First, Do No Harm
The dental management of patients with epidermolysis bullosa
Mark L. Cannon, DDS, MS
Dental health professionals are usually quite enthusiastic about helping their patients, as the vast majority made that long and financially burdensome commitment to dental education with the best of intentions. Sadly, in many situations, it takes more than just enthusiasm to correctly treat and actually benefit our patients. Nothing can substitute for training and experience, but it would take a lifetime for dental education to properly equip practitioners for all situations, especially those involving special needs patients. Perhaps that is why G.V. Black so succinctly stated, the “dental professional person has no right to be other than a continuous student.”1 Providing necessary dental care for patients with severe, life-changing, special needs is rewarding in and of itself; however, contributing to the advancement of special care in dentistry by innovation and education makes it doubly so.
Although there are roughly 300 genetic mutations, epidermolysis bullosa (EB) is traditionally divided into three main groups: epidermolysis bullosa simplex, junctional epidermolysis bullosa (JEB), and dystrophic epidermolysis bullosa (DEB).2 Epidermolysis bullosa simplex is the type that creates blisters at areas of friction and often affects the hands and feet. It is typically inherited in an autosomal dominant manner, affecting the keratin genes KRT5 and KRT14. JEB is the variant of the condition affecting laminin and collagen. JEB is characterized by blister formation within the lamina lucida of the basement membrane zone and presents with blisters at the site of friction, especially on the hands and feet. It is inherited in an autosomal recessive manner.3 This type of EB is estimated to occur in less than one person per million.4 And finally, DEB is an inherited type that affects the skin and other organs. It is caused by genetic defects (or mutations) within the human COL7A1 gene encoding the protein type VII collagen. DEB-causing mutations can be either autosomal dominant or autosomal recessive.5 Oftentimes, the term “Butterfly Children” is used to describe younger patients with the condition because their skin is said to be as fragile as a butterfly’s wings.
Articles and textbook references describing dental treatment and oral care for patients with EB are sparse, often dated, and lacking in discussion of preventive care.6-9 A major concern with DEB patients is microstomia (ie, the narrowing of the oral dimensions and commissure contracture due to scarring).10 Prevention of scarring during oral procedures is critical, not only to protect the tissue, but also to spare the patient any unnecessary discomfort. This article discusses the proper armamentarium required to successfully treat the special needs of a pediatric patient with DEB. First, do no harm—obtain the necessary adjuncts to protect and maintain the skin’s integrity.
All EB patients should receive the same general precautions to prevent blisters and their concomitant pain as well as scarring. To facilitate this, practitioners who treat patients with EB should assemble an “EB kit.” The EB kit contains more than 20 items, all necessary to provide the best possible care for these special needs patients. The contents of the kit will be described in the following protocol for treating a pediatric patient with EB.
The first step is always to assure the patient that ALL available measures will be used to reduce any skin complications and to show him or her what is going to be applied. The gloves should be lubricated, either with K-Y Jelly and chlorhexidine or with coconut oil. Organic, extra virgin, non-fractionated coconut oil that is used for cooking is a very good choice for a lubricant and is already used by many EB patients. However, be sure to identify any patients with coconut sensitivity before using it. In addition, K-Y Jelly is very well tolerated, and the chlorhexidine, besides being antimicrobial, which helps prevent secondary infections, is also an enzyme inhibitor that can help reduce skin blistering and inflammation. The face of the patient should also be lubricated, usually only with coconut oil, while the lips and oral mucosa should receive a coat of Carafate suspension (sucralfate) for added protection (Figure 1). Carafate suspension seems to prevent the formation of intra-oral blisters. It also reduces the discomfort from the ulcers and accelerates the healing process. Pressure points should be protected with Gel-E Donuts (Philips Medline) and lubricated gauze. In addition, all team members need to be instructed on the contact rules to ensure that no frictional forces are used during the procedure.
Once the patient has been properly protected, the dental prophylaxis may be completed utilizing a Butler® Prophyciency™ cup without abrasive. It is important to avoid using abrasives, not only because they can create a blister, but also because they can possibly contaminate the wound. To avoid the accidental contact of tubing or lines with the patient’s skin, the use of a portable polishing device is recommended (Figure 2). If the practitioner is careful, scaling may be accomplished, but this requires extra due diligence. Intraoral radiographs are almost impossible to perform with a CCD (charge coupled device) sensor, due to their thickness and rigidity. However, the radiographs may be obtained using PSP (phosphor storage plate) sensors, which are much thinner and more flexible. To further protect the patient, the sensors should be wrapped with cushions soaked in Carafate suspension (Figure 3). Due to the incidence of enamel hypoplasia and hypomineralization among these patients, MI Varnish™ and MI Paste™ (GC America) are used as a preventive treatment. Patients with EB seem to significantly benefit from xylitol products, especially the low abrasive tooth gels and the mouth sprays, such as Rain™ Oral Mist (Xlear) or CTx™2 Spray (CariFree). Due to the weeping ulcers, soft diet, and enamel defects, the oral microbiome of EB patients expectedly shifts to a more pathogenic nature, and xylitol and erythritol can be beneficial in modifying the pathogenic biofilm (Figure 4).
Simple operative procedures may be performed in the dental office setting. Nitrous oxide analgesia delivered using a lubricated Porter Silhouette nasal hood can provide adjunctive relief. In addition, the use of single tooth anesthesia (The Wand® STA® Milestone Scientific) has been well accepted by EB patients. The lack of adjacent soft tissue anesthesia greatly reduces the chance of accidental blister creation, especially post procedure. Proper insertion of the needle tip into only the periodontal ligamentary space is necessary. Placement of a rubber dam, which is well lubricated on the surfaces that contact tissue, can help to further protect the patient’s face. The biggest confounding factor among these patients is the presence of microstomia. Sometimes the result of scarring from previous dental visits due to lack of protective measures, the extremely small openings often impede dental procedures, requiring the adoption of special techniques and equipment. A set of laryngeal mirrors is absolutely necessary, along with modified bite blocks and customized quadrant isolators for when use of a rubber dam is impossible (Figure 2). Among patients with microstomia, ultrasonic cavity preparation is often the only appropriate technique for caries treatment in the posterior areas, as there are no handpieces available that can safely fit or be used in those impossibly tight situations (Figure 5).
Microstomia treatment by expansion should only be attempted by someone experienced with the dental care of EB patients. While caution is advisable, the ill effects of microstomia are multitudinous and tissue expansion is predictable in EB patients.11 Indeed, the use of a properly lubricated microstomia treatment appliance is necessary for the well-being of the EB patient with microstomia. The modification of existing commercial appliances (Figure 6) and the construction of custom appliances requires a significant time investment by the pediatric dentist, and normally there is little or no compensation (Figure 7). However, for many dentists, restoring the oral functionality of these patients and hearing their mothers say things like, “My daughter can eat with a fork again!” is reward enough. The microstomia treatment takes roughly three years, beginning with correcting the soft tissue and increasing the mandibular opening, followed by the use of special arch expanders, all of which require numerous adjustments (Figure 8).
Full mouth rehabilitation may require general anesthesia and the assistance of experienced pediatric anesthesiologists. Nasal intubation is preferred as bullae do not form in the trachea or nasal passageway, and it is performed with fiber-optic visualization (Figure 9). Some institutions mistakenly rely on laryngeal masks or oral intubation, possibly due to a lack of experience with EB patient care. When anesthetizing EB patients, the full patient protective measures previously described for dental treatment are indicated. The pulse oximeter should be applied to the ear lobe and held in place by the fabrication of a “c” clip cut from a facial mask, while the rest of the mask is placed reverse style on the patient to safely retain the nasal trachea tube (Figure 10). After venipuncture using the AccuVein®, the IV line is maintained with lubricated gauze wrap.
For young patients diagnosed with EB, every attempt should be made to save all of the dentition. Researchers in the field have indicated that they believe they may only be a few years from developing a successful treatment for EB that would prevent the formation of new bullae. If that is truly the case, then these young patients will only be left with two major hurdles: the scars and disabilities they already have and the state of their oral health.
References
1. Joseph R. The Father of Modern Dentistry - Dr. Greene Vardiman Black(1836-1915). J Conserv Dent. 2005;8:5-6.
2. James W, Berger T, Elston D. Andrews’ Diseases of the Skin: Clinical Dermatology. 10th ed. Philadelphia, PA: Saunders; 2006.
3. Freedberg, et al. Fitzpatrick’s Dermatology in General Medicine. 6th ed. New York, NY: McGraw-Hill; 2003.
4. NIH. Junctional Epidermolysis Bullosa. Genetics Home Reference. US national Library of Medicine. https://ghr.nlm.nih.gov/condition/junctional-epidermolysis-bullosa. Published June 27, 2017. Accessed June 27, 2017.
5. Varki R, Sadowski S, Uitto J, Pfendner E. Epidermolysis bullosa. II. Type VII collagen mutations and phenotype–genotype correlations in the dystrophic subtypes. J Med Genet. 2007;44(3):181-192.
6. Wright T, Fine JD, Johnson L. Hereditary epidermolysis bullosa: oral manifestations and dental management. Pediatr Dent. 1993;15(4):242-248.
7. Wright T. Comprehensive dental care and general anesthetic management of hereditary epidermolysis bullosa: A review of fourteen cases. Oral Surg Oral Med Oral Pathol. 1990;70(5):573-578.
8. Peñarrocha M, Larrazábal C, Balaguer J, Serrano C, Silvestre J, Bagán JV. Restoration with implants in patients with recessive dystrophic epidermolysis bullosa and patient satisfaction with the implant-supported superstructure. Int J Oral Maxillofac Implants. 2007;22(4):651-655.
9. Lanier P, Posnick W, Donly K. Epidermolysis bullosa--dental management and anesthetic considerations: case report. Pediatr Dent. 1990;12(4): 246-249.
10. Krämer S. Oral care and dental management for patients with epidermolysis bullosa. Dermatol Clin. 2010;28(2):303-309.
11. Whitney TM, Ramasastry S, Futrell JW. Combined tissue expansion and free tissue transfer for reconstruction of the hand in epidermolysis bullosa-associated malignancy. Ann Plast Surg. 1993;31(6):552-555.
About the Author
Mark L. Cannon, DDS, MS
Founder, Senior Partner
Associated Dental Specialists of Long Grove
Long Grove, Illinois
Diplomate, American Board of
Pediatric Dentistry
