Oral lesions in recessive dystrophic epidermolysis bullosa

OBJECTIVE: To study the prevalence of oral lesions in 35 patients diagnosed with generalized recessive dystrophic epidermolysis bullosa (RDEBg), with a quantification of their microstomia in comparison with a control group. MATERIAL AND METHODS: The presence of oral mucosal lesions and interincisal maximum oral aperture (MOA) was determined, classifying microstomia according to the method of Naylor, Douglass and Mix (1984). RESULTS: Blister lesions were identified in 92% of the patients at the time of exploration--the tongue being the most affected location. Microstomia and palatal atrophy were the most prevalent sequelae (100%), while ankyloglossia, vestibular obliteration and lingual depapillation were recorded in over 90%. In 80% of the patients interincisal MOA was <30 mm (severe microstomia), while in the remaining cases maximum aperture was in the range of 31-40 mm (moderate microstomia). CONCLUSIONS: Blister lesions were found throughout the oral mucosa in our series of patients with RDEBg, the most frequently affected location being the tongue. These lesions in turn led to invalidating sequelae such as microstomia and ankyloglossia.     

General anesthesia for dental care management of a patient with epidermolysis bullosa: 24‐month follow‐up

Epidermolysis bullosa comprises a group of uncommon skin‐related diseases, characterized by the formation of blisters on mucocutaneous regions occurring spontaneously, following a trauma, exposure to heat, or as a result of minimal mechanical trauma. The dental treatment of the patient with epidermolysis bullosa raises many questions and discussions, due to the difficulty of carrying out the procedures. This report aimed to detail the clinical considerations of the treatment under general anesthesia of a patient with epidermolysis bullosa. The extraction of all deciduous teeth under general anesthesia was recommended based on the clinical and radiographic examinations. At 24‐month follow‐up, the patient had great improvement in oral hygiene without new caries lesions. The patient has been followed‐up at every month for caries lesion prevention and permanent tooth development. The treatment under general anesthesia provided the ideal safe conditions and was beneficial for the patient.     

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.     

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.     

Recessive dystrophic Epidermolysis bullosa. Two case reports with 20-year follow-up

These two case reports highlight the enormous clinical difficulties faced by dentists in providing satisfactory long-term dental care to patients who suffer from Epidermolysis bullosa. Problems of bullae formation in oral soft tissues and subsequent scarring are outlined in relation to the difficulty of maintaining satisfactory oral hygiene and a diet leading to minimal dental caries experience. The behavioural problems of maintaining patient compliance for preventive and restorative dentistry in this painful and debilitating disease are illustrated in these case reports. Difficulties in providing restorative care, either under local anaesthesia or general anaesthesia are discussed, and a novel replacement of non-viable carious anterior teeth using a nine-unit porcelain fused to metal Rochette type bridge is presented. Dental management of patients with Epidermolysis bullosa should commence at birth, and non-compliance in dental attendances should be followed up by social workers to prevent the disastrous oral morbidity that frequently occurs in such patients.     

Endodontic management of a patient with dystrophic epidermolysis bullosa: A case report

Epidermolysis bullosa is a congenital genetic disease that causes blistering and erosion of the skin and mucosa. The main known forms include simple, junction, dystrophic and mixed subtypes. This case report presents the endodontic management and 1‐year follow‐up of a 27‐year‐old female patient with epidermolysis bullosa who was referred to the Faculty of Dentistry, K?r?kkale University, Turkey. An extraoral examination showed that the patient had multiple scars and blisters. The intraoral examination revealed ankyloglossia, microstomia, shallow buccal and vestibular sulci, enamel hypoplasia, gingival inflammation, mouth ulcers, symptomatic and asymptomatic deep caries, a tooth with an apical lesion and a tooth with pulpitis. The dental treatment was divided into four stages: (i) oral hygiene motivation and elimination of gingival bleeding, (ii) restorative and/or endodontic procedures, (iii) extractions and prosthetic treatments and (iv) recall appointments. A 1‐year follow‐up radiographic examination of the periapical status of the root canal treatments was clear.     

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.     

Paraneoplastic epidermolysis bullosa acquisita associated with multiple myeloma

Epidermolysis bullosa acquisita is a rare acquired autoimmune subepidermal blistering disease that clinically resembles other vesiculobullous lesions such as pemphigus vulgaris and cicatricial pemphigoid. Multiple myeloma is the most common plasma cell malignant disorder characterized by a single clonal expansion and increased level of a single immunoglobulin. Epidermolysis bullosa acquisita has been reported with other systemic diseases such as lymphoma. In this case report, we present a patient with paraneoplastic epidermolysis bullosa acquisita associated with multiple myeloma.     

Orthodontic treatment of a patient with recessive dystrophic epidermolysis bullosa: a case report

Epidermolysis bullosa (EB) represents a heterogeneous group of hereditary, chronic, non-inflammatory skin pathologies. Recessive dystrophic epidermolysis bullosa (RDEB) is characterized by sub-lamina dura separation, due to blistering below the lamina densa of the basement membrane zone, and is associated with the absence of Type VII collagen fibers. The mouth, pharyngeal, and esophageal mucosa are compromised by scarring and tissue contraction. The purpose of this article was to present a case report on an orthodontic patient with RDEB.     

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.     

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.     

Genotype–oropharyngeal phenotype correlation in Mexican patients with dystrophic epidermolysis bullosa

Previous investigations have attempted to correlate the genotype with the cutaneous phenotype in patients with epidermolysis bullosa (EB), but never with the oropharyngeal phenotype. Seventeen dystrophic EB (DEB) patients were genotyped for COL7A1 gene mutations and divided into five distinct groups. Oropharyngeal disease severity was assessed with the Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score by an oral medicine specialist. The genotype–phenotype correlation was calculated by Kruskal–Wallis analysis of variance using the Mann–Whitney test, applying the Bonferroni correction. The most severe oropharyngeal phenotype was found in the group with the 2470insG/3948insT mutation, with a mean disease severity score of 18.50 ± 2.12; the mildest was found in the 6862del16 mutation group, with a mean disease severity score of 0.57 ± 1.13. The most significant difference in median score was found in the total score (P = 0.009), followed by tongue (P = 0.02) and upper lip (P = 0.021), but no correlation was found between disease severity and the groups (P > 0.005, after Bonferroni correction). Multiple comparisons among the five different genotypic groups revealed no statistically significant genotype–oropharyngeal phenotype correlation; it was not possible to establish which group was more severe, or to associate a specific mutation to a specific oropharyngeal phenotype.     

Blood blisters on the soft palate in Angina bullosa haemorrhagica Case reports

Reports are presented on four patients who experienced blood blisters on their soft palates due to suspected Angina bullosa haemorrhagica. The appearances of the blood blisters and the ulcerated areas caused by their collapse are described. Means of differentiating blood blisters in the mouth caused by other disorders are discussed.     

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.     

Impaired secretory immunity in dystrophic epidermolysis bullosa

Dystrophic epidermolysis bullosa is a congenital disorder characterized by blistering of the skin and oral mucosa. This study investigated the hypothesis that children with dystrophic epidermolysis bullosa have impaired oral secretory immunity. Immunoglobulin A (IgA), secretory IgA and IgG concentrations, and IgA and secretory IgA antibody levels to Candida albicans, Lactobacillus casei and Streptococcus mutans were measured in whole saliva from 22 children with dystrophic epidermolysis bullosa and 22 matched controls. Salivary total IgA and total IgG concentrations were significantly raised in dystrophic epidermolysis bullosa due to serum leakage from oral blistering, but the converse was seen with secretory IgA. This suggestion of a mucosal immune defect was supported by decreased secretory IgA antibody responses to all three microorganisms tested. This apparent defect in secretory immunity in dystrophic epidermolysis bullosa may be due to mucosal involvement and damage resulting in impaired antigen sampling in mucosal associated lymphoid tissue or to impaired transport of secretory IgA across the salivary gland mucosa.     

A functional natural dentition for all – and for life? The oral healthcare system needs revision

The cost of dental care adds to the costs of the already overburdened health sector. Do we – as patients and as society –receive oral health care that is both aligned with the actual disease experience and also, critically based on up‐to‐date scientific knowledge about the major oral diseases? In many places, the practice of dentistry reflects a response to disease patterns that once existed and is based on diagnostic and therapeutic approaches that are no longer valid. Instead, a new cadre of dental professionals is needed, one that is capable of meeting the actual health needs of our populations. This cadre should ensure that patients maintain a functioning dentition from cradle to grave based on cost‐effective disease control principles. There is an urgent need to: (i) reconsider the roles of the different oral health cadres involved in the provision of oral health care; (ii) integrate oral health into general healthcare services; and (iii) restructure the training of oral health personnel. We advocate a radical reform of the oral healthcare system involving the training of two new types of professionals integrated with the general healthcare system: The oral healthcare provider – a highly skilled professional specialised in the diagnosis and control of oral diseases and with a profound understanding of oral health as part of general health – and the oral clinical specialist – whose role is the provision of advanced oral rehabilitation, able also to treat people with complex chronic diseases and multiple medications.     

To evaluate the comparative status of oral health practices,oral hygiene and periodontal status amongst visually impaired and sighted students

The aim of this study was to evaluate the comparative status of oral health practices, oral hygiene, and periodontal status amongst visually impaired and sighted students. In this study, 142 visually impaired children from a blind school in the age group of 6–18 years were enrolled with a similar number of age and sex matched sighted students studying in different schools of Chandigarh. The outcome variables were oral hygiene practices, oral hygiene status, and periodontal status. The visually impaired had been found to have better oral hygiene practices, a nonsignificant difference of oral hygiene scores but a significantly high value for bleeding scores as compared to sighted students. Age wise comparisons showed that bleeding scores were highly significant in 9–11 years and 12–14 years age group as compared to 6–8 years and 15–18 years age group. It could be related that the increased prevalence of bleeding sites despite of better oral hygiene practices in visually impaired group might be the result of their handicap to visualize plaque.