Oral Health Care for Patients with Epidermolysis Bullosa - Best Clinical Practice Guidelines

Department of Paediatric Dentistry, Facultad de Odontología, Universidad de Chile, Santiago, Chile.
International Journal of Paediatric Dentistry (Impact Factor: 1.34). 09/2012; 22 Suppl 1:1-35. DOI: 10.1111/j.1365-263X.2012.01247.x
Source: PubMed


  To provide the users with information on the current best practices for managing the oral health care of people living with EB.
  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.
  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.
  A preventive protocol is today's dental management approach of choice.

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    • "They comprise 17 items centered on wound care and related general health and patient issues. In addition, comprehensive best practice guidelines addressing all aspects of oral health care in EB have been published [9]. Finally, guidelines for wound and skin care in the major EB types have been generated by clinical experts and made available on the Debra International website [10]. "
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    ABSTRACT: Background Inherited epidermolysis bullosa (EB) comprises a highly heterogeneous group of rare diseases characterized by fragility and blistering of skin and mucous membranes. Clinical features combined with immunofluorescence antigen mapping and/or electron microscopy examination of a skin biopsy allow to define the EB type and subtype. Molecular diagnosis is nowadays feasible in all EB subtypes and required for prenatal diagnosis. The extent of skin and mucosal lesions varies greatly depending on EB subtype and patient age. In the more severe EB subtypes lifelong generalized blistering, chronic ulcerations and scarring sequelae lead to multiorgan involvement, major morbidity and life-threatening complications. In the absence of a cure, patient management remains based on preventive measures, together with symptomatic treatment of cutaneous and extracutaneous manifestations and complications. The rarity and complexity of EB challenge its appropriate care. Thus, the aim of the present study has been to generate multicentre, multidisciplinary recommendations on global skin care addressed to physicians, nurses and other health professionals dealing with EB, both in centres of expertise and primary care setting. Methods Almost no controlled trials for EB treatment have been performed to date. For this reason, recommendations were prepared by a multidisciplinary team of experts from different European EB centres based on available literature and expert opinion. They have been subsequently revised by a panel of external experts, using an online-modified Delphi method to generate consensus. Results Recommendations are reported according to the age of the patients. The major topics treated comprise the multidisciplinary approach to EB patients, global skin care including wound care, management of itching and pain, and early diagnosis of squamous cell carcinoma. Aspects of therapeutic patient education, care of disease burden and continuity of care are also developed. Conclusion The recommendations are expected to be useful for daily global care of EB patients, in particular in the community setting. An optimal management of patients is also a prerequisite to allow them to benefit from the specific molecular and cell-based treatments currently under development.
    Orphanet Journal of Rare Diseases 05/2014; 9(1):76. DOI:10.1186/1750-1172-9-76 · 3.36 Impact Factor
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    ABSTRACT: AimTo find the prevalence of molar-incisor hypomineralization (MIH) in a random sample of Spanish children, and to investigate the gender influence, distribution of defects, the treatment need associated and the relation between this disorder and dental caries. DesignA cross-sectional study was carried out to determine MIH and caries prevalence in a randomly selected sample of 840 children from the 8-year-old population of the Valencia region of Spain. The examinations were carried out in the children's schools by one examiner who had previously been calibrated with the MIH diagnostic criteria of the European Academy of Paediatric Dentistry (EAPD). ResultsThe percentage of children with MIH was 21.8% (95% CI 19.1-24.7), with a mean 3.5 teeth affected (2.4 molars and 1.1 incisors) been the maxillary molars the most affected. No gender differences were found. Of those with MIH, 56.8% presented lesions in both molars and incisors Children with MIH needed significantly more urgent and non-urgent treatment than those without MIH (chi-squared test P-value<0.005). Both caries indices were significantly higher (Student's t-test P-value <0.05) in the children with MIH than in the healthy children: the DMFT scores were 0.513 and 0.237 and the DMFS scores 1.20 and 0.79, respectively. Conclusions Molar-incisor hypomineralization prevalence is high in the child population of this region and equally affects boys and girls. The condition increases significantly the need of treatment of affected children. A significant association with dental caries was observed.
    International Journal of Paediatric Dentistry 01/2013; 24(1). DOI:10.1111/ipd.12020 · 1.34 Impact Factor
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    ABSTRACT: Children with epidermolysis bullosa can present with disease(s) of the ears, nose, and throat, often related directly to the pathophysiology of their epidermolysis bullosa. Otolaryngologic diseases in children with epidermolysis bullosa have to be managed having a proper understanding of the diagnosis and pathophysiology of epidermolysis bullosa. The purpose of this review is to describe the current nomenclature and diagnostic algorithms for epidermolysis bullosa, and methods for the management of cutaneous and mucosal lesions. Characterization of the gene defects leading to epidermolysis bullosa has allowed the utilization of immunofluorescent techniques as the primary method for epidermolysis bullosa diagnosis. Recognizing the difficulty in managing patients with epidermolysis bullosa, several multidisciplinary groups have developed guidelines using meta-analysis of the published literature, or expert panels. Though there are currently no effective treatment modalities for epidermolysis bullosa, techniques for gene and protein replacement show promising results for future use. Currently, the management of cutaneous and mucosal disease in epidermolysis bullosa is based on the principles of prevention and wound care. Understanding the cause of epidermolysis bullosa types and subtypes, characteristics of skin and mucosal involvement, and prognosis will guide in the development of individualized treatment plans.
    Current opinion in otolaryngology & head and neck surgery 10/2013; 21(6). DOI:10.1097/MOO.0000000000000004 · 1.84 Impact Factor
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