Oral manifestations vary markedly depending on the specific EB type, the molecular defect, site of tissue separation and other factors. Simplex Keratin genes KRT5, KRT14 Junctional Laminin genes LAMA3, LAMB3, LAMC2 Dystrophic Type VII Collagen COL7A1 (over 600 mutations)
Oral Soft Tissue Manifestations of EB Highly variable depending on EB type Increased fragility Perioral lesions Microstomia Ulceration of mucosa, lips, checks, tongue, palate Ankyloglosia Obliteration of oral vestibule
EB Associated Soft Tissue Manifestations Can markedly affect ability to care for teeth. Can make providing oral health care extremely challenging. CONT 0 EBS 39 JEB 81 DDEB 72 RDEB 95 0 20 40 60 80 100 percent oral lesions
Oral Cavity Width in Different EB Types Control 45 Simplex General Local 43 46 Junctional Non-Herlitz Herlitz 39 47 Dominant 47 Dystrophic General Res 34 Local Rec 41 0 10 20 30 40 50 Commisure-Commisure Width (mm)
Oral Soft Tissue Screening Individuals with Recessive Dystrophic EB are at increased risk for oral carcinoma. Self examination monitor oral lesions for marked changes, especially lesions developing hard or rolled borders and increasing in size. Professional oral examination every 6 months
Oral Soft Tissue Screening
Managing Discrete Soft Tissue Lesions Cyanoacrylate based topical covering that provides relief from sensitive oral ulcers.
Mouthwash for Stomatitis Treat stomatitis palliatively in the absence of infection Magic Mouthwash Maalox (100ml) Viscous Xylocaine (25-50ml) Benadryl (25-50ml)
Oral Candidiasis Individuals with chronic oral ulcerations (e.g. RDEB, Dowling Mera Simplex) are at increased risk for oral candida infections (thrush). Typically treated with oral nystatin or ketoconazol. Treatment with chlorhexidine rinse can reduce candida overgrowth.
Dental Problems and EB Junctional EB and occasionally other EB types will have severe enamel hypoplasia. Individuals with junctional and recessive dystrophic EB are at increased risk for dental caries. Individuals with recessive dystrophic EB frequently have severe dental crowding and a deep bite.
Enamel Hypoplasia in Different EB Types Control 10 Simplex Junctional General Local Non-Herlitz Herlitz Dominant 11 30 35 100 100 Dystrophic General Res Local Rec 6 41 0 20 40 60 80 100 Percent
Junctional Epidermolysis Bullosa Laminin defect affecting skin and tooth formation.
Factors Contributing to Increased Dental Caries Risk in EB Enamel hypoplasia Consumption of high calorie (carbohydrate) diet Slow eaters and increased feeding frequency Reduced oral clearance of food due to soft tissue strictures Inability to effectively brush teeth Difficulty receiving routine preventive dental care.
Prevalence of Dental Caries in EB Population 60 58.6 50 40 30 20 10 37.6 21.6 25.6 23.2 Rec Dys Dom Dys Junctional Simplex Control 0 DMFS
Caries Prevention Diet Control Minimize frequency of exposure to refined carbohydrates Mechanical plaque/substrate control Brushing, flossing, rinsing Chemotherapeutic control Fluoride, chlorhexidine Protective covering of teeth Protective covering of teeth Sealants, crowns
Toothbrush Selection Individuals with microstomia or severe fragility of the oral mucosa and gingiva. Select small headed brush Select soft bristles Run brush under hot water to further soften bristles Consider mechanical brush with small head and soft bristles.
RotoDent Pro-Dentec PO Box 3889 Batesville, AR 72503 800-228-5595 Mechanical Toothbrush
Chemotherapeutic Caries Control Fluoride therapy Systemic H 2 O, Supplements Topical - Toothpaste, Mouthwash Professional Application Gel, Varnish Antimicrobial Agents Chlorhexidine
Fluoride Varnish Placement
Fluoride Concentration of Rx Toothpastes Control RX, Prevident (NaF) = 0.5% F 5000ppm 5X stronger than regular toothpaste Rx only
Effective antimicrobial rinse Reduces candidiasis Reduces dental caries Chlorhexidine Rinse
Dental Restorations for EB Patients If extensive soft tissue and dental caries problems then - Use techniques and materials that cover the teeth. Stainless steel crowns Resin bonded stainless steel crowns Porcelain fused to metal crowns Ceramic or new tooth colored crown materials
Crowns for Primary and Early Permanent Teeth Junctional EB
Complete Restoration Young Permanent Dentition Junctional EB
Full Mouth Porcelain/Metal Crowns Junctional EB
Dental Implants in EB Implants in patient with RDEB- Inversa
Dental Implant Considerations Oral opening access Bone available to receive implant Soft tissue health Ability to maintain implants Oral hygiene
Dental Implants in RDEB Patient
Dental Malocclusion is Prevalent in RDEB Due to Soft Tissue Constriction and Crowding Can allow better alignment of incisors by selectively extracting primary canines and permanent first premolars.
Providing Dental Treatment In mildly affected individuals dental treatment can normally be provided with minor modifications to approach. Severely affected individuals will often require treatment with the aid of general anesthesia.
Indications for General Anesthesia to Provide Treatment in EB Patients Extensive soft tissue fragility Oral blistering and scarring Limited oral access due to microstomia Rampant dental caries Extensive restorative or surgical Tx required Invasive procedures Implant placement Soft tissue surgery
General Anesthesia and EB Allows comprehensive treatment of dental caries. Following special protocol greatly reduces soft tissue damage from procedure. Safety record based on literature of hundreds of cases using endotracheal intubation is excellent.
General Anesthesia and EB Use endotracheal intubation Use atraumatic techniques Lubricate instruments and tissues to prevent shear forces No tape or adhesives
Treatment and Vital Sign Monitoring Without Adhesives Pulse oximetry monitor Block adhesive on probe Clip probe EKG leads Use non-adhesive leads Cut adhesive off leads Use patient s weight to make contact
Dental Management of EB Early intervention and dental consult Regular evaluation of soft tissues for premalignant alterations Aggressive prevention approaches Regular recall appointments Restorative therapy provided before advanced destruction of the teeth
Current preventive and restorative therapies allow individuals with EB that have substantial oral involvement to maintain an esthetic and functional dentition: