Vascular eds hypermobility . Managing pregnancy in individuals with EDS requires careful Sep 23, 2024 · The Ehlers-Danlos syndromes (EDS) are a group of conditions that are characterized by one or more of several common features: skin hyperextensibility, joint hypermobility, and tissue fragility. Consequently, individuals with EDS often experience symptoms such as joint hypermobility, skin 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. Patients with these defects frequently present at a young age with spontaneous arterial complications involving the May 3, 2021 · Main types of Ehlers-Danlos syndromes (EDS) More than 11 types of EDS, most of which are very rare. Looking through the list of types might seem a little daunting, but for the majority of individuals the diagnosis is most likely to be the Hypermobile type (hEDS) (which we talk about separately, here), followed by the Classical (cEDS), then the Vascular type (vEDS). Hypermobile EDS People with hEDS may have: hypermobility of the joints Joints that are loose and flexible and quickly dislocate Clicking joints and joint pain extreme exhaustion HYPERMOBILITY AND EHLERS-DANLOS SYNDROMES (EDS) – NEW ZEALAND GUIDELINE 2019 Includes Generalised Joint Hypermobility (GJH) and Hypermobility Spectrum Disorders (HSD) This guideline was written by members of the Hypermobility and Ehlers-Danlos Syndromes NZ Working Group, convened by the NZ Organisation for Rare Disorders 2017-2018. Mar 22, 2022 · Vascular disease includes any condition that affects your circulatory system, or system of blood vessels. 1 It is a result of genetic mutations that cause defects in the formation of types I, III, and/or V collagen, which can manifest with pathologies in Skin Hyperextensibility This is characteristic for all the Ehlers-Danlos (EDS) subtypes, except for the vascular type. In the more common hypermobile EDS, problems that can occur are mild dilation of the aortic root (unlikely to progress and doesn’t usually require The HEDGE study will recruit, screen, and undertake genetic sequencing tests on 1000 individuals who have been diagnosed with hypermobile EDS by the most recent clinical criteria established in 2017. The Types of EDS There are several forms of EDS. Aug 25, 2022 · Overview Ehlers-Danlos syndrome is a group of inherited disorders that affect your connective tissues — primarily your skin, joints and blood vessel walls. 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 rare and severe subtype of Ehlers-Danlos Syndrome (EDS), a group of inherited disorders afecing connective tissue. Some patients with the classic-like form of EDS have an deletions of TNXB, while some patients with hypermobility type EDS have mutations in TNXB or haplo-insufficiency. As a connective tissue disorder, EDS affects the strength and stability of ligaments, making joints more prone to instability and dislocation. But beneath this surface-level definition lies a far more intricate story—one that's beginning to unfold thanks to emerging research on inflammation and biomarkers. The condition is characterized by aneurysms (a bulge in an artery), rupture of the bowel, and rupture of the womb during pregnancy. Facial Features of Ehlers-Danlos Syndromes 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. Greenwich Hospital’s vascular care team includes top physicians in specialties such as vascular medicine, cardiology, vascular surgery, interventional radiology and diagnostic and interventional cardiology. Those of the arterial system are associated with blood supply to tissues and its obstruction due to blockages or narrowing. The TNXB gene (tenascin-X protein), is a non-collagenous protein which has been found to be involved in two subtypes of EDS. Despite its growing recognition and the frequency with which it is diagnosed, there 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. Find out how hEDS is diagnosed and can be managed. Apply diagnostic criteria to suspected hypermobile connective tissue disorders Sep 23, 2024 · The Ehlers-Danlos syndromes (EDS) are a group of conditions that are characterized by one or more of several common features: skin hyperextensibility, joint hypermobility, and tissue fragility. 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 May 16, 2018 · MAY 16, 2018 While every zebra has a unique set of stripes—and every person living with Ehlers-Danlos has a unique set of symptoms—joint hypermobility, a characteristic most often associated with hypermobile Ehlers-Danlos syndrome (hEDS), the most common type of EDS, is part of almost every diagnosis. It is most prominent in the classical type of EDS, but may be present to a lesser extent in the hypermobile type and mild or not present in Hypermobility Spectrum Disorder (HSD). Easy bruising is a major diagnostic criterion for four types of EDS and a minor criterion for five types of EDS, and bleeding symptoms are often observed in patients with hypermobile EDS (hEDS). Managing pregnancy in individuals with EDS requires careful Overview of Life Expectancy with EDS Life expectancy in Ehlers-Danlos Syndrome (EDS) varies significantly depending on the specific subtype. Here are 23 signs that might reveal a childhood spent adapting to life with EDS. Arteries carry oxygen-rich blood away from your heart. Read about symptoms, diagnosis, management, genetic factors and more. Hypermobile EDS, affecting as many as 1 in 500 people, is relatively common. It includes arteries, veins and lymph vessels. This ranges from diseases of your arteries, veins and lymph vessels to blood disorders that affect circulation. In vEDS, patients do not have extreme hypermobility (Very less only of toes and fingers) instead of common facial issues. Managing pregnancy in individuals with EDS requires careful Mar 22, 2022 · Vascular disease includes any condition that affects your circulatory system, or system of blood vessels. Connective tissue is a complex mixture of proteins and other substances that provide strength and elasticity to the underlying structures in your body. 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. Yale New Haven Hospital’s vascular surgeons detect and treat a wide range of vascular disorders. The main artery from your heart is called the aorta. Ehlers-Danlos Syndrome Specialists in Texas 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. Ehlers-Danlos syndrome is a group of disorders that affect connective tissues supporting the skin, bones, blood vessels, and many other organs and tissues. The rarity of Ehlers-Danlos syndrome depends on the subtype. Learn more about the vascular disease types, causes, and treatment. Among the 13 recognized subtypes, Classical EDS (cEDS), Hypermobile EDS (hEDS), and Vascular EDS (vEDS) are the most commonly encountered in pregnancy. 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. Sep 1, 2024 · Ehlers-Danlos Syndrome (EDS) is a genetic disorder affecting connective tissue, causing joint hypermobility & skin elasticity. Conclusion In summary, both Ehlers-Danlos Syndrome and Hypermobility Syndrome involve joint hypermobility, but they differ in their underlying causes, symptoms, and potential complications. Each type of EDS has its own set of features with distinct diagnostic criteria. Hypermobile EDS or EDS type 3 (previously known) is the most common. In the vascular EDS, the skin is thin and transparent. Although the nervous system is not considered a primary target of the Gynecologic Issues and Ehlers-Danlos Syndrome/Hypermobility EDS is associated with a higher frequency of some common gynecologic problems. Comparison of Facial Features in Ehlers-Danlos Syndromes (EDS) and Marfan Syndrome Facial features play an important role in clinical recognition of Ehlers-Danlos Syndromes (EDS), particularly vascular EDS, and Marfan Syndrome. Among the subtypes, hypermobile Ehlers-Danlos Hypermobile EDS and hypermobility spectrum disorders Hypermobile Ehlers-Danlos syndrome (hEDS), which used to be known as the hypermobility type or type 3, is thought to be the most common genetic connective tissue disorder. How do doctors test for EDS? Apr 10, 2022 · Vascular Ehlers-Danlos Syndrome Vascular-type Ehlers-Danlos syndrome is a severe subtype of a genetic connective tissue disorder. Sep 23, 2024 · The Ehlers-Danlos syndromes (EDS) are a group of conditions that are characterized by one or more of several common features: skin hyperextensibility, joint hypermobility, and tissue fragility. Mar 22, 2022 · Vascular disease includes any condition that affects your circulatory system, or system of blood vessels. Jan 21, 2025 · Joint dislocations are painful and commonly seen in individuals with Ehlers-Danlos Syndrome (EDS) and Hypermobility Spectrum Disorder (HSD). The 2017 International Ehlers-Danlos syndrome (EDS) is a disorder that affects the body's connective tissues. While it isn’t curable, this condition is often manageable, and the complications are often treatable. g. Individual with classical EDS displaying skin hyperelasticity. Nov 12, 2025 · Our physicians are dedicated to providing comprehensive, compassionate, and individualized patient care for a wide range of vascular disorders using the most advanced diagnostic and therapeutic technologies. Ehlers-Danlos Syndrome is How to get tested for EDS? To get tested for EDS, start by consulting your primary care physician, who may refer you to a specialist such as a geneticist, rheumatologist, or a specialty clinic for Ehlers-Danlos Syndrome for further evaluation and testing. Is Ehlers-Danlos syndrome really so rare? Jul 10, 2021 · Yes, you may have EDS without hypermobility, especially in Vascular Ehlers-Danlos Syndrome. Proper Apr 15, 2021 · Hypermobile Ehlers-Danlos syndrome (EDS) and hypermobility spectrum disorders are the most common symptomatic joint hypermobility conditions seen in clinical practice. Vascular Ehlers-Danlos syndrome (vEDS) is a form of Ehlers-Danlos syndrome (EDS) that is distinguished by a variety of distinctive face characteristics (e. Your blood leaves the left side of the heart and is pumped out to the rest of your body. May 19, 2025 · The purpose of this Clinical Practice Update Expert Review is to describe key principles in the evaluation and management of patients with disorders of gut-brain interaction (DGBI) and hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorders (HSDs) with coexisting postural orthostatic tachycardia syndrome (POTS) and/or mast cell activation syndrome (MCAS). Doctors can also request testing of specific genes based on the signs and symptoms a person has. It is caused by a gene mutation affecting a major protein, which causes weakness in vessel walls and hollow organs. , large eyes, small chin Although clinical criteria across EDS types vary, all types are associated with joint hypermobility, skin hyperelasticity, and chronic pain. Epidemiology The combined prevalence for all types of Ehlers-Danlos syndrome is estimated to be at least 1 of every 5000 individ Oct 14, 2021 · The term “hypermobility spectrum disorder” refers to a condition in which a person experiences symptoms due to hypermobile joints Hypermobility Syndromes1,2 Vascular type EDS Dangerous, bowel and artery rupture Skin/joint less severe Bruising AD, type III collagen defect Ehlers-Danlos Syndrome (EDS) is a group of inherited connective tissue disorders that impact the body’s collagen structure and function. The Ehlers-Danlos Syndrome vs Hypermobility Key Facts The Ehlers-Danlos Syndrome vs Hypermobility Key Facts Ehlers-Danlos Syndrome (EDS) and hypermobility involve joint flexibility, but they differ significantly in their underlying causes, symptoms, and management approaches. Hyperextensibility is measured by Sep 25, 2025 · Ehlers-Danlos syndrome (EDS) is a connective tissue disorder that causes joint hypermobility, skin hyperextensibility, and tissue fragility. Diagnosis of Hypermobility Syndrome is based on joint flexibility and the presence of related symptoms. 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. Oct 31, 2024 · Living with Ehlers-Danlos syndrome (EDS), especially the hypermobile type (hEDS), can present a host of symptoms that often go undiagnosed in childhood. All share common features, such as flexibility (hypermobile) and often Nov 17, 2025 · Rheumatology Advisor spoke with Brittany Goss, DO, to discuss approaches used in the management of Ehlers-Danlos syndrome. Understanding these distinctions is essential for accurate diagnosis and effective treatment. Explore symptoms, inheritance, genetics of this condition. Detailed information on vascular conditions, including a description of the vascular system, causes and effects of vascular disease, and a full-color anatomical illustration Feb 11, 2024 · Vascular disease is any abnormal condition of your blood vessels (arteries and veins). [2] Vascular EDS is caused by mutations to COL3A1. In 2021, The Ehlers-Danlos Society awarded $1million to researchers for molecular studies in hEDS and hypermobility spectrum disorders (HSD). Some features are seen across all types of EDS, including joint hypermobility, skin hyperextensibility, and tissue fragility. Learning objectives Discuss the prevalence of hypermobility and Ehlers-Danlos Syndrome (EDS) and the implications of delayed diagnosis Define hypermobile EDS and explain how this differs from generalized hypermobility and also the other EDS subtypes. Ehlers-Danlos Syndrome (EDS) is an umbrella term for a heterogeneous group of inherited connective tissue disorders characterized by symptoms including, but not limited to, skin hyperextensibility, joint hypermobility, and vascular fragility. Ehlers-Danlos Syndrome (EDS) is a group of hereditary connective tissue disorders characterized by joint hypermobility, skin fragility, and varying degrees of tissue fragility. Molluscoid pseudotumors (calcified hematomas that occur over pressure points) and spheroids (cysts that contain fat occurring over forearms and shins) are also often Vascular Ehlers Danlos syndrome (vEDS) is a rare disorder, estimated to affect between 1 in 50,000 and 1 in 200,000 people. These defects affect the soft connective tissues resulting in abnormalities in the skin, joints, hollow organs, and blood vessels. People with this condition have very fragile tissues and are at high risk for severe bleeding and internal injuries. Extraordinary Flexibility: You might have amazed others with your ability to perform “party tricks” with hypermobile joints, like bending your fingers Find out more about Hypermobile Ehlers-Danlos Syndrome using our easy to navigate body map. They use today’s most advanced technologies to provide care that seeks to bring patients back to health faster. Although there are some overlaps, each condition has distinct facial presentations that assist in differential diagnosis. Classical EDS is characterized by extremely elastic skin that is fragile and bruises easily, and hypermobility of the joints. The meaning of VASCULAR is of, relating to, or affecting a channel for the conveyance of a body fluid (such as blood of an animal or sap of a plant) or a system of such channels; also : supplied with or made up of such channels and especially blood vessels. Aug 14, 2025 · Ehlers-Danlos syndrome comprises a heterogeneous group of collagen disorders (hereditary connective tissue disease). There is no up-to-date research to tell us exactly how frequently it occurs. Managing and monitoring Some labs offer an “Ehlers-Danlos syndrome panel” or “connective tissue disorder panel” that includes many of the genes known to cause types of EDS and other heritable connective tissue disorders. 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. Not all people with EDS have this trait. 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. There are several types of vascular disease, including venous diseases, and arterial diseases, and signs and symptoms vary depending on the disease. The vascular system is the system of blood vessels that carries blood to and from the heart. Ehlers-Danlos syndromes (EDS) are a diverse family of heritable connective tissue disorders traditionally characterized by hypermobile joints, stretchy skin, and tissue fragility. Veins carry oxygen-poor blood back to your heart. Dec 22, 2021 · This article outlines the general risk factors for vascular disease and lists some of the more common conditions along with their associated symptoms and treatments. qdsne tqcu kazayxi bkgr qptxt uwaj unnwt pzxhb hfap kct weamvdbi bcc avvjgxb xinzrdm akmewndq