Overview
Dentinogenesis imperfecta (DGI) is a rare inherited condition that affects the development of dentin, which is the hard tissue that makes up most of a tooth beneath the enamel. People with this condition have teeth that are weaker than normal, making them more likely to wear down, chip, or break easily. The condition affects both baby teeth and adult teeth. You may also hear it called 'hereditary opalescent dentin' because the teeth often have a distinctive bluish-gray, amber, or brownish color and a translucent or opalescent (pearly) appearance. Dentinogenesis imperfecta is divided into three types (Type I, II, and III). Type I occurs in people who also have osteogenesis imperfecta (brittle bone disease). Types II and III affect only the teeth. In all types, the dentin does not form properly, which weakens the teeth from the inside. The enamel on top can crack and peel away more easily, leaving the softer dentin exposed and vulnerable to rapid wear. The main goal of treatment is to protect the teeth, reduce pain, and maintain normal chewing function and appearance. Dentists and specialists use crowns, veneers, dentures, and other dental restorations to cover and protect affected teeth. While there is no cure, early and ongoing dental care can greatly improve quality of life and help preserve teeth for as long as possible.
Also known as:
Key symptoms:
Teeth that appear bluish-gray, amber, brown, or translucentTeeth that wear down, chip, or break more easily than normalEnamel that cracks and peels away from the tooth surfaceTooth sensitivity to hot, cold, or sweet foods and drinksTeeth that look shorter than normal due to wearPain or discomfort when chewingIncreased risk of tooth decay and cavitiesTeeth that may loosen or fall out earlier than expectedAbnormal tooth shape or sizeIn Type I, symptoms of osteogenesis imperfecta such as fragile bones and blue-tinted whites of the eyes
Clinical phenotype terms (21)— hover any for plain English
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Childhood
Begins in childhood, roughly ages 1 to 12
FDA & Trial Timeline
1 eventHOYA Lens Thailand LTD. — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Dentinogenesis imperfecta.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Dentinogenesis imperfecta at this time.
New trials open frequently. Follow this disease to get notified.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Dentinogenesis imperfecta.
Community
No community posts yet. Be the first to share your experience with Dentinogenesis imperfecta.
Start the conversation →Latest news about Dentinogenesis imperfecta
Disease timeline:
New recruiting trial: DG1 Spectacle Lens for Myopia Progression Control in Children
A new clinical trial is recruiting patients for Dentinogenesis imperfecta
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.Which type of dentinogenesis imperfecta does my child or I have, and what does that mean for treatment?,Should I have genetic testing, and should other family members be tested as well?,What is the best dental treatment plan for protecting my teeth at this stage?,How often should we have dental check-ups, and what specialists should be involved in care?,Are there any dietary or lifestyle changes I should make to protect my teeth?,What signs of complications should I watch for and when should I seek urgent dental care?,Are there any clinical trials or new treatments being studied for this condition?
Common questions about Dentinogenesis imperfecta
What is Dentinogenesis imperfecta?
Dentinogenesis imperfecta (DGI) is a rare inherited condition that affects the development of dentin, which is the hard tissue that makes up most of a tooth beneath the enamel. People with this condition have teeth that are weaker than normal, making them more likely to wear down, chip, or break easily. The condition affects both baby teeth and adult teeth. You may also hear it called 'hereditary opalescent dentin' because the teeth often have a distinctive bluish-gray, amber, or brownish color and a translucent or opalescent (pearly) appearance. Dentinogenesis imperfecta is divided into thre
How is Dentinogenesis imperfecta inherited?
Dentinogenesis imperfecta follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Dentinogenesis imperfecta typically begin?
Typical onset of Dentinogenesis imperfecta is childhood. Age of onset can vary across affected individuals.
Which specialists treat Dentinogenesis imperfecta?
1 specialists and care centers treating Dentinogenesis imperfecta are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.