AREDYLD syndrome

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ORPHA:1133OMIM:207780Q87.8
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Overview

AREDYLD syndrome is an extremely rare genetic condition whose full name stands for Acral-Renal-Ectodermal-Dysplasia-Lipoatrophic-Diabetes. This syndrome affects multiple body systems at once. People with AREDYLD syndrome typically have abnormalities of the hands and feet (acral features), kidney (renal) problems, issues with structures that develop from the outer layer of the embryo such as skin, hair, teeth, and nails (ectodermal dysplasia), loss of body fat under the skin (lipoatrophy), and diabetes that is related to insulin resistance. The condition is present from birth or early childhood, and the combination of these features helps distinguish it from other ectodermal dysplasia syndromes. Because AREDYLD syndrome is so rare, with only a handful of cases described in the medical literature, there is limited information about its full range of symptoms and long-term outcomes. Treatment is mainly supportive and focuses on managing each individual symptom. For example, diabetes may require insulin or other blood sugar-lowering medications, kidney problems may need monitoring by a nephrologist, and dental or skin issues may require specialized care. There is currently no cure for AREDYLD syndrome, and management requires a coordinated team of specialists working together to address the various aspects of the condition.

Also known as:

Key symptoms:

Abnormalities of the hands and feetKidney problemsThin or sparse hairAbnormal or missing teethNail abnormalitiesLoss of fat under the skin (lipoatrophy)Diabetes or insulin resistanceShort statureSkin abnormalitiesUnderdeveloped sweat glands leading to heat intoleranceDistinctive facial featuresSmall or underdeveloped fingers or toesDelayed growth

Clinical phenotype terms (26)— hover any for plain English
Craniofacial hyperostosisHP:0004493Refractory anemia with ringed sideroblastsHP:0004828Abnormal nasal morphologyHP:0005105
Inheritance

Autosomal recessive

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

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for AREDYLD syndrome.

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No actively recruiting trials found for AREDYLD syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the AREDYLD syndrome community →

No specialists are currently listed for AREDYLD syndrome.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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 AREDYLD syndrome.

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Community

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Latest news about AREDYLD syndrome

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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.What specific symptoms should I watch for that might indicate my condition is getting worse?,How often should my blood sugar and kidney function be checked?,Are there any genetic tests that could help confirm the diagnosis or identify the exact gene involved?,What precautions should I take to avoid overheating?,Should my family members be tested for this condition?,Are there any clinical trials or research studies I could participate in?,What specialists should be part of my care team, and how often should I see them?

Common questions about AREDYLD syndrome

What is AREDYLD syndrome?

AREDYLD syndrome is an extremely rare genetic condition whose full name stands for Acral-Renal-Ectodermal-Dysplasia-Lipoatrophic-Diabetes. This syndrome affects multiple body systems at once. People with AREDYLD syndrome typically have abnormalities of the hands and feet (acral features), kidney (renal) problems, issues with structures that develop from the outer layer of the embryo such as skin, hair, teeth, and nails (ectodermal dysplasia), loss of body fat under the skin (lipoatrophy), and diabetes that is related to insulin resistance. The condition is present from birth or early childhood

How is AREDYLD syndrome inherited?

AREDYLD syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does AREDYLD syndrome typically begin?

Typical onset of AREDYLD syndrome is neonatal. Age of onset can vary across affected individuals.