What is the Difference Between EDS and hEDS

EDS and hEDS are two related conditions. Ehlers-Danlos syndromes (EDS) are a group of rare inherited conditions that primarily affect the connective tissue. Most of them have genetic markers and are characterized mainly by generalized joint hypermobility. hEDS is the most common form of EDS among the other 13 subtypes.

The key difference between EDS and hEDS is their nature. EDS is a group of connective tissue disorders characterized by generalized joint hypermobility, tissue fragility, and skin hyperextensibility, while hEDS is the most common form of EDS and is characterized by generalized joint hypermobility, specific clinical features like skin involvement, musculoskeletal complications, digestive problems and internal organ problems.

CONTENTS

1. Overview and Key Difference
2. What is EDS 
3. What is hEDS
4. Similarities – EDS and hEDS
5. EDS vs hEDS in Tabular Form
6. Summary – EDS vs hEDS
7. FAQ: EDS and hEDS

What is EDS?

Ehlers-Danlos syndrome (EDS) is a group of rare inherited conditions that mainly affect the connective tissue of the body. Connective tissue supports the skin, tendons, ligaments, blood vessels, internal organs, and bones. There are 13 subtypes of Ehlers-Danlos syndrome. The symptoms of this condition may include an increased range of joint movement or joint hypermobility, stretchy skin, and skin that bruises easily. EDS is caused by pathogenic variants of at least 20 genes that provide instructions for making connective tissue proteins, such as variants COL5A1, COL5A2, COL1A1, TNXB, etc.

Figure 01: EDS

Ehlers-Danlos syndrome can be diagnosed through medical history, physical examinations, and genetic tests. Furthermore, treatment options for EDS may include prescribed drugs for pain, blood pressure, physical therapy, surgical, and other procedures. 

What is hEDS?

Hypermobile EDS (hEDS) is the most common type of EDS. People with hEDS may have symptoms such as joint hypermobility, joint pain, clicking of joints, loose, unstable joints that dislocate easily, extreme tiredness, skin that bruises easily, digestive problems like heartburn and constipation, problems with internal organs, and problems with bladder control. Moreover, the cause of hEDS has not been identified, and this condition appears to follow an autosomal dominant inheritance pattern.

hEDS can be diagnosed based on a person’s medical history and a physical examination. Furthermore, treatment options for hEDS may include taking pain relief medications, physiotherapy, gentle exercise, talking therapies, and surgeries to correct mitral valve problems and organ prolapse.

Similarities Between EDS and hEDS

• EDS and hEDS are two related conditions.
• hEDS is a subtype of EDS.
• EDS and hEDS both have joint hypermobility issues and skin problems.
• Both can be diagnosed through medical history and physical examination.
• They can be treated through pain relief medications, physical therapy, and surgery.

Difference Between EDS and hEDS

Definition

1. Ehlers-Danlos syndrome (EDS) is a group of rare inherited connective tissue conditions with generalized joint hypermobility.
2. hEDS is the most common subtype of the hEDS.

Subtypes

1. 13 subtypes are grouped under EDS.
2. There are no subtypes in hEDS.

Causes

1. EDS is caused by variants in genes such as COL5A1, COL5A2, COL1A1, TNXB, etc.
2. No genetic variation that causes hEDS has been identified.

Symptoms

1. The main symptoms of this condition may include an increased range of joint movement or joint hypermobility, stretchy skin and fragile skin that breaks easily or skin that bruises easily.
2. The main symptoms of this condition may include joint hypermobility, joint pain, clicking of joints, loose, unstable joints that dislocate easily, extreme tiredness, skin that bruises easily, digestive problems, such as heartburn and constipation, problems with internal organs (mitral valve problems and organ prolapse), and problems with bladder control (urinary incontinence).

Diagnosis

1. Ehlers-Danlos syndrome can be diagnosed through medical history, physical examination, and genetic tests.
2. EDS can be diagnosed through medical history and physical examination.

Treatment

1. Treatment options for EDS may include taking prescribed drugs for pain, blood pressure, physical therapy, surgical, and other procedures.
2. Treatment options for hEDS may include taking pain relief medications, physiotherapy, gentle exercise, talking therapies, and surgeries.

The following table summarizes the difference between EDS and hEDS.                      

Summary – EDS vs hEDS

Ehlers-Danlos syndrome is a genetic condition that makes the body’s connective tissue weaker. The main symptoms of EDS are an increased range of joint movement or joint hypermobility, stretchy skin, and skin that bruises easily. Healthcare providers classify Ehlers-Danlos syndrome into 13 subtypes. hEDS is the most common type of EDS. It is characterized by the presence of generalized joint hypermobility, specific clinical features like skin involvement, musculoskeletal complications, digestive problems, and internal organ problems. This summarizes the difference between EDS and hEDS.                      

FAQ: EDS and hEDS

1. What are EDS symptoms?

• The symptoms of classical EDS may include joint hypermobility, loose, stretchy skin, fragile skin that can split easily, smooth, velvety skin that bruises easily, unstable joints that dislocate easily, wounds that are slow to heal and leaves wide scars, and hernias.

2. What is the best treatment for EDS?

• The conservative treatment, such as taking pain relief medications, physical therapy and RICE (rest, ice, compression, elevation) is preferred in EDS. The other treatment procedures may include surgeries.

3. Does EDS shorten life expectancy?

• Most forms of Ehlers-Danlos syndrome don’t affect or lower your life expectancy. However, the vascular type is typically a more severe form of EDS and is often associated with a short lifespan. The patients with vascular EDS have an average life expectancy of 48 years.

4. What happens if people have hEDS?

• People with hEDS may expericne joint hypermobility, joint pain, loose, unstable joints that dislocate easily, and clicking joints. Other than these typical symptoms, they may also have digestive and internal organ problems.

5. What is the best treatment for hEDS?

• The best treatments for hEDS are taking pain relief medications, physical therapy, exercises to strengthen the muscles and stabilize joints. Physical therapist may also recommend braces to help prevent joint dislocations.

Reference:

1. “What Is Ehlers-Danlos Syndrome?” Cleveland Clinic.
2. “Heds.” The Ehlers Danlos Society.

Image Courtesy:

1. “Arthrochalasia EDS” By Daniela Melis,1 Gerarda Cappuccio,1 Virginia Maria Ginocchio,1 Giorgia Minopoli,1 Maurizia Valli,2 Massimiliano Corradi,3 and Generoso Andriacorresponding author1 – Open NIM (CC BY 2.5) via Commons Wikimedia

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