Search

Objective:   To provide the users with information on the current best practices for managing the oral health care of people living with EB., Methods:   A systematic literature search, in which the main topic is dental care in patients with Epidermolysis Bullosa, was performed. Consulted sources, ranging from 1970 to 2010, included MEDLINE, EMBASE, CINAHL, The Cochrane Library, DARE, and the Cochrane controlled trials register (CENTRAL). In order to formulate the recommendations of the selected studies the SIGN system was used. The first draft was analysed and discussed by clinical experts, methodologists and patients representatives on a two days consensus meeting. The resulting document went through an external review process by a panel of experts, other health care professionals, patient representatives and lay reviewers. The final document was piloted in three different centres in United Kingdom, Czech Republic and Argentina., Results:   The guideline is composed of 93 recommendations divided into 3 main areas: 1) Oral Care--access issues, early referral, preventative strategies, management of microstomia, prescriptions and review appointments 2) Dental treatment: general treatment modifications, radiographs, restorations, endodontics, oral rehabilitation, periodontal treatment, oral surgery and orthodontics, and 3) Anaesthetic management of dental treatment., Conclusions:   A preventive protocol is today's dental management approach of choice., (© 2012 The Authors. International Journal of Paediatric Dentistry © 2012 BSPD, IAPD and Blackwell Publishing Ltd.)

Epidermolysis bullosa (EB) is a genetically inherited condition that can lead to significant disability, but specialist nurses can help.

Basic principles of present-day classification of hereditary epidermolysis bullosa are outlined, and morphologic grounds for distinguishing common, borderline, and dystrophic e. bullosa are validated. Potentialities of prenatal diagnosis of grave forms of e. bullosa as one of the major means of prevention are discussed. Data of clinical follow-up of 25 patients with common, 5 with borderline, and 29 ones with dystrophic e. bullosa are presented, as are results of 8 cases of prenatal diagnosis. The authors emphasize the necessity of electron microscopic examination of the skin in every case.