Epidermolisis bulosa

Oral Manifestations and Management of Epidermolysis Bullosa

Tim Wright, DDS, MS Department of Pediatric Dentistry The University of North Carolina

Oral manifestations vary markedly dependingon 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 MicrostomiaUlceration 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 makeproviding oral health care extremely challenging.
CONT 0 EBS JEB DDEB RDEB 0 20 40 60 80 72 95 100 39 81

percent oral lesions

Oral Cavity Width in Different EB Types
Control
Simplex

45 4346 47 39 47 34
41 0 10 20 30 40 50

General Local Non-Herlitz

Junctional

Herlitz Dominant

Dystrophic General Res

Local Rec

Commisure-Commisure Width (mm)

Oral Soft TissueScreening
• 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 rolledborders and increasing in size. • Professional oral examination every 6 months

Oral Soft Tissue Screening

Managing Discrete Soft Tissue Lesions
Cyanoacrylate based topical covering that providesrelief 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...