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Related Experiment Videos

Hereditary epidermolysis bullosa

J T Wright1, J D Fine

  • 1Department of Pediatric Dentistry, School of Dentistry, University of North Carolina at Chapel Hill 27599.

Seminars in Dermatology
|June 1, 1994
PubMed
Summary

Epidermolysis bullosa (EB) causes blistering and oral tissue fragility. Dental interventions can prevent tooth loss in EB patients, even those with severe cases.

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Area of Science:

  • Dermatology
  • Oral Medicine
  • Genetics

Background:

  • Epidermolysis bullosa (EB) encompasses genetic disorders characterized by skin and mucosal blistering.
  • Oral manifestations are common in all EB types, ranging from tissue fragility to severe scarring.
  • Specific EB subtypes, like generalized recessive dystrophic EB and junctional EB, present distinct oral and dental challenges.

Purpose of the Study:

  • To review the oral and dental implications of Epidermolysis Bullosa.
  • To highlight the dental management strategies for patients with EB.
  • To emphasize the importance of preserving dentition in severely affected individuals.

Main Methods:

  • Literature review of Epidermolysis Bullosa and its oral manifestations.
  • Analysis of dental challenges associated with different EB subtypes.
  • Evaluation of dental treatment modalities for EB patients.

Main Results:

  • All EB types exhibit oral tissue fragility and blistering.
  • Severe oral debilitation and microstomia are noted in recessive dystrophic EB.
  • Enamel hypoplasia and rampant dental caries are prevalent in junctional and recessive dystrophic EB.

Conclusions:

  • Aggressive dental interventions are crucial for maintaining oral health in EB patients.
  • Modern dental restorative techniques, including general anesthesia, can preserve dentition.
  • Preventive dental therapy is essential to avoid tooth destruction and loss in individuals with EB.

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