[Syndromes 13. Epidermolysis bullosa].
Epidermolysis Bullosa is characterised by blister formation of skin and mucous membranes. Three major varieties of epidermolysis bullosa (EB) are reviewed including their dental and oral aspects: EB simplex, junctional EB and dystrophic EB. Marked oral involvement of the soft and hard tissues can produce potentially devastating alterations in anatomy and function. Oral debilitation is limited primarily to the recessive dystrophic EB type due to soft tissue scarring following blister formation. Microstomia, ankyloglossia and obliteration of the oral vestibule are typical features of dystrophic EB. In other cases enamel hypoplasia and cementum disorders can be present. Epidermolysis bullosa has considerable impact on oral health and dental care.
Meijndert
L
L
Afdeling Mondziekten, Kaakchirurgie en Bijzondere Tandheelkunde, Academisch Ziekenhuis te Groningen.
Jonkman
M F
MF
D001768:Blister; D003744:Dental Enamel Hypoplasia / Q000209:etiology; D004820:Epidermolysis Bullosa / Q000145:classification / Q000473:pathology; D016108:Epidermolysis Bullosa Dystrophica / Q000145:classification / Q000473:pathology; D016110:Epidermolysis Bullosa Simplex / Q000145:classification / Q000473:pathology; D016109:Epidermolysis Bullosa, Junctional / Q000145:classification / Q000473:pathology; D006801:Humans; D008865:Microstomia / Q000209:etiology; D009059:Mouth Diseases / Q000209:etiology; D009092:Mucous Membrane / Q000473:pathology; D012867:Skin / Q000473:pathology
Syndromen 13. Epidermolysis bullosa.