Hereditary epidermolysis bullosa:oral manifestations and dental management

Dan Boboia 3/5/10 Lit. Review

Title: Hereditary epidermolysis bullosa:oral manifestations and dental management
Author: Wright et al
Type of Article: Review

Summary:
Epidermolysis (EB) feature blister formation with tissue separation occuring at variable depths in the skin and under the mucosa (depending on the specific EB type). Oral involvement can occur. Although oral tissue fragility and blistering is common in all EB types, oral debilitation secondary to scarring is primarily limited to the recessive dystrophic EB subtypes. With junctional EB you often see generalized enamel hypoplasia. Rampant caries is associated with generalized recessive dystrophic EB. Intervention and prevention strategies targeting the dental effects of EB is often helpful in reducing the negative oral consequences associated with this disease. Patients with mild forms of EB are treated the same as those without the disorder. When using local anesthesia, injections should be deep and slow in order to prevent tissue distortion (which may cause mechanical tissue separation and blistering-nerve blocks are preferred). Apply only compressive forces rather then traction or other shear forces to oral tissues, these could induce tissue separation. Make certain to lubricate patient lips. Care must be taken with general anesthesia as to not induce airway obstruction secondary to blister formation. Generalized enamel hypoplasia, characteristic of junctional EB, is best managed with stainless steel crowns. Most individuals with EB can tolerate orthodontic therapy with minor design modifications to reduce soft tissue irritation. Small dental arches and collapsed dental arches do predispose these patients to significant crowding. Serial extractions can often benefit these patients. Fluoride rinses, Peridex rinses, soft-bristled toothbrushes with small heads may all help with caries control.

Assessment: Good review of oral management in these patients