Dental treatment is an important part of the multidisciplinary care of patients with epidermolysis bullosa (EB). Routine dental treatment can be difficult to provide in patients with severe tissue fragility caused by mucosal sloughing, microstomia, ankyloglossia, and scarring. This article provides a review of dental practices, exploring different areas of oral management and highlighting the importance of early referral, preventive programs, and close follow-up to maintain oral health. It also discusses treatment modifications and precautions needed for each of the 4 mayor types of EB.
The approach to dental treatment of patients with epidermolysis bullosa (EB), in particular for those with the more severe types, has changed dramatically during the last 30 years. Crawford and colleagues in 1976 stated that extractions were the treatment of choice for patients with recessive dystrophic EB (RDEB). Two decades later, in 1999, Wright declared that it was possible to manage dental abnormalities successfully with a combination of anesthetic and restorative techniques. Recently Skogedal and colleagues showed that caries can be successfully prevented in patients with RDEB by continuous follow-up aimed at dietary advice, oral hygiene habits, frequent professional cleaning, and fluoride therapy. Thus, a preventive protocol is today’s dental management approach of choice.
Dental treatment is an important part of the multidisciplinary care of patients with EB, especially the severe types. Maintaining a functional dentition reduces the potential for oral and esophageal soft-tissue damage through more efficient mastication and favors nutrition.
The only level of evidence available to support most recommendations is case reports and a few case series.
Access to dental care
Access to dental care can be a challenge for some patients. Even although in most developed countries it is guaranteed, it is still a privilege for many patients around the world. There is a lack of familiarity with the disease in the dental profession and dental care can be complicated by the fears of the patient and the dentist.
Early referral
Patients with EB should be referred to a dentist as early as possible to identify any related feature that needs special attention, for example generalized enamel hypoplasia. Whenever possible, patients should be managed by specialized dental services. This strategy enables dentists to start preventive programs and reduces the risk of developing dental diseases. Many case reports have shown that patients visit the dentist only when they already have several carious lesions or pain. However, members of the multidisciplinary team should refer patients to the dentists before oral problems present, as early referral and close follow-up are the keys to maintain oral health.
Early referral
Patients with EB should be referred to a dentist as early as possible to identify any related feature that needs special attention, for example generalized enamel hypoplasia. Whenever possible, patients should be managed by specialized dental services. This strategy enables dentists to start preventive programs and reduces the risk of developing dental diseases. Many case reports have shown that patients visit the dentist only when they already have several carious lesions or pain. However, members of the multidisciplinary team should refer patients to the dentists before oral problems present, as early referral and close follow-up are the keys to maintain oral health.
Treatment modifications: precautions
Even although patients with milder oral involvement do not require many modifications to the treatment, a careful approach benefits every patient. Patients with the severe generalized RDEB subtype of EB require the most specific precautions during treatment to minimize soft-tissue damage.
EB Simplex
Most investigators agree that routine dental treatment can be provided for patients with EB simplex (EBS). Clinicians should, however, assess history of mucosal fragility because manipulation can precipitate lesions in mildly affected patients.
Junctional EB
Because there is minimal scarring, dental management of junctional EB (JEB) has been described as not requiring many modifications. Mucosal and skin fragility, however, vary considerably between subtypes of JEB and patients. The avoidance of adhesive contact with the skin and careful manipulation are always advised. This group of patients require a special dental rehabilitation plan, as they present with generalized enamel hypoplasia ( Fig. 1 ).
Dominant Dystrophic EB
Patients with dominant dystrophic EB (DDEB) can receive routine dental treatment with little or no modifications. A report describes a patient wearing dentures for several years without difficulties.
RDEB
As patients with severe generalized RDEB present with severe mucosal fragility, oral mucosa blistering, ankyloglossia, vestibule obliteration and microstomia; they require several treatment modifications and a careful approach to avoid as much tissue damage as possible. Ideally, the management of these patients requires a well-organized multidisciplinary team approach.
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Lubrication: Lips, buccal mucosal, gloves, and instruments should always be lubricated before any procedure is performed to reduce adherence and formation of bullae and erosions. Vaseline, petroleum jelly, and hydrocortisone ointment have been used for this purpose.
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Suction tip: Bullae formation or epithelium sloughing can occur on minimal contact with the suction tip. It is suggested to lean the suction tip on hard tissue (ie, on tooth surface).
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Bullae: Blood- or fluid-filled bullae that occur during treatment need to be drained with a sterile needle or cut with scissors to avoid spreading.
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Pressure: Extreme care of the fragile tissue in the patient with RDEB is important. To handle the tissues, a little pressure (compressive force) can be applied, but sliding movements (lateral traction or other shear forces) should be avoided, as these can cause tissue sloughing.
Kindler Syndrome
A careful approach to Kindler syndrome is advised as blisters can form after dental treatment such as scaling. The scarce literature available suggests periodontal health as a main area of concern for dental therapy.
Oral blisters
Only 1 study has been published on a therapeutic approach for oral blisters. Marini and Vecchiet studied the effectiveness of sucralfate powder on the oral mucosa of 5 patients with DEB. They concluded that sucralfate seemed to be a cost-effective treatment to reduce oral blisters and discomfort.
Caries prevention
Although systemic treatment of EB remains primarily palliative, it is possible to prevent destruction and loss of dentition through appropriate interventions and dental therapy, even in the most severely affected patients with EB. Skogedal and colleagues presented a poster of successful caries prevention in 5 patients with RDEB, among whom after 10 to 13 years of continuous follow-up, only 1 presented with 2 caries and the other 4 were caries free. In 2 of these patients preventive extractions of second and third molars were performed as they were experiencing problems in performing oral hygiene because of reduced mouth opening.
Oral Hygiene
Concern is expressed by some patients, parents, and dentists regarding the use of tooth brushes and potential damage to the oral mucosa. Dentists have described that “preventive techniques, such as regular brushing, are too traumatic for these patients” and several patients do not brush their teeth at all because of bullae formation. However, tooth brushing is possible in all patients with EB, even in patients with the severe generalized RDEB subtype ( Fig. 2 ). The following suggestions may help to find the appropriate tooth brush for each patient:
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Small head.
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Soft bristles, which can be further softened by soaking in warm water.
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Short bristles may be needed in severe microstomia. For this purpose bristles can be cut.
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Special adaptations of the toothbrush handle can help patients with mitten deformities.
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Parents are advised to assist children to improve plaque removal and reduce tissue damage.
Adjuvant Therapies
Fluoride and chlorhexidine have been widely advised for oral disease prevention in patients with EB. A variety of application methods have been used, including mouth washes, swabs, sprays, gels, and topical varnish. The gel has been said to have a better taste than mouth wash (and is therefore preferred). Topical application of high-dose fluoride varnish each visit is preferred by some investigators. For children living in nonfluoridated communities, daily fluoride supplements have been suggested. The main concern in EB is that patients with oral lesions can be sensitive to flavoring agents, acids, and alcohol in dental preparations. Neutral, nonflavored, and alcohol-free formulations are advised.
Diet
The patients’ diet can be an important factor in their increased risk of caries, as they usually choose soft, pureed, or liquid food to avoid mucosal damage, and consume it in small amounts throughout the day. They have a prolonged oral clearance time and may be taking medicines that contain sugar. A thoughtful dietary caries prevention program is important and should begin at an early age. Suggestions to reduce prolonged oral clearance include increased fluid intake while eating and rinsing with warm water during the day, especially after meals. It is important that dentists and nutritionists work together on thoughtful programs for each patient, instead of giving confusing contradictory advice.
Fissure Sealants and Other Aids
Sealing fissures and fossae has been recommended, as oral hygiene and other preventive measures can be difficult to perform. Fitting preformed stainless steel crowns has also been advised.
Related posts:
How to Take Skin Biopsies for Epidermolysis Bullosa
Epidermolysis Bullosa Simplex with Muscular Dystrophy
Genitourinary Tract Involvement in Epidermolysis Bullosa
Growth and Pubertal Delay in Patients with Epidermolysis Bullosa
Epidermolysis Bullosa Care in Germany
Epidermolysis Bullosa Care in Israel
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