Rare Dental Conditions

By Premier Dental of Ohio

Inherited diseases and autoimmune disorders can affect every area of the human body, including the mouth.  There are many different rare dental conditions that can cause major problems throughout someone’s lifetime.  Because the list of genetic diseases that affect the mouth is extensive, this is a very limited list of some of the rare dental conditions that are present today.

Aggressive Juvenile Periodontitis

Periodontal disease is not rare at all.  Millions of adults all over the world suffer from periodontal disease, also called periodontitis.  It is, however, much rarer among children and teenagers.  Thankfully, most children do not develop severe gum disease despite poor oral hygiene.  The most that most young ones will suffer is some gingivitis (inflammation on the surface).

In rare cases, though, children can develop aggressive gum disease that progresses rapidly.  This means that the body responds to the bacterial toxins present in dental plaque by quickly and aggressively attacking the bone surrounding a tooth.  Most cases are “localized”, meaning they only affect a few teeth.  Slightly rarer is generalized aggressive periodontitis, which affects all of a child’s teeth.

The signs of aggressive juvenile periodontitis may not be easy for a parent to notice as the disease process occurs underneath the gum tissue.  It is necessary to take dental x-rays in order to properly evaluate the extent of the disease.  Inflammation in the gums and bad breath are the most likely signs detectable by a non-dental professional.

The treatment for aggressive juvenile periodontitis may include surgical and non-surgical therapy.  It will also include antibiotic therapy to fight the disease-causing bacteria present in the affected child’s mouth.  This type of treatment should be coordinated between a pediatric dentist and a periodontist (a specialist in the treatment of gum diseases).

Ectodermal Dysplasia

Ectodermal dysplasia is a genetic disease that affects the teeth, hair, and fingernails because these tissues all develop from the “ectoderm” during fetal development.  Dysplasia means the abnormal development of cells or tissues.  This rare condition affects less than 200,000 people in the US at this time.

This syndrome causes, most notably, multiple missing teeth.  The teeth that do form have an unusual, conical shape and are usually smaller than normal.

Treatment of the dental concerns caused by ectodermal dysplasia should be coordinated between a pediatric dentist and a prosthodontist (a specialist in replacing missing teeth).  A patient with ectodermal dysplasia is likely to go through multiple phases of dental prosthetics while he or she grows.  In adulthood, more permanent tooth replacements are possible.

Sjögren’s Syndrome

Sjögren’s Syndrome is an autoimmune disorder that causes severely dry mouths and eyes.  It can also cause chronic, difficult to manage pain throughout life.  The dental concern with Sjögren’s Syndrome is the negative effect that a dry mouth causes on oral health.

Extreme dry mouth greatly increases one’s risk for both cavities and gum disease.  It also leads to more frequent mouth sores, ulcers, and trauma to the tissues (like cheek and lip biting).

Your dentist will help you treat the oral symptoms of Sjögren’s Syndrome by taking many preventive measures.  Utilizing salivary replacement gels or salivary stimulants can help moisturize the mouth.  Your dentist may prescribe anti-cavity treatments, like professional fluoride applications, to fight the increased cavity risk that dry mouth causes.  Many patients with Sjögren’s Syndrome need to see the dental hygienist more frequently for professional teeth cleanings to lower the risk for gum disease.


This condition is not uncommon, affecting 2.5% of adult Caucasians.  The cause is unknown at this time.  Taurodontism is characterized by very large pulp chambers within a tooth.  The pulp chamber is the hollow chamber within each tooth that contains the nerves and blood vessels.  When enlarged, there is less hard tooth structure, so teeth may crack or break easily.  They also may experience a higher risk for dental abscesses because cavities that begin on the external tooth surface have a shorter distance to travel before reaching the nerve.

There is no treatment for taurodontism unless specific develop problems like cavities or fractures.  If decay reaches these enlarged pulp chambers, then root canal treatment is necessary.  Because of the unusual shape of the pulp chambers, we recommend that patients see endodontists (root canal specialists) for this treatment.

Amelogenesis and Dentinogenesis Imperfecta

These genetic disorders cause malformations in the developing teeth.  Amelogenesis imperfecta affects the development of tooth enamel, and dentinogenesis imperfecta affects the development of dentin (the hard tooth structure lying just underneath the enamel).

When someone suffers one of these conditions, the teeth are abnormal, weak, and highly susceptible to decay and cracking.  In many cases, all of the teeth require protection and coverage by dental crowns.  This treatment would not take place until the patient reaches adulthood.  A child with any of these conditions should see a pediatric dentist consistently for preventive treatments to protect and strengthen these weak teeth.

When the patient is fully grown, then a prosthodontist will work to protect and rehabilitate the teeth for a lifetime of health and good function.

Autoimmune Disorders affecting the Soft Tissues of the Mouth

There are multiple autoimmune disorders that can cause pain and/or visible abnormalities inside the mouth.  The most common ones are lupus erythematosus (SLE), pemphigus vulgaris, pemphigoid, and lichen planus.  These autoimmune disorders usually affect the skin and inner lining of the mouth.  They can cause painful ulcers, burning mouth syndrome, and sensitivity of the tissues lining the inside of the mouth.

Patients who suffer from any of these disorders must be very careful about their diets and oral care products.  Spicy or acidic foods will aggravate the tenderness, and some mouth rinses or toothpaste can lead to painful burning.  You should avoid any products containing alcohol or sodium lauryl sulfate (SLS) in order to prevent these symptoms.

The treatment of these disorders is only symptomatic when it comes to their oral manifestations.  An immunologist will treat the overall disorder, which could lead to improvement in the symptoms the patient experiences.

More Questions about Rare Dental Conditions?

Call your nearest Premier Dental of Ohio location to discuss your specific concerns with one of our great dentists.  We can schedule a consultation and help you get answers as soon as possible.

Premier Dental Shop


No items found.
View More ProductsView More ProductsView More ProductsView More ProductsView More ProductsView More ProductsView More ProductsView More ProductsView More ProductsView More ProductsView More ProductsView More Products

Join our 37,500+ patients who are maintaining healthy teeth and gums for life.

Find Your LocationSchedule online
Schedule Now