Spondylodysplastic Ehlers-Danlos syndrome

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ORPHA:536471OMIM:615349Q79.6
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15Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Spondylodysplastic Ehlers-Danlos syndrome (spEDS) is a rare inherited connective tissue disorder. Connective tissue is the 'glue' that holds the body together — it supports skin, bones, joints, blood vessels, and organs. In spEDS, a genetic change causes problems with how the body builds and maintains this tissue, leading to a wide range of symptoms affecting the skeleton, joints, skin, and sometimes the eyes and muscles. The most recognizable features of spEDS include short stature, bowing of the arm and leg bones, and a flattening of the spinal bones (called platyspondyly). People with spEDS also tend to have very flexible joints, skin that stretches more than usual or bruises easily, and reduced muscle tone (hypotonia), especially in infancy. Some individuals experience delayed motor development, meaning they may take longer to sit, stand, or walk as babies. There is currently no cure for spEDS. Treatment focuses on managing symptoms and improving quality of life. This may include physical therapy to strengthen muscles and protect joints, orthopedic care for bone and spine problems, and regular monitoring by a team of specialists. With the right support, many people with spEDS can lead meaningful and active lives, though the level of impact varies from person to person.

Also known as:

Key symptoms:

Short statureBowing of the arms and legsFlattened or compressed spinal bones (platyspondyly)Overly flexible joints (joint hypermobility)Skin that stretches more than normal or bruises easilyLow muscle tone (hypotonia), especially in babiesDelayed motor milestones such as sitting or walking lateOsteoporosis or fragile bonesFlat feetMild intellectual disability in some individualsEye problems such as myopia (nearsightedness) or blue-tinted whites of the eyesMuscle weakness

Clinical phenotype terms (50)— hover any for plain English
Generalized joint hypermobilityHP:0002761Hypoplastic iliaHP:0000946Soft skinHP:0000977Soft, doughy skinHP:0001027
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Spondylodysplastic Ehlers-Danlos syndrome.

View clinical trials →

No actively recruiting trials found for Spondylodysplastic Ehlers-Danlos syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Spondylodysplastic Ehlers-Danlos syndrome community →

Specialists

15 foundView all specialists →
GL
Göran Larson
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
DL
Delia Lorenz
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
WK
Wolfram Kress
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
AZ
Ann-Kathrin Zaum
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
HH
Helge Hebestreit
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
AD
Antara Dattagupta
SAINT LOUIS, MO
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
SW
Shelley Williamson
HOUSTON, TX
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
MN
Mahnaz Nikpour
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
FN
Fredrik Noborn
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
JN
Jonas Nilsson
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
TD
Tim Van Damme
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
OK
Olivier Kaye
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
DS
Delfien Syx
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
FM
Fransiska Malfait
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication
CS
Christian P Speer
SALEM, OR
Specialist
1 Spondylodysplastic Ehlers-Danlos syndrome publication

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Spondylodysplastic Ehlers-Danlos syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Spondylodysplastic Ehlers-Danlos syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Which gene is causing my (or my child's) condition, and what does that mean for the severity and progression?,What specialists should be part of our care team, and how often should we see each one?,Are there specific activities or sports that should be avoided to protect the joints and spine?,What signs of spinal instability should I watch for, and when should I go to the emergency room?,Should we test other family members, and what is the chance of passing this on to future children?,Are there any clinical trials or research studies we could participate in?,What can we do now to protect bone density and prevent fractures long-term?

Common questions about Spondylodysplastic Ehlers-Danlos syndrome

What is Spondylodysplastic Ehlers-Danlos syndrome?

Spondylodysplastic Ehlers-Danlos syndrome (spEDS) is a rare inherited connective tissue disorder. Connective tissue is the 'glue' that holds the body together — it supports skin, bones, joints, blood vessels, and organs. In spEDS, a genetic change causes problems with how the body builds and maintains this tissue, leading to a wide range of symptoms affecting the skeleton, joints, skin, and sometimes the eyes and muscles. The most recognizable features of spEDS include short stature, bowing of the arm and leg bones, and a flattening of the spinal bones (called platyspondyly). People with spED

How is Spondylodysplastic Ehlers-Danlos syndrome inherited?

Spondylodysplastic Ehlers-Danlos syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Spondylodysplastic Ehlers-Danlos syndrome typically begin?

Typical onset of Spondylodysplastic Ehlers-Danlos syndrome is infantile. Age of onset can vary across affected individuals.

Which specialists treat Spondylodysplastic Ehlers-Danlos syndrome?

15 specialists and care centers treating Spondylodysplastic Ehlers-Danlos syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.