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Jeffrey H. Camm, DDS, Lt Col; Scott E. Gray, DDS, Major; Thomas C. Mayes, MD, Major

Combined medical-dental treatment of an epidermolysis bullosa patient Epidermolysis bullosa presents a wide range of clinical symptoms. In this case, a patient had recessive dystrophic epidermolysis bullosa that required dental treatment. Standard protocol modifications and medical considerations were required in preparation for general anesthesia. Postop erative follow-up, including monitoring the relationship of the patient’s disease state to dental health, is recommended

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pidermolysis bullosa (EB) is a group of congenital chronic noninflammatory skin diseases in which slight mechanical trauma causes large bullae and erosions to the skin and oral m ~ c o s a .The ~-~ disease is most commonly divided into three main groups: epidermolytic EB, characterized by nonscarring lesions; junctional EB, characterized by skin atrophy; and dermolytic EB, characterized by scar lesions and skin a t r ~ p h y .A~ . ~ fourth group of nonhereditary origin, EB aquisita, has also been described, and as many as 18 different varieties within these four main groups has been re~ognized.~ Epidermolysis bullosa diseases may affect the oral mucosa and teeth.The wide range of clinical features may include vesicles that arise spontaneously or as a result of friction,4r6esophageal strictures, corneal erosions, mitten deformity of the hands and feet, loss of nail beds, scarring of the buccal mucosa, and hypoplastic defects of the ename1.2-5*7 The pathogenesis is poorly understood for all groups and may be the result of structural defects or enzySome of the dematic abnormalitie~.~,~ fects in the tissues include hypoplasia of the hemidesmosomes, defects in anchoring fibrils, and a decrease in anchoring fibrils and collagen.*The modes of transmission may be autosoma1 dominant, autosomal recessive, or X-linked.2,3 The type of disease, its severity, and extent affect the prognosis of the disease. Some forms have minimal complications while others include secondary infection of the vesicles, nutritional deficiency caused by painful oral ulcerations or esophageal strictures, widespread scarring, syndactyly, and debilitating flexion c ~ n t r a c t u r e . ~ , ~ Treatment modes for EB are prima-

rily palliative. Antibiotics and steroids may be prescribed to control the vesicle formation and limit secondary i n f e ~ t i o n Both . ~ , ~ diphenylhydantoin and vitamin E have been used to reduce and control blister formation.3,* Plastic surgery may be indicated to correct digital deformities and dilatation of the esophagus may be attempted to relieve esophageal stricture.2,* In this report, a patient with dermolytic EB reported for routine dental treatment and was found to have multiple carious teeth. Consultation with her physician indicated nonemergent medical needs. The management of a systemically debilitated patient is discussed, with emphasis on the need for health care providers to seek multi-disciplinary treatment for such patients.

Report of case A five-year-old girl reported for routine dental examination and treatment. Her medical history showed recessive dystrophic epidermolysis bullosa, diagnosed when the patient was 1week old. Numerous bullae and vesicles coated the patient’s tongue and oral mucosal tissue. Her oral stoma was very small and round, due to continual bullae formation, healing, and scarring at the oral commissures (Fig 1).Examination was compromised by the patient’s limited opening, and the pain associated with tissue manipulation. Numerous carious teeth were noted, as well as mandibular anterior crowding. Radiographs were not taken because of poor patient cooperation and the potential for further tissue damage. Clinical symptoms of EB included tapering of the fingers and toes with loss of nail beds (Figure 2), multiple

Fig 1.Small stoma, secondary to continual bullae formation and healing.

skin bullae, and mild flexion contracture . Her medications included prednisone, diphenylhydantoin and vitamin E to limit bullae formation and esophageal strictures. Her diet was totally pureed for patient comfort and to limit intra-oral and esophageal damage. Because of the extent of dental treatment required, the patient’s behavior, and the medical condition, it was decided that the patient must be treated as an inpatient, with general anesthetic. The patient’s physician, a pediatric intensivist, indicated a nonemergent need for balloon dilation of the esophagus, to attempt widening of the numerous strictures. It was previously thought that the risks of dilation and general anesthesia were not warranted for the strictures. Because it was necessary to perform the dental treatment while the patient was under general anesthesia, it was an opportune time to perform the esophageal dilation also, thereby limiting the number of general anesthetic procedures. The possible damage of a nasoendotrachial tube to the nasal mucosa, made an oral endotracheal airway necessary to deliver the anesthetic gas. The airway was coated with 1%hydrocortisone cream and held in place by the anesthesiologist.Tape was not used for securing the tube or for any other procedure since tape removal would precipitate bullae formation. The intravenous needle was secured with gauze and the patient’s arms wrapped in gauze before the blood pressure cuff was placed, to minimize bullae formation. To reduce frictional trauma, the operating room table was covered with foam and sheepskin. Before the dental procedure, the

patient’s throat was packed with hydrocortisone coated gauze. All instruments used for retraction of the buccal mucosa were broad surfaced to dissipate the forces applied to the tissues. They, too, were coated with hydrocortisone cream. Despite using care and delicately manipulating the tissues, a significant amount of sloughing occurred (Fig 3). The patient’s mandibular right and left primary canines were grossly carious and extraction was chosen as the definitive treatment, because of the extreme crowding. Space maintainers were considered a relative contraindication because the appliances could irritate the mucosa and aggravate bullae formation. All four maxillary primary incisors were mobile (early exfoliating) and were extracted to limit any anticipated irritation and trauma. Hemostasis was easily obtained by di-

Fig 2. Tapered, scarred fingers with loss of nail beds.

rect pressure. After the dental treatment, including the alloy restoration of four primary teeth, the esophageal dilation was successfully performed by the medical team. The patient was taken to the intensive care unit. While there, the patient was fed parenterally. Postoperative rounds revealed multiple intraoral bullae formation, but with little apparent morbidity to the patient. On the third postoperative day, the patient was taken to the children’s ward, the IV discontinued and a pureed diet reinstated. Postoperative recovery remained uneventful. The patient returned for her scheduled one month postoperative examination with diminished intraoral and perioral lesions. The parents were instructed about the patient’s diet and hygiene and another routine dental ex-

amination was scheduled.

Discussion The extreme form of epidermolysis bullosa can be quite debilitating. Chronic pain, mucosal ulcerations, secondary infections, and limited jaw opening are some of the complications affecting the patients. Carefully orchestrated treatment, using atraumatic procedures must be accomplished to minimize morbidity. Combining dental and medical procedures in these patients is a viable and effective treatment mode.9 With many EB patients, routine treatment modalities must be modified or eliminated. For example, the use of tape may cause frictional trauma and should be avoided. The clamp of a rubber dam can cause trauma of the gingival or perioral tissues from friction. Its use should be considered carefully and may not be warranted. While naso-tracheal intubation is preferred for most dental patients, it becomes traumatic in these patients and therefore should be avoided, in favor of oral intubation. The obvious exception is when the oral stoma is so small that it prohibits oral intubation and access for successful dental treatment. The simple procedure of climbing on to an operating room table and positioning of the patient can cause frictional trauma. This can be lessened with the use of sheepskin under the sheets. Oral ulceration caused by trauma during dental restoration is unavoidable but can be controlled. Ample lubrication of all intraoral instruments with hydrocortisone cream will limit friction and help control sloughing. Broad shaped retraction instruments will spread and decrease the force

Fig 3. lntraoral sloughing secondary to manipulation.

Special Care in Dentistry, Vol 11 No 4 1991 149

needed on the mucosal tissue. Combining esophageal dilation with dental rehabilitation limited the number of times the patient was placed under general anesthesia. As scarring is a long-term complication of recessive dystrophic epidermolysis bullosa, limiting the number of traumatic procedures improves the patient's quality of life. In addition, the steroidal therapy used to treat the esophageal strictures and manage the esophageal dilation aided the postoperative intraoral healing of the dental procedures. Perhaps the most significant contribution made to the patient is a 4month postoperative recall appointment. These patients are at an increased risk for plaque accumulation, periodontal complications, and caries formation for a variety of reasons-most obvious of which is the pain of the ulcerated oral tissues, discouraging the patient from brushing and flossing thoroughly. In these situations, oral hygiene should be accomplished with the parents aid to ensure optimal oral health. In addition, as the patient ages, the flexion contracture and digital scarring increases, thereby decreasing the needed manual dexterity for adequate plaque removal. The patient's liquified diet also affects plaque retention since the mechanical debridement of chewing food has been eliminated. Intraoral ulcerations will always be present, but plaque removal must be accomplished to maintain gingival health and prevent secondary infection. A promising therapeutic regimen, to decrease plaque accumulation and bacterial activity, is the use of 0.12% chlorhexidine mouthrinse. It's plaque inhibitory activity is the result of antimicrobial activity and ability to adsorb to the surfaces of the oral cavity.l0," This antimicrobial activity has been proven against numerous potentially pathogenic oral organisms.'O Parameters such as dental plaque levels, gingivitis, microbial levels, and mucositis decrease with its use in medically compromised patients. 11,12 Although not recommended by the FDA for use in children, studies have shown it can be used in a medically compromised pe-

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diatric population without significant detrimental side effects.13 Another way to decrease microbial activity is the well documented use of fl~0ride.I~ Daily use of a fluoridated toothpaste, daily rinses with a fluoride mouthrinse (.05% NaF), and triannual topical fluoride (1,23%APF) applications, administered by a dentist, all have additive effects in regard to antimicrobial activity. Enamel remineralization, lower free surface energy, and increased mineralization in a developing dentition are proven anticariogenic benefits of f l ~ 0 r i d e .A l ~custom fitted fluoride tray for daily fluoride (.4% SnF) applications can be considered. Although trauma induced by the impression necessary for tray construction, as well as the changing tooth relationship in a developing dentition, may contraindicate this treatment modality. Sealant placement, properly timed orthodontic extractions, and full mouth periodontal scaling are treatment needs that may be more efficacious on a passive and well-controlled anesthetized patient.

are those of the authors. They do not purport to express views of the Department of the Air Force or any other Department or Agency of the United States Government. To the best of the authors' knowledge, the above work contains no material whose publication would violate any copyright or other proprietary rights of any person. The work in its present form has not been published previously elsewhere by the authors. Dr. Camm is chief, Pediatric Dentistry; Dr. Gray is chief, Restorative Dentistry Section, Department of General Dentistry; Dr. Mayes is chief, Pediatric Intensive Care, Wilford Hall USAF Medical Center, Lackland AFB, TX 78236-5300. Address requests for reprints to Dr. Camm. 1. Stedman's medical dictionary. 23rd ed. Baltimore: Williams & Wilkins; 1976. 2. Behrman RE, Vaughan VC 111. Nelson

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Summary A patient with a debilitating disease, such-asepidermolysis bullosa, is a challenge to the dentist wishing to provide optimal and comprehensive dental treatment. While tissue sloughing and bullae formation during treatment cannot be eliminated, the previously outlined protocol can limit the severity of it. Medical assessment of these patients is mandatory to determine their overall health status. If indicated, a combination medical and dental procedure can be performed under general anesthesia, thereby limiting the number of stressful operations a patient may have to undergo. Triannual dental examinations and continual re-evaluation are necessary to promote continued dental health and recognition of early pathosis. Aggressive preventive measures are necessary to ensure the patient's future dental needs are minimal. The authors are officers of the United States Air Force at Lackland AFB, TX. Opinions expressed therein, unless otherwise specifically indicated,

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textbook of pediatrics. 13th ed. Philadelphia: W.B. Saunders; 1987:1400-2. Jarratt M. Diagnosis and treatment of epidermolysis bullosa. South Med J 1976;69:113-7. Heddie OS, Gorlin RJ. Epidermolysis bullosa. Oral Surg Oral Med Oral Pathol 1989;67555-63. Zitelli BJ, Davis HW. Atlas of pediatric physical diagnosis. St. Louis: C.V. Mosby; 1987;8.6-8.7. Wright JT, Gantt DG. Epidermolysis bullosa. J Oral Pathol 1983;1273-83. Kempe CH, Silver HK, OBrien D, Fulginiti VA. Current pediatric diagnosis and treatment. Norwalk, C T Appleton and Lange; 1987259-60. Wright JT. Epidermolysis bullosa: dental and anesthetic management of two cases. Oral Surg Oral Med Oral Pathol 1984;57155-7. Wright IT. Comprehensive dental care and general anesthetic management of hereditary epidermolysis bullosa. Oral Surg Oral Med Oral Pathol1990;70:573-8. Baker PJ, Coburn RA, Genco RJ, and Evans RT. Structural determinants of activity of chlorhexidine and alkyl bisbiguanides against the human oral flora. J Dent Res 1987;66(6):1099-106. Ferretti GA, Ash RC,Brown AT, Largent BM, Kaplan A, Lillich TT. Chlorhexidine for prophylaxis against oral infections and associated complications on patients receiving bone marrow transplants. JADA 1987114,461-7. McGaw WT, Belch A. Oral complications of acute leukemia: prophylactic impact of a chlorhexidine mouth rinse regimen. Oral Surg Oral Med Oral Pathol. 1985;60:275-80. Raether D, Walker PO, Bostrum B, Weisdorf D. Effectiveness of oral chlorhexidine for reducing stomatitis in a pediatric bone marrow transplant population. Pediatr Dent 1989;11 :37-42. Harris NO, Christen AG. Primary preventive dentistry. 2nd ed. Norwalk, CT: Appleton & Lange; 1987198-9,268-9.

Combined medical-dental treatment of an epidermolysis bullosa patient.

Epidermolysis bullosa presents a wide range of clinical symptoms. In this case, a patient had recessive dystrophic epidermolysis bullosa that required...
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