Hereditary epidermolysis bullosa. Dental management of three cases.
Hereditary epidermolysis bullosa (EB) is a mucocutaneous disorder characterized by the appearance of blisters and vesicles in response to minimum friction. The digestive mucosa is one of the most frequently affected regions--including the oral mucosa. Three types of EB have been established according to the histological level of the lesion. Thus, simple EB involves intraepidermal bullae that leave no scars, while junctional EB exhibit blisters between the lamina lucida and lamina densa of the basal membrane. These lesions heal leaving atrophy and involve important hypoplastic lesions in the dental enamel. In turn, dystrophic EB presents synechiae-forming subepidermal blisters--the recessive form being the variant involving the greatest oral lesions (microstomia, ankyloglossia, milium cysts and rampant caries). Three cases of EB are presented and their clinical-dental management difficulties are described. The oral manifestations are described, along with the dental treatments provided and the evolution of the periodontal indices over a two-year period following the application of hygiene-preventive and therapeutic measures.
Serrano Martínez
C
C
Departamento de Estomatología de la Universidad de Valencia, España.
Silvestre Donat
F J
FJ
Bagán Sebastián
J V
JV
Peñarrocha Diago
M
M
Alió Sanz
J J
JJ
D000328:Adult; D002675:Child, Preschool; D004820:Epidermolysis Bullosa / Q000150:complications / Q000235:genetics*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D014076:Tooth Diseases / Q000209:etiology* / Q000628:therapy*