No abstract

Epidermolysis bullosa (EB) is an inherited disorder affecting the skin and mucous membranes, characterized by blister formation following minor trauma. It is a chronic mechanobullous disease related to the specific abnormal or absent proteins. The disease is associated with conspicuous clinical and oral manifestations. The oral involvement of EB includes generalized enamel hypoplasia, dental caries, limited mouth opening, ankyloglossia, microstomia and obliteration of the vestibule. Amelogenesis imperfecta (AI) is a hereditary disorder with dental enamel defects and enamel hypoplasia both in deciduous and permanent dentition. There is very limited information in the literature, which indicate the presence of EB together with AI. The aim of this report is to present the clinical and radiographic manifestations and dental management of EB simplex in a child patient with hypoplastic form of AI.