Ehlers-Danlos syndrome, Kyphoscoliotic type (type VI)

Overview

What is kyphoscoliotic Ehlers-Danlos syndrome?

Ehlers-Danlos syndrome (EDS) is a group of related, but distinct, genetic disorders. They affect connective tissue. Connective tissue is the material between the cells of the body that connects and supports different tissues and organs. It gives skin strength and elasticity. A structural protein called collagen is one of the major components in connective tissue. People with EDS have an unexpected change (or mutation) in one of the genes that is important in producing or processing collagen. Because of these gene changes, they do not produce or process collagen properly and their connective tissue does not develop in the right way.

There are six major subtypes of EDS. They are related, but separate disorders. Kyphoscoliotic Ehlers-Danlos syndrome is also known as EDS Type VI. It has the additional symptoms of kyphosis (hunchback) and scoliosis (sideways curve in the spine). Both are often present at birth, or occur early in infancy. They will get progressively worse over time.

Symptoms that are common to all types include loose skin that stretches more than normal, but is also fragile and can tear or bruise easily. The skin also heals poorly and usually leaves distinctive scars. Loose joints that can be flexed or bent farther than other people can is also common. Joints in EDS are usually prone to popping out of place (dislocation) and chronic pain.

References
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Are there other names for kyphoscoliotic Ehlers-Danlos syndrome?

How common is kyphoscoliotic Ehlers-Danlos syndrome?

What is the usual abbreviation for kyphoscoliotic Ehlers-Danlos syndrome?

What health problems are part of kyphoscoliotic Ehlers-Danlos syndrome?

Is kyphoscoliotic Ehlers-Danlos syndrome a connective tissue disease?

Is kyphoscoliotic Ehlers-Danlos syndrome more common in some parts of the world?

Are there other names for kyphoscoliotic Ehlers-Danlos syndrome?

Kyphoscoliotic Ehlers-Danlos syndrome is also known as:

  • EDS IV
  • Ehlers-Danlos syndrome, Kyphoscoliotic type
  • kEDS
  • EDS, kyphoscoliotic type
  • Nevo syndrome
  • Ehlers-Danlos syndrome type VI
  • EDS type IV
References
How common is kyphoscoliotic Ehlers-Danlos syndrome?

Ehlers-Danlos syndrome (EDS) is a group of related, but distinct, genetic disorders. Kyphoscoliotic Ehlers-Danlos syndrome is a member of this group. Overall, Ehlers-Danlos syndrome affects between 1 in 5,000 to 1 in 10,000 people.

Kyphoscoliotic EDS is very rare. As of 2018 the exact numbers are not known. It is believed to happen in 1 in 100,000 live births. People with mild symptoms of kyphoscoliotic EDS may often go unnoticed.

References
What is the usual abbreviation for kyphoscoliotic Ehlers-Danlos syndrome?

Common abbreviations for Kyphoscoliotic Ehlers-Danlos syndrome include:

  • EDS IV
  • kEDS
  • EDS, kyphoscoliotic type
  • EDS type IV
References
What health problems are part of kyphoscoliotic Ehlers-Danlos syndrome?

Ehlers-Danlos syndrome (EDS) is a group of related, but distinct, genetic disorders. There are six major subtypes of EDS. Symptoms that are common to all types include loose skin that stretches more than normal, but is also fragile and can tear or bruise easily. The skin also heals poorly and usually leaves distinctive scars. Loose joints that can be flexed or bent farther than other people can is also common. Joints in people with EDS are usually prone to popping out of place (dislocation) and chronic pain. Symptoms that are associated specifically with kyphoscoliotic Ehlers-Danlos syndrome include kyphosis (hunchback) and scoliosis (sideways curve in the spine). These symptoms can appear at birth or in early infancy and they get worse over time.

References
Is kyphoscoliotic Ehlers-Danlos syndrome a connective tissue disease?

Ehlers-Danlos syndrome (EDS) is a group of related genetic disorders that affect connective tissue. Connective tissue is the material between the cells of the body that connects and supports other tissue and organs. It gives skin elasticity. A structural protein called collagen is one of the major components in connective tissue. People with kyphoscoliotic EDS have an unexpected change (or mutation) in a gene that is important in processing collagen. Because of this gene mutation, people with kyphoscoliotic EDS have collagen that doesn't work properly, and their connective tissue does not develop in the right way.

References
Is kyphoscoliotic Ehlers-Danlos syndrome more common in some parts of the world?

As of 2018, there are no known ethnicities or parts of the world that are more likely to have kyphoscoliotic Ehlers-Danlos syndrome. Many people with kyphoscoliotic Ehlers-EDS have been reported in the Middle East, Turkey, and Greece. However, researchers cannot yet say definitively that that people from these parts of the world are more likely to have kyphoscoliotic EDS.

References

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