Hereditary epidermolysis bullosa. Dental management of three cases. 2001

C Serrano Martínez, and F J Silvestre Donat, and J V Bagán Sebastián, and M Peñarrocha Diago, and J J Alió Sanz
Departamento de Estomatología de la Universidad de Valencia, España.

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.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004820 Epidermolysis Bullosa Group of genetically determined disorders characterized by the blistering of skin and mucosae. There are four major forms: acquired, simple, junctional, and dystrophic. Each of the latter three has several varieties. Acantholysis Bullosa
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D014076 Tooth Diseases Diseases involving the TEETH. Disease, Tooth,Diseases, Tooth,Tooth Disease

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