TEETH Dominant Dystrophic Epidermolysis Bullosa

TEETH Dominant Dystrophic Epidermolysis Bullosa
TEETH
Dominant Dystrophic Epidermolysis Bullosa (DDEB)
1. Introduction
Dental problems can occur with different frequencies and different degrees of
severity in many forms of EB. In some (not many!) forms of EB tooth damage is
hard to avoid, since genetic enamel defects exist. But even with forms of EB where
there are no enamel defects, normal dental condition often leads to tooth decay,
because oral hygiene can be difficult due to blister formation or difficulty in opening
the mouth.
With appropriate care, a dental healthy diet, regular dental check-ups and
professional oral hygiene, damage to the baby teeth as well as the permanent teeth
due to caries in many cases is preventable. It is worthwhile in any case - despite all
the other worries and problems caused by EB - to pay regular attention to your
teeth and their care. When parents and children are well informed and from the
beginning a good working relationship exists between the family, the supervising
dentist and EB-specialists, the condition of the teeth can generally be positively
influenced.
Important points in a nutshell
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Dental care starting with the first tooth is important in EB to avoid
many subsequent problems.
It is based on regular teeth cleaning at home, at best twice daily with
a soft toothbrush, with a small brush head and age-appropriate
fluoride toothpaste.
Find a good dentist as early possible, because professional cleaning
and rapid intervention when problems arise is necessary.
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© A. Diem, B. Sailer: Teeth_DDEB, 09/2014
Translated by Lynne Hinterbuchner
2. General Information
In epidermolysis bullosa dental and oral hygiene may be difficult due to blistering of
the mouth. The best possible dental and oral hygiene is necessary, despite the risk
of blistering, to avoid developing dental caries. A healthy and functional set of teeth
is very important for eating and nutrition.
People with dystrophic epidermolysis bullosa have several problems with their teeth
(tooth enamel, caries) and/or their oral mucosa. Excessive tooth decay with risk of
premature tooth loss affects all severe forms of EB. Blisters and erosions on the lips,
tongue and oral mucosa complicate a consistent oral hygiene and food intake.
Frequently it comes to changes of the gums caused by chronic inflammation of the
gums (gingivitis) and mechanical damage by the necessary dental care
Tooth decay, even in dominant dystrophic epidermolysis bullosa with normal tooth
quality can develop. Oral hygiene can be difficult due to blister formation or limited
ability to open the mouth. Younger patients often suffer a rapidly progressing
destruction of the teeth. As a result, this creates additional problems in food intake.
However, the prognosis of the disease depends largely on an adequate diet and
thus on the state of the teeth.
Nutrition in itself is a big problem; often the person concerned cannot eat - and/or swallow the calories they need, so they will often consume a lot of high calorie
foods, often food containing a high amount of sugar. This is understandable, yet it
does not make the dental problems any simpler.
Once a cavity occurs you subsequently develop pain. When this is the first dental
visit that has taken place, it is not uncommon to be associated with a poor
experience. It may be that made fillings are needed or even teeth are pulled. This is
sometimes only possible under general anesthesia, which is often not easy in EB.
Furthermore, opening your mouth (to brush your teeth, or by the dentist) is often
very limited, so brushing is already difficult. The severity of these problems is
usually mild in dominant dystrophic epidermolysis bullosa, but nevertheless
important to note.
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© A. Diem, B. Sailer: Teeth_DDEB, 09/2014
Translated by Lynne Hinterbuchner
3. Options for Prevention of Dental Caries
With appropriate dental care at home, a healthy dental diet, regular dental checkups and professional oral hygiene, the destruction of the milk teeth as well as the
permanent teeth due to caries in many cases is preventable.
Special Measures:
Consistent daily teeth brushing/oral care
Regular professional cleaning by a dentist/dental hygienist
Fluoride prophylaxis
The classical pillars of prevention include "diet", "oral hygiene", "Fluoride" and
"fissure sealing". Fluoride tablets now play a secondary role. More important is the
topical fluoride treatment. This includes child fluoridated toothpastes and
fluoridation with coatings or gels applied by the dentist. Mouthwashes are only
useful if the children have learned to spit the mouthwash back out properly. The
nature and frequency of the respective fluoride treatment should be discussed with
your dentist. Professional oral hygiene in a dental office, in conjunction with the
examination of all teeth should be done at least 4x annually, in severe forms of EB
more frequently.
When special requirements are needed, oral hygiene can be supplemented by
special mouthwashes such as chlorhexidine. There are chlorhexidine rinses as well
as gels; the mode of administration should always be discussed with a dentist. This
treatment usually takes place as a short-term treatment for about one to two
weeks, which may be repeated periodically as needed. Prolonged use will lead to
discoloration of the teeth and an irritating taste left in the mouth. The repeated use
of chlorhexidine about every 2-3 months for a period of 1 week has been proven to
be effective in children with EB. A more recent approach is the use of pastes made
from a calcium and phosphate base. This has to be used in addition to fluoride
toothpaste and is well accepted by most children because these pastes do not
irritate the mucous membrane and have a pleasant taste. These pastes (e.g. GC
tooth mousse) are only available from dentists or from the Internet.
In addition to the above-mentioned fundamentals of prevention, namely "diet",
"oral hygiene" and "fluoridation" is dental sealing of molar fissures. Dental sealing is
the only measure which lies solely in the hands of the dentist. For this treatment a
plastic coating is applied to the surfaces of the molars (back teeth used for chewing)
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© A. Diem, B. Sailer: Teeth_DDEB, 09/2014
Translated by Lynne Hinterbuchner
this can prevent the development of dental caries. The teeth need to be kept
completely dry for a short time, which can be difficult when you have a hard time
opening your mouth.
4. Age Related Information
4.1
Infants
For the "primary prevention" of the so-called "early childhood caries" (formerly
called bottle tooth decay) you should pay attention to the spread and prevention of
dental caries. This usually starts with a rapidly progressive destruction of the upper
incisors. While it used to be caused by instant baby teas that were offered in the
baby bottle, today it is rather caused by supposedly healthier fruit juices. Pure fruit
juice also contains fructose which corresponds to approximately a 10% sugar
solution. In combination with a very low pH in juices, continuous bottle drinking
leads to massive early destruction of the milk teeth. Studies have shown that
children with early childhood dental caries that have been treated are still at a
higher risk of developing dental carries later in life.
Infants are often found to have a greater acceptance of a toothbrush during the socalled "oral phase" because the mouth is the contact element to the outside world.
This is just as frequently followed by an absolute "Toothbrush refusal" phase.
Even with “tooth brushing opposition" consistency is important, it often helps to
integrate teeth brushing into a ritual. The objectives in this age group are
preventing the transmission of germs and making oral hygiene a "daily habit”.
The bottle should be weaned as soon as possible, this is usually around the first
birthday and the child should then become accustomed to drinking water from a
cup.
In the so-called "window of infectivity" (window for infection), between the age of
6-30 month - is the phase where the “milk teeth”, primary dentition break through
the gums. During this period of time, the child can be infected with caries from
contact with the saliva of caregivers, usually their mother. The top priority at this
time is to keep the milk teeth healthy. Therefore, an early examination by a dentist
is important. In this way, the visit is experienced in a relaxed atmosphere and time
can also be used by the dentist for educating the parents.
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© A. Diem, B. Sailer: Teeth_DDEB, 09/2014
Translated by Lynne Hinterbuchner
4.2
Toddlers
Starting between the ages of 2 - 3 years, most children want to try to brush their
own teeth. This is important so that they can learn a certain technique and not lose
the desire to brush their own teeth. Kindergarten aged children are particularly
susceptible to praise and reward, they often live in a fantasy world that can be
supported by stories or songs in oral hygiene. Make brushing teeth fun.
The best way is to always clean with the same technique; we recommend the "CEI"
system that is - chewing surfaces 1st, then Exterior surfaces followed by Interior
surfaces. Tooth brushing should be started as soon as the first tooth breaks through
the gums. No later than the 1st birthday a tiny bit of fluoridated toothpaste should
be used, at least 1 time a day, preferably in the evening. It is particularly important
that the parents help with brushing and check for spots that are missed. When the
permanent teeth breakthrough the gums, you should change the toothpaste from a
child’s to a junior type of paste. Tooth paste containing Menthol can cause
discomfort for children with EB. In this case a tooth paste without menthol, yet
containing fluoride is a good alternative.
Particularly dangerous are frequent snacks!
Even seemingly healthy things such as bananas can cause caries due to their
stickiness. If you cannot reduce eating snacks due to general medical reasons and
teeth cleaning after every meal is not feasible, you can make do with a little
compromise: for example a dental chewing gum, which stimulates the flow of saliva
and promotes self-cleaning or at least rinsing your mouth with water between meals
and snacks.
4.3
Children
It is not until about 10 years of age that the fine motor skills are advanced enough
that brushing your own teeth is possible. Electric toothbrushes are from about
kindergarten age just as suitable for use as manual toothbrushes. Many children
find electric toothbrushes simply "exciting" and are therefore more motivated to
brush their teeth. Electric tooth brushes are especially helpful with disease-related
limitation of fine motor skills in dystrophic EB. It is also easier for the parents to use
an electric tooth brush when helping the child get spots they have missed while
brushing.
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© A. Diem, B. Sailer: Teeth_DDEB, 09/2014
Translated by Lynne Hinterbuchner
However, an effective plaque removal by the child himself is not possible until about
the age of 10. Until then, the parents really need to check for spots missed. Good
teeth brushing technique and help from the parents is far more important than the
frequently asked question about the best toothbrush or toothpaste to use. True to
the saying "you cannot teach old dog new tricks" means that behaviors that are
practiced in infancy are very resistant to future changes. It does not matter whether
these behaviors are positive or negative. In other words, at this age the foundations
should be laid for a good dental health-conscious life.
4.4
Adolescents
The closer the children get to puberty, the less parents can influence choices in diet
and oral hygiene. With pocket money they can buy sweets and oral hygiene is no
longer regularly monitored by the parents. Negative motivation along the lines of "if
you eat too many sweets and do not brush your teeth, you’re going to get bad
teeth" is usually unlikely to be successful. It is usually better to use positive
motivation, for example, “beautiful teeth look so attractive”.
The aim of these measures is to motivate children from the beginning, so that diet,
professional prophylaxis and oral hygiene, at home are included in a daily routine so
that as adults the risk of caries can be held correspondingly low.
Another topic at this age can be braces. In such cases, a precautionary approach is
recommended. Especially important is a good working relationship with the
orthodontist.
4.5 Adults
In adulthood, the regular daily dental care, professional prophylaxis and dental
appointments should be routine. Problems that might arise in dominant dystrophic
epidermolysis bullosa should be discussed with a dentist.
The restoration of already damaged teeth by removing corroded altered tooth
substance and the reconstruction of the tooth structure and function of teeth, with
fillings and "protective crowns" is sometimes necessary. The extraction of badly
damaged teeth to eliminate inflammation is needed to prevent bacterial infections
and abscesses. Root canal treatments are possible, treatment with implants can be
difficult, yet with DDEB it is possible under certain conditions.
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© A. Diem, B. Sailer: Teeth_DDEB, 09/2014
Translated by Lynne Hinterbuchner
5. Special Problems and Therapy
At this point we would like to mention a few products to help make your search a
little easier, with products that have proven effective for EB. However, we would like
to point out that there are a large number of similar products that have the same or
a similar effect. Which products you use, should be discussed with your dentist.
For rinsing and treatment of open areas on the inner cheek, sage tea or chamomile
tea have been found to be useful. An oral ointment (Kamistad A gel, available in
Germany), which also contains a topical anesthetic in addition to chamomile extract,
is another option. Other topical anesthetic creams, also used for pressure areas
from false teeth, are for example Solcoseryl ointment or Dynexan gel. While the
open areas do not heal faster, it does take some pain away, for example before
eating. For rinsing we can recommend Bepanthen mouth rinse.
EB children:
Non-alcoholic mouthwashes containing chlorhexidine ("CHX", has an antibacterial)
and fluoride (caries protection) for the daily care:
Gum Paro ex 0.06% CHX + 950ppm sodium fluoride
Curasept rinse solution 0.05% CHX + 0,05% fluoride bottle 200 ml
Mirafluor Liquid CHX 0.06% CHX + 250ppm amine fluoride
Elmex caries protection 0.025% sodium fluoride rinse bottle 400 ml
Meridol med 0.2% CHX
What form the toothbrush should have should be discussed together, at this time
for children with EB. Depending on the severity of the disease softer bristles or a
small brush head may need to be used. Ultrasonic powered toothbrushes with a
very small brush head have proven useful. Depending on the severity of the
disease, the condition of the teeth and of course the ability to open the mouth one
must improvise with children affected by EB. Also aids such as interdental brushes,
Q-tips or gauze swabs can be used, the dentist or dental assistant should find the
aids that are most suitable for the child.
Local anesthesia:
Whether a dental treatment can be performed under local anesthesia or not
depends amongst other things on the cooperation of the patient, the possibility of
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© A. Diem, B. Sailer: Teeth_DDEB, 09/2014
Translated by Lynne Hinterbuchner
adequate mouth opening and the extent of blister formation and the need for
treatment.
General anesthesia:
In some cases it may be useful or even necessary to perform dental treatment
under general anesthesia. Indications in favor of such an approach are for example,
too small of a mouth opening, severe pain and blistering, or the need for extensive
treatment.
Page 8 of 8
© A. Diem, B. Sailer: Teeth_DDEB, 09/2014
Translated by Lynne Hinterbuchner
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