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.     

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.     

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.     

Oral lesions and dental caries status in perinatally HIV-infected children in Northern Thailand

Summary. Objective. To describe the prevalence of oral lesions and dental caries status in perinatally HIV-infected children.
Design. A cross-sectional study.
Setting. Paediatric HIV outpatient department at the Nakornping Provincial Hospital, Chiang Mai, Thailand.
Patients and methods. Forty children with perinatal HIV infection, from early infancy to 12 years of age, were included in the study. These children were examined for oral lesions and dental caries. A number of children receiving antifungal and antiretroviral (ART) therapy were recorded.
Results. The mean DMFT and DMFS scores were both 2·1 (SD = 2·3). The dft and dfs scores were 4·1 (SD = 5·0) and 10·9 (SD = 14·8), respectively. A total of 57·5% of the children had one or more oral lesions. Oral candidiasis and hairy leukoplakia were the most common oral lesions. Only 12·5% of children had received ART. A total of 22·5% of the children had a history of receiving antifungal therapy.
Conclusions. Oral lesions and dental caries were relatively high in this study. Consequently, treatment and prevention for oral lesions and dental caries are inevitably required for children with HIV infection in Northern Thailand. Furthermore, ART should be made available for all HIV-infected children to decrease the prevalence of HIV-associated oral lesions.     

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.     

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.     

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.     

Patterns of oral manifestation of HIV/AIDS among 225 Nigerian patients

Objective:  The purpose of this study was to determine the prevalence of the oral manifestations of HIV/AIDS and to correlate the prevalence of these lesions with the stages of the disease in the Ife-Ijesa zone, Nigeria. No comprehensive data were available for correlating it with the staging of HIV/AIDS in this region.
Subjects:  The pattern of oral HIV lesions as classified by the EC-Clearinghouse was studied in 225 confirmed consecutive HIV-infected patients in this zone.
Methods:  Clinical dental examinations were conducted under natural daylight on all consenting HIV patients, sitting in an upright chair, using dental mirrors and probes.
Results:  The prevalence of oral HIV lesions was 84.0%, with lesions ranging in number from one to six. The commonest HIV lesion was pseudo-membranous candidiasis (43.1%) followed by erythematous candidiasis (28.9%), angular cheilitis (28.9%), linear gingival erythema (24.0%) and ulcerations (8.9%). Lesions less commonly found were oral hairy leukoplakia (1.3%) and salivary gland swellings (1.3%). Heterosexual intercourse was the most common mode of transmission (94.7%) and HIV-1 (96.9%) the most prevalent pathogen among the study population. The majority of the patients were in the WHO clinical stage III (59.1%) and presented late.
Conclusions:  The prevalence of oral HIV lesions in the present study was high.     

Parental perspectives on preterm children's oral health behaviour and experience of dental care during preschool and early school years

Background.  Children born preterm (PT) have medical conditions and impairments that may affect their oral health.
Hypothesis.  Our hypothesis for the study was that PT children display more dental behaviour management problems (BMPs) and less favourable oral health behaviour than controls (C).
Methods.  Parents of 153 PT children and 153 C children were interviewed regarding the children's oral health behaviour and experience of dental care on two occasions, 2 years apart. The interviews concerned the preschool period and the early school years, respectively.
Results.  BMPs were more common in PT children of preschool age, but not during the early school years. Regarding oral health behaviour, there were no differences between the groups, except that PT children had more problems with toothbrushing than C children in the preschool period, in spite of the fact that the PT group reported more medical health problems and more anxious behaviour and indications of cognitive problems than the C group.
Conclusions.  Children born PT exhibit several risk factors for both BMP and impaired oral health. It is essential that this group of patients is identified early and receives special attention from the dental services.     

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 manifestations of eating disorders: a critical review

Background:  Eating disorders (ED) are a group of psychopathological disorders affecting patient relationship with food and her/his own body, which manifests through distorted or chaotic eating behavior; they include anorexia nervosa, bulimia nervosa and ED not otherwise specified and may be burdened with life-threatening complications.
As oral manifestations of ED can occur in many phases of disease progression, they play a significant role in assessment, characterization and prognosis of ED.
Methods:  Mucosal, dental, and salivary abnormalities associated with ED have been reviewed. Relations between oral menifestations and pathogenesis, management and prognosis of ED have been critically analysed.
Results:  Oral manifestations of ED include a number of signs and symptoms involving oral mucosa, teeth, periodontium, salivary glands and perioral tissues; differences exist between patients with anorexia and bulimia. Oral manifestations of ED are caused by a number of factors including nutritional deficiencies and consequent metabolic impairment, poor personal hygiene, drugs, modified nutritional habits and underlying psychological disturbances.
Conclusion:  Oral manifestations of ED can cause impairment of oral function, oral discomfort and pain, and an overall deterioration of aesthetics and quality of life. Their treatment can contribute to overall patient management and prognosis.     

Oral soft tissues in hereditary epidermolysis bullosa

Epidermolysis bullosa (EB) is associated with diverse oral manifestations, which can potentially involve both hard and soft tissues, depending on the specific EB subtype. This study determined the frequency and extent of oral soft tissue involvement in the inherited forms of EB. Examination of 216 affected persons revealed significant differences in the oral soft tissue involvement among the various types of EB. The frequency of oral involvement was greater in the dominant dystrophic (81.1%) and simplex (generalized, 58.6%; localized, 34.7%) types than previously reported. The marked frequency of oral blistering was similar in both major subtypes of junctional (Herlitz, 83.3%; non-Herlitz, 91.6%) and recessive dystrophic EB (generalized, 100%; localized, 92.3%). Obliteration of the oral vestibule, ankyloglossia, and microstomia were consistent findings in generalized recessive dystrophic EB. Oral milia were present in all major EB categories, most prevalently in the dystrophic forms, but were not seen in all the distinct EB subtypes. These findings indicate that although there are no pathognomonic intraoral soft tissue manifestations in the various forms of inherited EB, there are predictable patterns of involvement associated with specific subtypes of this disease. Understanding the oral involvement associated with EB may aid clinicians in the development of more advanced therapeutic approaches that are compatible with and directed at the unique characteristics of each EB subtype.     

Recessive dystrophic epidermolysis bullosa—oral rehabilitation using stereolithography and immediate endosseous implants

Dental management of patients with epi-dermolysis bullosa (EB) is challenging because of the severe soft tissue lesions associated with this disease. A case history is presented where two immediate endosseous implants were placed in the mandible of a patient with recessive dystrophic EB using computer-aided technology to plan the surgery and prosthetic rehabilitation. After a 24-month follow-up, the prosthesis was stable with healthy asymptomatic soft tissue around the implants. The stereolithographic model provides a precise and noninvasive copy of the mandibular and maxillary arches of patients with EB for rehabilitation of the dentition with immediate endosseous implants and a prosthesis.     

Oral Langerhans cell histiocytosis in Malaysian children: a 40-year experience

Background.  Oral Langerhans cell histiocytosis is generally seen in children.
Objective.  To determine the clinicopathological features of oral LCH in Malaysian paediatric patients.
Methods.  A retrospective study was carried out to determine the clinicopathological features of Langerhans cell histiocytosis (LCH), Letterer–Siwe disease, Hand–Schuller–Christian disease, eosinophilic granuloma, and histiocytosis X occurring in the oral cavity in children, diagnosed histologically in the main oral histopathology laboratory in Malaysia from 1967 to 2007.
Result.  There were 17 cases (eight girls and nine boys) with age ranging from 1 to 7 years. There were ten Malays, four Chinese, two Indians, and one of other ethnicity. Thirteen cases presented as gingival swellings with six of these cases accompanied with mobility of the teeth. Nine cases involved the mandible, two in the maxilla, and two cases in both the maxilla and mandible. The radiographic findings were mentioned only in nine cases with presence of bony erosion or destruction of the jaw bones. Four cases had punched-out radiolucencies of the skull. The patients also had other systemic signs and symptoms: skin lesions ( n  = 5), hepatosplenomegaly ( n  = 2), prolonged fever ( n  = 2), diabetes insipidus ( n  = 1), and exophthalmos ( n  = 1). Two cases were known cases of systemic LCH.
Conclusion.  The histopathologic features of LCH are easily recognized; however, with the development of immunostaining, the use of CD1a helps in confirming the diagnosis.     

Oral health-related quality of life of children with oligodontia

Objectives.  To assess the functional and psychosocial impact of oligodontia in children aged 11–14 years.
Methods.  Children aged 11–14 years with oligodontia were recruited from orthodontic clinics when they presented for orthodontic evaluation. All completed a copy of the Child Perceptions Questionnaire for 11- to 14-year olds, a measure of the functional and psychosocial impact of oral disorders. Information on the number and pattern of missing teeth for each child were obtained from charts and radiographs.
Results.  Thirty-six children were included in the study. The number of missing teeth ranged from one to 14 (mean = 6.8). Just over three-quarters of the subjects reported experiencing one or more functional and psychosocial impacts 'Often' or 'Everyday/almost everyday'. Correlations between scale and sub-scale scores and the number of missing teeth were weak and nonsignificant.
Conclusions.  Children with oligodontia experience substantial functional and psychosocial impacts from the condition. When compared with other clinical groups, children with oligodontia appear to have worse oral health-related quality of life than children with dental decay and malocclusion, but better oral health-related quality of life than children with oro-facial conditions.     

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.     

Do maternal factors influence the dental health status of Nigerian pre‐school children?

Objective.  This study was designed to investigate the relationship between maternal related factors and the dental health status of pre-school children in Lagos State, Nigeria.
Methods.  A cross-sectional study of 404 pairs of mothers and their pre-school children was conducted at two selected primary health centres in Lagos State Nigeria. An interviewer administered questionnaire recorded the mother's socio-demographic characteristics and assessed her perception of her child's dental needs, attitude to oral disease prevention, level of dental health knowledge and attitude to oral health. The child's dental status was assessed using the dft index (caries status) and the simplified oral hygiene index. Data analysis tools included Spearman's correlation coefficient and multivariate logistic regression.
Results.  Maternal age, maternal education, location of residence, maternal knowledge, and attitudes were all positively correlated with the child's caries and oral hygiene status. There were statistically significant correlations between maternal attitude and the oral hygiene index ( P  = 0.01) and dft score ( P  = 0.001). Maternal age also had a significant relationship with the child's caries status ( P  = 0.003).
Conclusion.  This study concluded that maternal age and attitude were important determinants of caries experience whereas the mother's attitude was an important determinant of oral cleanliness in pre-school children in Lagos State Nigeria.     

Epidermolysis bullosa: dental and anesthetic management of two cases

Epidermolysis bullosa (EB) is a group of rare genetic disorders which are of dental interest because of their specific oral manifestations and management difficulties. Two cases of recessive EB which may be classified clinically as the atrophicans-gravis Herlitz type are described. Dental therapy consisted of extractions and stainless steel crown restorations, with inhalation general anesthesia. The need for and advantages of early preventive and restorative dental care are illustrated by the cases presented.