CLINICAL MANAGEMENT FOR EPIDERMOLYSIS BULLOSA DYSTROPHICA

Epidermolysis bullosa (EB) consists of a group of genetic hereditary disorders in which patients frequently present fragile skin and mucosa that form blisters following minor trauma. More than 20 subtypes of EB have been recognized in the literature. Specific genetic mutations are well characterized for most the different EB subtypes and variants. The most common oral manifestations of EB are painful blisters affecting all the oral surfaces. Dental treatment for patients with EB consists of palliative therapy for its oral manifestations along with typical restorative and periodontal procedures. The aim of this article is to describe two dental clinical treatments of recessive dystrophic EB cases and their specific clinical manifestations. The psychological intervention required during the dental treatment of these patients is also presented.     

Syndromes 13. Epidermolysis bullosa

Epidermolysis Bullosa is characterised by blister formation of skin and mucous membranes. Three major varieties of epidermolysis bullosa (EB) are reviewed including their dental and oral aspects: EB simplex, junctional EB and dystrophic EB. Marked oral involvement of the soft and hard tissues can produce potentially devastating alterations in anatomy and function. Oral debilitation is limited primarily to the recessive dystrophic EB type due to soft tissue scarring following blister formation. Microstomia, ankyloglossia and obliteration of the oral vestibule are typical features of dystrophic EB. In other cases enamel hypoplasia and cementum disorders can be present. Epidermolysis bullosa has considerable impact on oral health and dental care.     

Dental and anaesthetic management of children with dystrophic epidermolysis bullosa

Background.  Epidermolysis bullosa (EB) is a genetic disease associated with fragility and bullous lesions of the skin and mucous membranes. There are various patterns of inheritance and histopathology. The disease is associated with systemic and oral manifestations, among which may be dental decay necessitating oral rehabilitation. General anaesthesia is frequently required for oral rehabilitation in children with dystrophic EB. Paediatric dentists should be aware of the implications of dystrophic EB for dental and anaesthetic management.
Case report.  Two siblings with moderate to severe systemic and oral manifestations of dystrophic EB requiring extensive oral rehabilitation for rampant decay are described, including special anaesthetic techniques required for airway management and maintenance of skin integrity.
Conclusion.  Dystrophic EB is a rare genetic disorder in which vesiculobullous lesions occur with erosions and scarring of cutaneous and extracutaneous surfaces in the oral cavity. Poor oral hygiene results from efforts to avoid mucosal trauma, resulting in decay. The comprehensive care of children with dystrophic EB impacts not only dental management but anaesthesia administration as well.     

Sucralfate: a help during oral management in patients with epidermolysis bullosa

BACKGROUND: Epidermolysis bullosa (EB) is a group of genetic disorders that lead to blister formation at variable depths in skin and mucosa. Vesicles may arise spontaneously or be caused by friction or trauma. Oral tissue fragility and blistering is common in all EB types. The majority of patients with mild forms of EB are able to receive dental treatment. The prevention of dental caries is most challenging in subjects with severe mucosal involvement, as they are least able to routinely undergo correct preventive procedures. The aim of this study was to evaluate the effectiveness of a sucralfate suspension in reducing both pain and the number of blisters in patients with EB, and to obtain improved oral hygiene and a lower incidence of caries. METHODS: Five patients with dystrophic EB were treated with sucralfate suspension for the prevention and management of oral blisters. Oral blisters were assessed using a quantitative scale, while pain was assessed using visual analogue scale (VAS), and hygiene was evaluated through plaque and gingival indexes. RESULTS: The number of blisters, oral pain, and plaque decreased in all cases. CONCLUSIONS: Oral prophylaxis with sucralfate prevented oral blisters and oral discomfort. The procedure proved to be cost effective and easy to administer. It did not show significant side effects and may be used routinely in patients with EB.     

Oral-clinical findings and management of epidermolysis bullosa

Epidermolysis bullosa (EB) is a diverse group of disorders that have as a common feature blister formation with tissue occuring at variable depths in the skin and/or mucosa. This article reports two cases of EB and review oral-clinical findings of the EB types and approaches for managing the oral-clinical manifestations. While systemic treatment remains primarily palliative, it is possible to prevent destruction and subsequent loss of the dentition through appropriate interventions and dental therapy.     

Dental treatment in a patient with epidermolysis bullosa

Epidermolysis bullosa (EB) is a rare inherited group of genodermatoses characterized by mucocutaneous fragility and blister formation, either spontaneously or as a result of minimal mechanical trauma. The repetition of these episodes in the oral cavity leads to atrophy of the mucosa, causing microstomia, ankyloglossia, tongue denudation, and vestibule obliteration, characteristics that make dental treatment difficult. Patients with EB are at high risk for caries due to the presence of dental anomalies; they also tend to have a soft diet and difficulties with mechanical removal of the dental biofilm. This case report presents a patient diagnosed with EB and describes the difficulties faced by the clinician during dental treatment as well as the measures adopted to safely manage the patient's dental care.     

Inherited epidermolysis bullosa: an update and suggested dental care considerations

BackgroundEpidermolysis bullosa (EB) is a skin disease characterized by epithelial fragility that leads to blistering and erosion of the skin and mucosae. The authors conducted a literature review to provide an update on oral manifestations and dental care of patients with EB.Literature SearchThe authors reviewed the dental literature on EB in relation to clinical findings and provision of dental care. They searched textbooks and three databases: MEDLINE, Cochrane Library and Embase. The authors did not impose any date or publication status restrictions. They searched all databases up to August 2010.ResultsThe literature review revealed that four major groups and 32 subtypes of EB can be distinguished on the basis of the ultrastructural characteristics of skin cleavage, genetic mode of transmission and clinical phenotype. Oral manifestations differ in frequency and severity according to the disease subtype, but the most common are bullae, which leave painful ulcers on rupture, followed by scarring and tissue contraction. Although good oral health status is essential to maintaining oral function, dental treatment can induce new lesions and be hindered by the sequelae of existing lesions.Clinical ImplicationsDental treatment in patients with EB requires a multidisciplinary approach. Dental procedures must be minimally traumatic, and the effectiveness of treatment is determined mainly by the patient&;apos;s general health, cooperation in the dental office and at home, oral hygiene and diet.     

Clinical evaluation of patients with epidermolysis bullosa: Review of the literature and case reports

Epidermolysis bullosa (EB) is a relatively rare inherited disorder, which includes blister and vesicle formation on the skin and mucous membranes as a result of trauma or heat. There are different forms of this disorder. Mild manifestations are relatively uncomfortable, usually involving the knees, elbows, and fingers. Severe forms of this disease compromise normal functioning of multiple organs, which may result in premature death. The lack of a specific treatment to cure EB makes genetic counseling and prenatal diagnosis of primary importance to control this disorder. Three case histories of persons with dystrophic recessive epidermolysis bullosa are reported, focusing on appropriate dental care for patients with EB.     

Junctional epidermolysis bullosa: a case report

Epidermolysis bullosa (EB) is a group of genetically determined disorders characterized by blistering of the skin and mucosae. There are three major forms--simple, junctional and dystrophic--and each has several varieties. The present case report describes a male child with junctional EB. The aim of the report is to present the dietary situation and the dental status of the child, examples of potential dental and nutritional consequences, and the therapeutic interventions possible for children with this disease.     

Dental and anesthetic management of a child with epidermolysis bullosa

Epidermolysis bullosa (EB) is a wide spectrum of rare genetic disorders characterized by marked fragility of the skin and mucous membranes in which vesculobullous lesions occur in response to trauma, heat, or no apparent cause. This case report deals with the dental and anesthetic management of a 10-year-old girl with dystrophic EB.     

Dental care management in a child with recessive dystrophic epidermolysis bullosa

Epidermolysis bullosa (EB) is a heterogeneous group of rare genetic disorders characterized by marked fragility of the skin and mucous membranes in which vesiculobullous lesions occur in response to trauma, heat or no apparent cause. The recessive form of EB presents the greatest oral alterations including repeated blistering and scar formation leading to limited oral opening, ankyloglossia, tongue denudation, microstomia, vestibule obliteration and predisposition to oral carcinoma. Routine dental care may cause bullae formation on the lips and oral mucosa. Together with the ingestion of soft and frequently carbohydrate food, these anomalies lead to a high caries risk. This paper documents a case of a child diagnosed with recessive dystrophic epidermolysis bullosa (RDEB); describes the phases and difficulties of dental treatment and the measures that dentists and health care providers should adopt in order to provide a safe and effective dental treatment as well as earlier prevention to these patients.     

Conservative dental management of a patient with Epidermolysis bullosa. A case report

Epidermolysis bullosa (EB) is a vesiculobullous disorder characterized by skin and mucous membrane fragility. Intubation in general anesthesia could incite formation of bullae. It is crucial to make a comprehensive dental care plan and conservative dental treatment to eliminate pain. We present a patient with EB that treated under local anesthesia. It can prevent adverse effects of general anesthesia. We applied partial pulpotomy with Biodentine to manage deep carious lesions. To manage gag reflex and anxiety, mild oral sedation was used. At 1-year follow-up, the patient was at a good oral health condition.     

Direct measurement of the bactericidal effect of chlorhexidine on human dental plaque

The purpose of the present study was to determine by means of a fluorescence test the ratio between vital and dead bacteria in dental plaque before and after 0.1% chlorhexidine rinses. Plaque was stained by fluoresceindiacetate (FDA), which gave a green color to the living micro-organisms, and by ethidiumbromide (EB), which introduced a red color into the nucleic acids of the dead bacteria. 1-, 2- and 3-day-old undisturbed plaque harbored 80 to 85% living micro-organisms. 1 h after chlorhexidine use, this % was reduced to a plateau of 19 to 34% of vital bacteria present in the sampled plaque. Between 6 and 8 h later, this % rose towards its original value. This study has shown that FDA/EB staining provides a method for direct measurement of bacteria vitality in dental plaque and, as such, can be of great value for testing the efficacy of various antiplaque agents.     

Salivary function of persons with hereditary epidermolysis bullosa

Oral alterations of the hard and/or soft tissues are commonly associated with the different types of epidermolysis bullosa (EB). The relationship of oral soft and hard tissue changes to the disease mechanisms in different EB types remains to be elucidated. The purpose of this investigation was to evaluate selected aspects of salivary function in a healthy control population and in persons affected with different types of EB. Sixty-one patients with EB, representing all the major types of EB, and 36 unaffected persons were examined to measure their stimulated salivary flow rates and salivary levels of IgA, albumin, and total protein. Our results show that none of the types of EB demonstrated a decreased salivary flow rate. However, patients with recessive dystrophic EB had significantly elevated salivary IgA, albumin, and total protein levels. The increased IgA level seen in this form of EB appears most likely to be related to the high prevalence of oral blistering rather than the result of altered mucosal immune function. Despite severe cutaneous and extracutaneous involvement associated with inherited EB, we found no evidence to support the hypothesis of abnormal salivary function or mucosal immunity in this disease. Taken together, these findings suggest that the rampant dental caries seen in the severe forms of EB are likely attributable to nonsalivary factors such as enamel involvement, soft tissue alterations, and/or diet. Alternatively, there may be mucosal immunity or salivary enzyme alterations that influence oral disease in these patients, but these were not evaluated in this investigation.     

Dental implants in patients with oral mucosal diseases – a systematic review

To reveal dental implants survival rates in patients with oral mucosal diseases: oral lichen planus (OLP), Sjögren's syndrome (SjS), epidermolysis bullosa (EB) and systemic sclerosis (SSc). A systematic literature search using PubMed/Medline and Embase databases, utilising MeSH and search term combinations identified publications on clinical use implant‐prosthetic rehabilitation in patients with OLP, SjS, EB, SSc reporting on study design, number, gender and age of patients, follow‐up period exceeding 12 months, implant survival rate, published in English between 1980 and May 2015. After a mean observation period (mOP) of 53·9 months (standard deviation [SD] ±18·3), 191 implants in 57 patients with OLP showed a survival rate (SR) of 95·3% (SD ±21·2). For 17 patients with SjS (121 implants, mOP 48·6 ± 28·7 months), 28 patients with EB (165 implants, mOP 38·3 ± 16·9 months) and five patients with SSc (38 implants, mOP 38·3 ± 16·9 months), the respective SR was 91·7 ± 5·97% (SjS), 98·5 ± 2·7% (EB) and 97·4 ± 4·8% (SSc). Heterogeneity of data structure and quality of reporting outcomes did not allow for further comparative data analysis. For implant‐prosthetic rehabilitation of patients suffering from OLP, SjS, EB and SSc, no evidence‐based treatment guidelines are presently available. However, no strict contraindication for the placement of implants seems to be justified in patients with OLP, SjS, EB nor SSc. Implant survival rates are comparable to those of patients without oral mucosal diseases. Treatment guidelines as for dental implantation in patients with healthy oral mucosa should be followed.     

Studies on microleakage of three kinds of dental restorative materials

In this article we studied the microleakage of three kinds of dental restorative materials by using 131I label compounds and 3H label compounds. The results revealed that the radioactive isotope reverse diffusion method has the advantages of which it is quantitative and sensitive. It is an acceptable and feasible measuring method. Under the condition described in this article, microleakage phenomenon exists in all the test materials and the margin sealing ability of EB composite resins is superior to the others.     

Filament end-rounding quality in electric toothbrushes

OBJECTIVES: A good quality of filament tips is desirable to protect both gingiva and dental hard tissues. The aim of the present study was to compare the end-rounding quality of the filaments in 15 electric toothbrushes (Rowenta dentaclip ZH-07, dentaclip ZH 010, rotaclip ZH-11; Blend-a-dent Wellenprofil 2000 hart, Wellenprofil 2000 mittel-weich, Medic for kids; Broxo; UltraSonex; Krups 548, Waterpik BH-4U; Butler Gum; Dr. Best e-Flex3; Oral-B (EB3, EB 17-8, Plak Control Kids)). MATERIAL AND METHODS: From each brand five brushes were chosen randomly. Five tufts were selected from each brush and studied with a scanning electron microscope (x 45) at a viewing angle of 45 degrees. The filament tips were numbered from top left to bottom right and selected filaments were judged by a well-trained, blinded second examiner according to the Silverstone & Featherstone method. RESULTS: Nine of the 15 brands examined showed a high ("acceptable": >89%), four products a medium (76-84%) and two a bad (34-38%) end-rounding quality. CONCLUSION: A good quality of filament tips is claimed by the dental profession to protect both gingiva and dental hard tissues from abrasion. This could be observed for 13 of the 15 brands examined.     

Epidermolysis bullosa

Epidermolysis bullosa (EB) describes a group of rare genetic mechanico-bullous disorders. The disease has several modes of inheritance with various degrees of severity and expression. Two cases of recessive dystrophic EB are presented which show typical cutaneous, mucosal and occular involvement.
The teeth were severely affected in both cases with much of the coronal enamel absent upon emergence into the oral cavity. The cervical enamel showed occasional hypoplastic pits. In one case, there was taurodontism of the permanent first molars. This case illustrates the increased occurrence of taurodontism associated with diseases affecting tissues of ectodermal origin. Taurodontism has not been previously reported with EB. Histologic studies of the teeth using light microscopy (LM) and scanning electron microscopy (SEM) revealed that the cementum and dentin were normal. The enamel, under LM, showed a prismatic structure with a broad neonatal line. SEM showed a wide amorphous zone of enamel adjacent to the dentin. Generalized throughout the enamel were globular areas within the prisms, which appeared to be local deformations. Hypoplastic pits on the surface were round and of varying diameters. The affected teeth exhibited both hypomaturation and hypo-plastic defects of the enamel.     

Dystrophic epidermolysis bullosa: Oral findings and problems

Dystrophic epidermolysis bullosa (DEB) is one of the three major types of epidermolysis bullosa (EB), an inherited cutaneous disease with blister formation following minor trauma. A subtype of DEB is recessive dystrophic epidermolysis bullosa, Hallopeau‐Siemens type (RDEB‐HS), where marked scarring leads to deformities of extremities. In RDEB‐HS the mucous membranes may also be involved and form adhesions with ankyloglossia and microstomia. Oral hygiene is difficult. A 7‐year‐old boy with RDEB‐HS was brought to the Johannes Gutenberg University dental clinic with dental pain. He had multiple carious lesions, poor oral hygiene and gingivitis. Because he was noncompliant and had microstomia, he required dental therapy under general anesthesia. The recall visits over the past two years had demonstrated that the dental health of this patient with RDEB‐HS could be maintained by means of improved oral home care, using antibacterial agents.     

The talon cusp--an uncommon anomaly

Talon cusps are an uncommon dental anomaly of odontogenic origin. Talon cusps can also present with other dental anomalies, however these have been infrequently reported in the literature.The following is a report of two cases in which talon cusps were detected in combination with two distinct dental anomalies: an ectopic canine and transposition.