Geroderma osteodysplastica

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ORPHA:2078OMIM:231070Q82.8
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What is Geroderma osteodysplastica?

Geroderma osteodysplastica (GO), also known as Walt Disney dwarfism or gerodermia osteodysplastica, is a rare autosomal recessive connective tissue disorder characterized by the combination of prematurely aged (wrinkled) skin and skeletal abnormalities. The condition is caused by mutations in the GORAB gene (also known as SCYL1BP1) on chromosome 1q24, which encodes a protein involved in Golgi apparatus function and protein glycosylation. The disease primarily affects the skin and skeletal system. Skin manifestations include lax, wrinkled skin that gives patients a prematurely aged appearance, most prominently on the dorsum of the hands and feet, as well as the face and abdomen. Skeletal features include severe osteoporosis leading to frequent fractures, platyspondyly (flattened vertebral bodies), metaphyseal abnormalities of long bones, and short stature. Facial features may include a small jaw (micrognathia), midface hypoplasia, and a beaked nose. Joint hypermobility or laxity is also commonly observed. Some patients may develop kyphoscoliosis and wormian bones in the skull. There is currently no cure or disease-specific treatment for geroderma osteodysplastica. Management is supportive and multidisciplinary, focusing on monitoring and treating osteoporosis (bisphosphonates have been used in some cases), orthopedic management of fractures and skeletal deformities, and dermatological care. Genetic counseling is recommended for affected families. The condition is extremely rare, with fewer than 50 cases reported in the medical literature, and has been described in several consanguineous families, particularly of Middle Eastern and Mediterranean descent.

Clinical phenotype terms— hover any for plain English:

  • Vertebral compression fractureHP:0002953
  • Beaking of vertebral bodiesHP:0004568
  • Biconcave vertebral bodiesHP:0004586
  • Abnormal bone ossificationHP:0011849
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

Source: openFDA + DailyMed · NDA / BLA labels with structured indications · refreshed weekly

No FDA-approved treatments are currently listed for Geroderma osteodysplastica.

View clinical trials →

Source: ClinicalTrials.gov · synced daily · phases, status, and PI names normalized at ingest

No actively recruiting trials found for Geroderma osteodysplastica at this time.

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

Search ClinicalTrials.gov ↗Join the Geroderma osteodysplastica community →

Source: NPI Registry + PubMed · trial PI roles cross-referenced with ClinicalTrials.gov · ranked by match score (publications + PI activity + community signal)

No specialists are currently listed for Geroderma osteodysplastica.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers

Source: NORD Rare Disease Centers + NIH Undiagnosed Diseases Network (UDN) · centers verified active within last 12 months

🏨 Children's

Children's Hospital Colorado Rare Disease Program

Children's Hospital Colorado

📍 Aurora, CO

👤 Boston Children's Hospital Rare Disease Program

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Boston Children's Hospital Rare Disease Program

Boston Children's Hospital

📍 Boston, MA

👤 Boston Children's Hospital Rare Disease Program

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

🏨 Children's

Ann & Robert H. Lurie Children's Hospital Genetics

Lurie Children's Hospital

📍 Chicago, IL

👤 Boston Children's Hospital Rare Disease Program

🏥 NORD

Cincinnati Children's Hospital Medical Center

Cincinnati Children's

📍 Cincinnati, OH

👤 Boston Children's Hospital Rare Disease Program

🏨 Children's

Nationwide Children's Hospital Rare Disease Center

Nationwide Children's Hospital

📍 Columbus, OH

👤 Boston Children's Hospital Rare Disease Program

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

Travel Grants

No travel grants are currently matched to Geroderma osteodysplastica.

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Community

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Latest news about Geroderma osteodysplastica

Source: PubMed + NIH RePORTER + openFDA + clinical-journal RSS · last 30 days · disease-tagged at ingest by AI extraction with human QC

No recent news articles for Geroderma osteodysplastica.

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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.

Common questions about Geroderma osteodysplastica

What is Geroderma osteodysplastica?

Geroderma osteodysplastica (GO), also known as Walt Disney dwarfism or gerodermia osteodysplastica, is a rare autosomal recessive connective tissue disorder characterized by the combination of prematurely aged (wrinkled) skin and skeletal abnormalities. The condition is caused by mutations in the GORAB gene (also known as SCYL1BP1) on chromosome 1q24, which encodes a protein involved in Golgi apparatus function and protein glycosylation. The disease primarily affects the skin and skeletal system. Skin manifestations include lax, wrinkled skin that gives patients a prematurely aged appearance,

How is Geroderma osteodysplastica inherited?

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

At what age does Geroderma osteodysplastica typically begin?

Typical onset of Geroderma osteodysplastica is neonatal. Age of onset can vary across affected individuals.

Frequently asked questions about Geroderma osteodysplastica

Auto-generated from canonical disease facts (Orphanet, OMIM, ClinicalTrials.gov, openFDA, NPPES). Not a substitute for clinical guidance.

  1. What is Geroderma osteodysplastica?

    Geroderma osteodysplastica is a rare disease catalogued in international rare-disease ontologies (Orphanet ORPHA:2078, OMIM 231070). It is typically inherited as autosomal recessive. Age of onset is generally neonatal. For verified primary sources, see the UniteRare Geroderma osteodysplastica page.

  2. How is Geroderma osteodysplastica inherited?

    Geroderma osteodysplastica follows autosomal recessive inheritance. Genetic counseling is recommended for affected families to understand recurrence risk in offspring and the likelihood of unaffected siblings being carriers. Variants in the underlying gene(s) may be identified via clinical genetic testing.

  3. Are there FDA-approved treatments for Geroderma osteodysplastica?

    Approved treatments for Geroderma osteodysplastica are tracked from openFDA and DailyMed primary sources. Many rare diseases have no specific FDA-approved therapy; for those, supportive care and management of complications form the basis of clinical care. Orphan-drug-designation status is noted where applicable.

  4. Are there clinical trials for Geroderma osteodysplastica?

    Active clinical trials for Geroderma osteodysplastica are tracked daily from ClinicalTrials.gov. Trial availability changes frequently; check the UniteRare trial listings for the current count and recruitment status. Sponsors of rare-disease research often welcome inquiries even when a trial is not actively recruiting at a given moment.

  5. How do I find a specialist for Geroderma osteodysplastica?

    Verified Geroderma osteodysplastica specialists are identified through ClinicalTrials.gov principal-investigator records, peer-reviewed publication authorship (via PubMed), and the NPPES NPI registry. NORD-designated Centers of Excellence and NIH-affiliated rare-disease clinics are also tracked. UniteRare's specialist directory is updated continuously as new evidence becomes available.

See full Geroderma osteodysplastica page for complete clinical details, sources, and verified-specialist listings.

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