Case report: Dystrophic Epidermolysis Bullosa: dental management and oral health promotion.

BACKGROUND

Epidermolysis Bullosa (EB) is a group of rare genetically determined disorders characterised by the development of blisters following minor or insignificant trauma or traction to the skin or mucosal surfaces. It appears that the cause of EB is related to abnormal enzyme activity and collagen degradation, but the exact pathogenesis and aetiology remain obscure. There are three major forms -- simple, junctional, and dystrophic. Oral manifestations and dental involvement of EB vary in frequency, and severity according to the form.

CASE REPORT

A 13-year old boy with a known diagnosis of Dystrophic Epidermolysis Bullosa (DEB) since birth was seen in our dental practice in 2004. Upon examination, the patient had multiple scars and blisters involving his neck, head, elbows and knees. Pseudosyndactyly was present on both hands. Intraoral examination revealed multiple carious lesions affecting almost the entire dentition, with severe gingival inflammation, mycrostomia, ankyloglossia and elimination of buccal and vestibular sulci.

TREATMENT

This was divided into four phases- (1) oral hygiene and dietary instructions, elimination of gingival inflammation, (2) restorative procedures, (3) extractions of the carious destroyed teeth, and (4) recall system.

FOLLOW-UP: The patient was recalled on a schedule every three months with continued evaluation of oral status (Pl and GI indices). The preventive regimen included twice yearly topical fluoride application (Duraphat 2.26%). The final recommendations for our patient (except brushing twice a day), were to use daily fluoride mouthrinse (NaF 0.05%), to avoid cariogenic food products, and to visit a dietician to correct his diet.

CONCLUSION

By applying present knowledge of DEB the oral health of these patients can be improved and eliminate complications arising from the oral condition. After three routine check-ups and due to the cooperation of this patient, his oral health status remains very satisfactory.