Dental and anaesthetic management of children with dystrophic epidermolysis bullosa.
BACKGROUND
Epidermolysis bullosa (EB) is a genetic disease associated with fragility and bullous lesions of the skin and mucous membranes. There are various patterns of inheritance and histopathology. The disease is associated with systemic and oral manifestations, among which may be dental decay necessitating oral rehabilitation. General anaesthesia is frequently required for oral rehabilitation in children with dystrophic EB. Paediatric dentists should be aware of the implications of dystrophic EB for dental and anaesthetic management.


METHODS
Two siblings with moderate to severe systemic and oral manifestations of dystrophic EB requiring extensive oral rehabilitation for rampant decay are described, including special anaesthetic techniques required for airway management and maintenance of skin integrity.


CONCLUSIONS
Dystrophic EB is a rare genetic disorder in which vesiculobullous lesions occur with erosions and scarring of cutaneous and extracutaneous surfaces in the oral cavity. Poor oral hygiene results from efforts to avoid mucosal trauma, resulting in decay. The comprehensive care of children with dystrophic EB impacts not only dental management but anaesthesia administration as well.
Lindemeyer
Rochelle
R
Department of Preventive and Restorative Sciences, Division of Pediatric Dentistry, School of Dental Medicine, University of Pennsylvania, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. lindemeyer@email.chop.edu
Wadenya
Rose
R
Maxwell
Lynne
L
D000766:Anesthesia, Dental / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D019140:Dental Care for Children / Q000379:methods*; D017742:Dental Care for Chronically Ill / Q000379:methods*; D003793:Dental Restoration, Permanent / Q000379:methods; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications* / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D009059:Mouth Diseases / Q000150:complications / Q000628:therapy*; D014076:Tooth Diseases / Q000150:complications / Q000628:therapy