Ichthyosis-oral and digital anomalies syndrome

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ORPHA:2272OMIM:258840Q87.8
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Overview

Ichthyosis-oral and digital anomalies syndrome, also sometimes called Ichthyosis with oral and digital anomalies or Schubert-Bartsocas syndrome in some references, is a very rare inherited condition that affects the skin, mouth, and fingers or toes. The word 'ichthyosis' refers to dry, scaly skin that can look like fish scales. In this syndrome, that skin problem is combined with unusual features in the mouth (such as abnormal gums or teeth) and in the hands or feet (such as fused or extra fingers and toes, or webbing between digits). People with this condition are usually affected from birth or very early in life. The skin can be thickened, rough, and uncomfortable, and may cover large areas of the body. The mouth and dental problems can make eating and speaking more difficult. The finger and toe differences may affect how well a person can grip or walk. There is currently no cure for this syndrome. Treatment focuses on managing symptoms — keeping the skin moisturized and protected, addressing dental needs, and sometimes surgery to separate fused digits. A team of specialists is usually needed to provide the best care. Because this condition is so rare, treatment plans are highly individualized.

Also known as:

Key symptoms:

Dry, thick, scaly skin covering large areas of the body (ichthyosis)Abnormal or missing teethOvergrowth or thickening of the gumsFused fingers or toes (syndactyly)Extra fingers or toes (polydactyly)Webbing between fingers or toesRough, uncomfortable skin that may crack or peelDifficulty with fine hand movements due to digit abnormalitiesPossible problems with eating or speaking due to mouth changes

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 Ichthyosis-oral and digital anomalies syndrome.

View clinical trials →

No actively recruiting trials found for Ichthyosis-oral and digital anomalies syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Ichthyosis-oral and digital anomalies syndrome community →

No specialists are currently listed for Ichthyosis-oral and digital anomalies 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 Ichthyosis-oral and digital anomalies syndrome.

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Community

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Latest news about Ichthyosis-oral and digital anomalies 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 genetic tests should we do to confirm the diagnosis and understand the cause?,What is the best daily skin care routine for my specific situation?,Should my fingers or toes be evaluated for surgery, and if so, when is the best time?,What dental specialists should I see, and how often?,Are there any clinical trials or research studies I could participate in?,What should I watch for that would mean I need urgent medical attention?,Are other family members at risk, and should they be tested?

Common questions about Ichthyosis-oral and digital anomalies syndrome

What is Ichthyosis-oral and digital anomalies syndrome?

Ichthyosis-oral and digital anomalies syndrome, also sometimes called Ichthyosis with oral and digital anomalies or Schubert-Bartsocas syndrome in some references, is a very rare inherited condition that affects the skin, mouth, and fingers or toes. The word 'ichthyosis' refers to dry, scaly skin that can look like fish scales. In this syndrome, that skin problem is combined with unusual features in the mouth (such as abnormal gums or teeth) and in the hands or feet (such as fused or extra fingers and toes, or webbing between digits). People with this condition are usually affected from birth

How is Ichthyosis-oral and digital anomalies syndrome inherited?

Ichthyosis-oral and digital anomalies syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Ichthyosis-oral and digital anomalies syndrome typically begin?

Typical onset of Ichthyosis-oral and digital anomalies syndrome is neonatal. Age of onset can vary across affected individuals.