Dr. Wen-Ting Lin, Dr. Movina Wu &  Dr. Yu-Fen Yen
(Special Care Dental Center of NCKU Hospital)

 

Epidermolysis bullosa (EB) is a disease which affects various structural proteins that make up the skin, mucosa (including digestive system) and tooth. Therefore, EB not only affects patient’s appearance but also leads to fragile oral mucosa and poor dental condition. The severity of these conditions varies significantly within different EB types. Different treatment management might be carried out due to different level of mucosa and tooth structure fragility. Details of the proposed treatments will be discussed later. However, oral health prevention and avoid mucosa trauma during treatment are the most important rules of every kind of EB patients. Through maintaining good dental health and masticatory function, it reduces the risk of mucosal injury of digestive system and increase the healing process of bodily wound with nutritional balance and good intake. 

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Currently, EB has been classified with 4 major types: EB simplex, junctional EB, dystrophic EB, and Kindler EB (Kindler EB has few studies, we will not discuss this type in this article.). Identify the types of EB helps the dentists provide appropriate oral hygiene instruction, preventive oral care and dental treatment.

The oral manifestation of patients who was diagnosed with EB simplex (EBS) are similar with people without EB: No or very mild mucosal involved with normal tooth structure and dentition were noted. Besides, fusion of fingers and abnormal bending of joints was not found in the patients with EBS. They are able to brush their teeth without any difficulty. General oral health care and dental treatment can be performed by dentists. However, we still need to treat EBS patients with plectin mutation (which cause muscle weakness) very carefully during aerosol treatment and prevent airway obstruction.

Almost 100% patients with junctional EB (JEB) were diagnosed with enamel hypoplasia, which means lack of intact enamel structure (pitting tooth surface and excessive tooth wear was noted.) The result is a high dental caries rate among JEB patients (DMFT index is around two times than average. D=Decay, M=Missing, F=Filling). Compared to mucosa expression of EBS, patients with JEB often experience blister and ulcer by trauma and heat in oral mucosa. Therefore, in this type of patients, we should put in more effort on oral hygiene improvement and dental caries prevention. Measures such as regular oral health examination, regular fluoride supplement and full mouth scaling are recommended. If dental caries or any dental problem was detected, suitable dental treatments such as operative dentistry or endodontic treatment are necessary. No matter which dental procedure we choose to perform, avoid soft tissue trauma via the following methods is important. During dental management, saliva ejector and tube should rest on hard tissue instead of soft tissue. The frequency and strength of air blow decrease with the use of gauze or cotton instead (but rubbing and sliding movement are not allowed). Dental anesthesia injected into muscle layer directly is preferred, otherwise, blisters can form easily. Blood- or fluid-filled bullae that occur during treatment should be drained with a sterile needle or by a cut with scissors to avoid lesion expansion. 

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Dystrophic EB patients (DEB) show higher caries rate than average due to soft tissue lesion even if they have normal tooth structural surface (DMFT index is around one and half times than average, but lower than JEB.) Generally, DEB patients, especially recessive DEB patients (RDEB), are presented with very severe soft tissue fragility. In their growing process, blisters appear, and break and are healed with scar and fibrosis in the oral cavity and perioral soft tissue. Therefore, microstomia (average maximum mouth opening (MMO) in RDEB is only 12.6mm, while MMO is up to 35mm in general.), ankyloglossia, vestibule obliteration and pseudosyndactyly (lead to brushing teeth difficultly) were detected and lead to oral hygiene maintenance difficulty. Treating RDEB patients presents the biggest challenge for dentists due to poor vision and narrow working space. Besides avoiding soft tissue trauma (described above in treating JEB patients), lubrication with vaseline®/petrolatum, using pediatric instrument, and modified dental treatment (such as endodontic treatment without straight access and approach pulp from buccal side) will be needed during dental procedures. Sometimes, no matter how much effort we put in, some teeth still cannot be saved. Therefore, the importance of dental prevention and regular examination should be particularly emphasized since childhood. In addition, skin and oral cancer dominants high mortality rate in RDEB patients. Regular oral cancer screening (a laryngeal mirror can also be helpful in patients with severe microstomia) and pre-malignancy lesion removal are necessary. In some studies, facial stretch exercise is recommended to improve the mouth limitation. Early exercise since child may delay the rate of oral fibrosis. 

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No matter which type of EB, good oral hygiene is the most important purpose we need to fulfill. Furthermore, since traumatic pain and soft tissue trauma while brushing hard-to-approach areas in JEB and RDEB patients are a big obstacle of oral care, dentists play an important role and has the responsibility of preventing dental caries and periodontitis as much as possible. Through routine dental scaling, topical fluoride application and oral examination every 3 months and daily extra-flouride supplement using at home by patients, dental caries rate have been shown to decrease significantly. In addition, some assistive holding equipment and special floss for EB patients may be helpful for dental cleaning. Taking good care of teeth may bring better life quality for EB patients. 

In conclusion, identify the type of EB patients according to their oral manifestation or gene analysis when we first met them. It helps we provide suitable oral hygiene instruction and make treatment plan appropriately.