A Rare Case Epidermolysis Bullosa in a Child Patient with Amelogenesis Imperfecta
WEST INDIAN MEDICAL JOURNAL, vol.1, pp.124-127, 2016 (SCI-Expanded)
- Publication Type: Article / Article
- Volume: 1
- Publication Date: 2016
- Doi Number: 10.7727/wimj.2015.339
- Journal Name: WEST INDIAN MEDICAL JOURNAL
- Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Page Numbers: pp.124-127
- Akdeniz University Affiliated: Yes
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.