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Oral and Dental Changes Associated with Hereditary Epidermolysis Bullosa

Description

A patient with hereditary epidermolysis bullosa (EB) experiences increased fragility of skin and other tissues. A number of problems may affect the teeth and oral mucosa of these patients. Oral and dental changes may be found in all forms of epidermolysis bullosa but are most often seen in generalized juvenile EB (JEB) and recessive-dystrophic epidermolysis bullosa of the Hallopeau-Siemens subtype (RDEB-HS).


 

Patient selection: hereditary epidermolysis bullosa

 

Changes in the non-dental oral structures:

(1) blisters and erosions of the oral mucosa

(2) scarring of intra-oral soft tissues

(3) ankyloglossia (especially in RDEB-HS)

(4) microstomia (especially in RDEB-HS)

 

Dental changes (especially in generalized juvenile epidermolysis bullosa):

(1) hypoplasia and pitting of the enamel

(2) severe and extensive secondary caries

(3) premature tooth loss

 

Long-term complications may include:

(1) prolonged clearance of food

(2) malnutrition

 

Early and aggressive oral and dental management can preserve function and delay complications.

 


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