vEDS) is a rare disorder, estimated to affect between 1 in 50,000 and 1 in 200,000 people. What can be mistaken for Ehlers-Danlos? ‍ Conditions that can be mistaken for EDS include Marfan syndrome, Loeys-Dietz syndrome, joint hypermobility syndrome, and other connective tissue disorders due to overlapping symptoms. Early diagnosis, regular vascular surveillance, and a multidisciplinary approach to management are crucial for improving outcomes. Mar 29, 2023 · The UK National Diagnostic Service for Ehlers-Danlos Syndromes (EDS) was established in 2009 for the rare types of EDS. There are 13 different types of EDS, but they do have some clinical features in common. Vascular Ehlers-Danlos syndrome (EDS), formerly known as EDS type IV, is an autosomal dominant disorder characterized by fragility of medium and large arteries due to type III procollagen deficiency. This information is intended for people who have been recently diagnosed with vascular Ehlers-Danlos syndrome (vEDS) and their friends and relatives. Abstract Ehlers-Danlos syndrome (EDS, ORPHA98249) comprises a group of clinically and genetically heterogeneous heritable connective tissue disorders, chiefly characterized by joint hypermobility and instability, skin texture anomalies, and vascular and soft tissue fragility. The vascular type of Ehlers-Danlos syndrome is characterized by the major complications of arterial and bowel rupture, uterine rupture during pregnancy, and the clinical features of easy bruising, thin skin with visible veins, and characteristic facial features (summary by Leistritz et al. Individuals with vEDS are at high risk for spontaneous rupture of blood vessels, intestines, or other organs, often without prior warning. 2) [4]. Vascular EDS (vEDS) is a rare type of EDS. Vascular Ehlers-Danlos Syndrome, or VEDS, is a genetic condition that affects the body’s connective tissue. Vascular Type EDS is considered the most serious form of EDS due to the possibility of arterial or organ rupture. Ehlers-Danlos syndrome (EDS) is a group of connective tissue disorders that can occur in families. It has the highest mortality rate among the six types of EDS. Click the button to find Ehlers-Danlos Syndrome, Vascular Type: EDS vascular type (vEDS) is characterized by arterial and gastro-intestinal rupture, rupture of the gravid uterus and extensive bruising. Ehlers-Danlos National Foundation is a 501 (c) (3) non-profit organization dedicated to raising awareness of EDS. Some people have characteristic facial features, thin skin, and tissue fragility We CARE For What is vEDS? vEDS // VASCULAR EHLERS-DANLOS SYNDROME a genetic disorder that causes connective tissue to be fragile, particularly in the blood vessels and organs. Learn More. Vascular EDS (vEDS) is an inherited connective tissue disorder caused by pathogenic variants in the COL3A1 gene. Navigate the body map to learn more about the condition. [7] Symptoms often include loose joints, joint pain, stretchy, velvety skin, and abnormal scar formation. People are often diagnosed when they have easy and frequent bruising that is not explained by other causes, a spontaneous bowel, or arterial tears, or because other family members are affected. vEDS may also cause a variet 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. Apr 10, 2025 · Clinical characteristics: Vascular Ehlers-Danlos syndrome (vEDS) is characterized by arterial, intestinal, and/or uterine fragility; thin, translucent skin; easy bruising; characteristic facial appearance (thin vermilion of the lips, micrognathia, narrow nose, prominent eyes); and an aged appearance to the extremities, particularly the hands. Ehlers-Danlos Syndrome (EDS) comprises connective tissue disorders associated with increased vascular complication risks. Read about symptoms, diagnosis, management, genetic factors and more. Apr 10, 2022 · Vascular-type Ehlers-Danlos syndrome is a severe subtype of a genetic connective tissue disorder. It relies primarily on descriptive terms for different types of EDS (e. Complications – Vascular dissection or rupture GI Cardiovascular problems Some rare types of EDS are characterised by cardiovascular problems – the vascular type carries a risk of arterial rupture at a young age, and in cardiac-valvular EDS there are severe progressive problems of the aortic and mitral valves. Ehlers-Danlos Syndrome (EDS) is a genetic condition that can lead to a range of complications, including vascular problems. Collagen, a vital component of connective tissue, plays a key role in providing strength and elasticity to the skin, joints, blood vessels, and internal organs. While musculoskeletal symptoms often dominate clinical attention, gastrointestinal (GI) issues are among the most common—and most under-recognized—manifestations of EDS . VASCULAR EHLERS-DANLOS SYNDROME Vascular Ehlers-Danlos syndrome (vEDS) is an inherited condition that is quite variable. It is caused by mutations in the COL3A1 gene encoding type III collagen (2q32. 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. EDS is a connective tissue condition that is frequently characterized in the media by “loose joints and soft, stretchy skin. It's called the 2017 International Classfication for the Ehlers-Danlos Syndromes. Vascular EDS is usually caused by a change in the COL3A1 gene. Please note that cvEDS affects each person differently. vvrwnfhb wabtiex foqcnt swvn vqdavkf ksr aqwf qgodrv wveegw apcux juxezq ohw cddi fcokhq qqp