|Year : 2014 | Volume
| Issue : 1 | Page : 30-32
Comprehensive and novel dental management of a child with epidermolysis bullosa
Sai Sankar Avula Jogendra, Ratna Aditya Akurati, Pratap Gowd Margana Jai Shankar, Raj Kumar Chowdhary Kopuri
Department of Pedodontics and Preventive Dentistry, Sibar Institute of Dental Sciences, Guntur, Andhra Pradesh, India
|Date of Web Publication||2-May-2014|
Sai Sankar Avula Jogendra
Department of Pedodontics and Preventive Dentistry, Sibar Institute of Dental Sciences, Guntur, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Epidermolysis bullosa (EB) represents a group of hereditary skin disorders manifested by an exceptional tendency of the skin and mucosa to form bullae and vesicles following minor friction and trauma. The pathogenesis of these disorders is unknown. Depending on the type, the clinical manifestations vary from very mild to a disfiguring and disabling disease. Along with the systemic complications, the oral findings like early childhood caries, microstomia, and limited mouth opening are quite evident in these children. Health care professionals should be aware of preventive and therapeutic measures to be adopted in order to provide a safe and effective dental treatment in these patients. The present case documents a child diagnosed of EB with early childhood caries, describing the phases and difficulties of dental treatment, and also presents a novel impression technique.
Keywords: Early childhood caries , epidermolysis bullosa, microstomia
|How to cite this article:|
Jogendra SA, Akurati RA, Jai Shankar PM, Kopuri RC. Comprehensive and novel dental management of a child with epidermolysis bullosa. Indian J Paediatr Dermatol 2014;15:30-2
|How to cite this URL:|
Jogendra SA, Akurati RA, Jai Shankar PM, Kopuri RC. Comprehensive and novel dental management of a child with epidermolysis bullosa. Indian J Paediatr Dermatol [serial online] 2014 [cited 2020 Feb 25];15:30-2. Available from: http://www.ijpd.in/text.asp?2014/15/1/30/131836
| Introduction|| |
Epidermolysis bullosa (EB) is a spectrum of hereditary mechanobullous disorders which are characterized by blistering of tissues containing stratified epithelia.  Patients with this condition present with long-lasting blisters, and bullae formation in their skin and mucosal membranes as a response to minor friction or trauma.  Von Hebra in 1870 first described this disease as "erblichen pemphigus" and it was later termed as "epidermolysis bullosa hereditaria" by Kobner in 1886.  The overall prevalence of EB is estimated to be 1:10,000, and there is no gender, racial, ethnic, or geographic predilection. The disease often manifests at birth or during the first year of life. 
Clinically, two main types of EB are described: Simplex type and dystrophic type. Scarring does not occur and mucus membranes are rarely affected in the simplex type, whereas in the dystrophic type, scarring occurs.  The most recent classification recognizes 4 major EB types and 30 subtypes depending upon the histological level of tissue separation. The groups include intraepidermal EB (simplex), junctional EB, dermolytic EB (dystrophic), and mixed EB (Kindler syndrome).  Skin biopsy is the first diagnostic tool. The standard hematoxylin and eosin staining reveals the level of skin separation. Electron microscopy (EM) and immunofluorescence mapping differentiate various EB types. 
The present case documents a child diagnosed of EB simplex (EBS) with early childhood caries. Emphasis has been given on the measures that health care professionals should adopt.
| Case report|| |
A 7-year-old boy presented with pain in the lower right back tooth region since 3 days. According to the medical records, the patient was diagnosed of EBS for which he was receiving palliative treatment. There was no relavent family history. The child had a short stature, was undernourished and anemic, and his diet was limited to soft, pureed foods. On physical examination, widespread blisters and scars were observed all over the body, which were more conspicuous on the extremities with no evidence of pseudo-syndactyly [Figure 1]. Extraoral examination showed less pigmented, sparse scalp hair and microstomia with restricted mouth opening. No prominent bullae were observed on the oral mucosa. Generalized gingival inflammation was evident with extensive carious lesions on all primary teeth except canines [Figure 2]. History revealed that oral bullae had decreased as the age advanced. Radiographic investigation confirmed the presence of a complete set of permanent teeth and pulpal involvement of clinically carious teeth [Figure 3]. Based on the findings and history, the condition was provisionally diagnosed as early childhood caries and was planned for complete oral rehabilitation.
Treatment planned was to extract the unrestorable primary teeth (64 and 84), followed by space maintainers, to perform pulp therapy in pulpally involved primary teeth (51, 52, 61, 62, 75 and 85) and to carry out restorations for 54, 55, 71, 72, 81 and 82. Extraction of 64 and 84 was carried out under local infiltration. Pulp therapy was performed on the maxillary incisors, 75 and 85, followed by restoration with omega loops and crowns. The occlusal and proximal lesions in relation to 54, 55, 71, 72, 81 and 82 were restored [Figure 4] and [Figure 5]. Due to limited mouth opening, sectional trays were designed for making impressions in fabricating space maintainers. Extreme care was taken to avoid trauma and ulcerations during the oral examination and dental treatment.
|Figure 1: (a) Front view of the face; (b and c) upper and lower extremities|
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| Discussion|| |
The EBS type is the least serious form of the disease. This variant appears at birth or shortly after, and is characterized by the development of bullae, mainly limited to the hand and feet. Extracutaneous organs and nails are rarely involved. The blister formation occurs within basal keratinocytes due to mutations in the keratin 5 and 14 genes. In most patients, blisters are mild and do not scar after they heal. Some forms of EBS affect just the hands and feet (simplex localized with hypodontia "Kallin"); other forms exhibit more widespread blistering, as well as hair loss and missing teeth (simplex herpetiformis "Dowling-Meara").  Blistering of the mouth and scarring are associated with multiple, life-altering oro-dental complications. Tissue distortions and abnormalities such as tooth crowding, decreased maxillary growth, ankyloglossia (decreased tongue movements), microstomia, vestibular obliteration, loss of palatal rugae and/or papillae may develop in all types of EB.  The effect of EB on tooth formation and structure is unclear, and its clinical expression ranges from mild pitting to severe hypoplasia. Dentine formation in all types of EB appears to be normal, although taurodontism has been documented.  This condition has to be differentiated from bullous impetigo (pemphigus neonatorum), Ritter's disease, porphyria congenita, congenital syphilis, juvenile bullous dermatitis herpetiformis, and amelogenesis imperfecta.  Defective enamel, poor oral clearance of foods, and inability to achieve adequate oral hygiene may lead to rampant decay in these children. In the present case, the child reported with poor oral hygiene and multiple carious lesions, necessitating complete oral rehabilitation.  Since the child was cooperative in this case, rehabilitative procedures were carried out under local anesthesia.
The presence of microstomia with restricted mouth opening made the delivery of dental treatment in this case a difficult task. Hence, alternative means like sectional trays were designed on the diagnostic casts with 1-mm wax spacer and a lock. Impressions were made with these trays and were joined before pouring the master cast for fabrication of space maintainers [Figure 6].
|Figure 6: (a) Upper and lower impression trays with attachments; (b) split impressions joined in upper and lower arch|
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Individuals with EB can be treated much like any other patient with few alterations in the treatment protocol and the focus should be on preventive measures.
| Conclusion|| |
The following are the points learnt from this case:
- Modifications to operative technique in patients with EB include extreme care of fragile tissues using little pressure with no sliding movements and entering the mouth in as few occasions as possible
- The air syringe and suction must be used carefully
- In case of applying a high-vacuum suction, it is suggested to use a padded gauge piece
- Airotor should be used cautiously, or a slow-speed hand piece under irrigation is a better alternative
- Adequate oral hygiene should be encouraged with the gentle use of small-headed soft toothbrushes and small sponge applicators
- Physical removal of bacterial plaque is achieved by using 0.2% chlorhexidine gluconate solution twice daily.
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[Figure 1], [Figure 3], [Figure 4], [Figure 5], [Figure 6]