Epidermolysis bullosa

Overview

Board takeaway: Prevent friction and shear during every dental step; protect fragile tissues and use an experienced airway team when general anesthesia is needed.

Inherited fragility of skin and mucosa: friction causes blisters. Scarring causes microstomia, and every touch in the mouth must be gentle and lubricated.

How does Epidermolysis bullosa affect dental management?

• Prevent friction: lubricate lips and contact surfaces, press rather than slide, and avoid adhesive tape on fragile skin.
• Oxygen and anesthesia: When oxygen or anesthesia is used, select compatible dressings/lubricants with the anesthesia team; do not apply petrolatum indiscriminately around an oxygen source.
• Local anesthesia: Inject local anesthetic slowly and sufficiently deep to avoid mechanically separating superficial mucosal layers.
• Injection route: Select the injection route from the procedure and tissue condition; the specialist guideline does not require routine intraligamental injection
• Limited access: microstomia and vestibular scarring can restrict access. Keep suction tips padded or off the mucosa; use small-head soft brushes.
• Prevention and restoration: Intensive prevention: fluoride, chlorhexidine, diet counseling; stainless steel crowns over extensive restoration
• GA: GA with a team experienced in EB (airway blistering, difficult intubation)

Exam pearl

What is Epidermolysis bullosa?

A group of inherited disorders that make the skin and, in some types, mucous membranes fragile, causing blistering after minor friction or trauma.

What are the main systemic features of Epidermolysis bullosa?

• Forms and severity: Major forms include simplex (often keratin-related), junctional, dystrophic (type VII collagen-related), and Kindler EB. Severity varies within each group; simplex is not invariably mild
• Trauma and scarring: Blisters from minor friction; scarring, mitten hand deformities, esophageal strictures
• Systemic risks: Malnutrition, anemia; recessive dystrophic EB carries a high risk of squamous cell carcinoma in young adults

What oral findings occur in Epidermolysis bullosa?

• Dystrophic disease: Oral bullae and ulceration; vestibular obliteration, ankyloglossia, microstomia (dystrophic)
• Junctional disease: Generalized enamel hypoplasia and pitting (junctional)
• Caries: High caries risk: soft high-calorie diet, painful brushing

For Epidermolysis bullosa, what long-term risk requires monitoring in recessive dystrophic disease?

• Aggressive squamous cell carcinoma, including oral lesions, in adolescents/young adults
• An ulcer that is persistent, changing, unusually painful, indurated, or otherwise unlike the patient’s usual lesions needs prompt specialist assessment and biopsy when suspicious; do not dismiss it as routine EB blistering

For Epidermolysis bullosa, which EB type has enamel defects?

Junctional EB (laminin-332 and others): generalized enamel hypoplasia and pitting with high caries risk. Dystrophic EB causes the scarring and microstomia.

Sources

• Supplemental study guide: Pediatric Dental Study Guide: Epidermolysis bullosa
• Management of Dental Patients with Special Health Care Needs
• Supplemental clinical source: DEBRA-supported clinical practice guidelines: Oral health care in EB (2020), Chapters 3–5