Epidermolysis bullosa (EB) is a large rare group of genetically determined disorders that are inherited as autosomal dominant or recessive.
Most common feature of this disease is blisters formation on the skin and oral mucosa at different levels following trauma or traction.
Exact cause still unknown, but mostly appears to be related to abnormal enzyme activity and collagen degradation.
There are three major subtypes; simplex, junctional and dystrophic.
The most common oral manifestations involve, enamel hypoplasia, rampant caries, microstomia tongue atrophy, oral ulceration with scaring and constriction of buccal vestibules.
Dental treatment of these patients is a great challenge that involves preventive measures by reinforcing oral hygiene and regular fluoride applications, in addition to treatment of carious teeth and extraction of teeth with poor prognosis.
In this study two cases of EB; simplex and dystrophic types are reported and their specific clinical manifestations with their dental clinical treatment are described.
Key Words: Epidermolysis bullosa, Blisters, Dental caries, Treatment, Fluoride applications.
Epidermolysis bullosa is a group of rare inherited disease of the skin and oral mucosa, the severity of this disease ranges from localized to generalized lethal blisters.1 It manifests clinically as erosions, blisters and ulcers that are formed in response to pressure, mild trauma or friction.2 The cause of this disorder is due to absent or infective connective tissue anchoring fibrils, causing a loosely bound epithelial-sub epithelial interface.3
The children that are affected by epidermolysis bullosa are called Butterfly or Cotton wool babies because their skin is fragile.4 Recent classification depends on the level of tissue cleavage following trauma5, there are three major subtypes; EB simplex where cleavage is intraepidermal, it appears at birth with bullae formation limited to hands and feet that heal without scaring6, dystrophic EB where splitting is at sub lamina densa with bullae formation on feet, ankles, elbows and hands with absent or dystrophic nails and oral lesions that healed with scars, junctional EB the cleavage at lamina lucida , lesions appear at birth bullae and erosions formed which healed with scars on hands and feet with dystrophic nails and oral lesions.6
The most dental complications are increase prevalence of dental caries, hypodontia, defective crowns and enamel hypoplasia with oral tissue scaring leading to microstomia.7,8 The disease is painful, pervasive and debilitating and there is no cure, only palliative management by close care of patient with support and prevention of new bullae formation is done.9
Dental treatment of these patients is a great challenge to avoid new bullae formation, so they need special dental care by using soft dental brush, restoration of carious teeth and preventive treatment by frequent application of fluoride, also patients may need diet supplement as proteins, vitamins and iron to prevent anemia.7 The aim of presenting these two case reports was to describe the oral and dental features of two different types of Epidermolysis bullosa; simplex and dystrophic types and to discuss their dental management.
CASE REPORT 1
A 7 years old female patient with recessive dystrophic EB attended our pediatric clinic for treatment of carious primary teeth. Clinical examination showed, hemorrhagic blisters in the mouth, which started at birth that appeared spontaneously after minor trauma. All the patient`s teeth were destroyed by caries and gingiva was inflamed because of the difficulty of performing good oral hygiene using tooth brush.
During dermatological examination, several blisters were observed in different areas of her body; on her face, trunk, upper and lower limbs and there were hemorrhagic blisters and scars on the palms and soles. There was a marked dystrophy on the fingernails and absence and fusion with deformity of toenails and there was severe intraoral blistering with scar formation, microstomia and obliteration of the oral vestibule.
After evaluation of panoramic radiograph and intraoral examination the following treatment plan was made; extractions of the primary teeth with poor prognosis by using topical local anesthesia for 4 minutes, followed by infiltration local anesthesia that should be injected deeply and slowly to avoid tissue damage and blistering. The patient was taught to maintain good oral hygiene by brushing techniques and topical application of 0.12% chlorohexidine gel by cotton bud after lunch and at bed time for 8 days. The patient was followed once every 3 months to control her oral health and dental caries.
CASE REPORT 2
A 5 years old boy was referred from routine clinic to our pediatric clinic because of pain related to carious upper left primary first molar. The patient had Epidermolysis Bullosa simplex since birth. There was no family history of EB, clinical examination showed several blisters on his hands, feet, face, neck, trunk and inside the mouth and absence of his toenails with a degree of deformity and blisters on his soles due to friction from his socks.
Intraorally, blisters of different sizes were seen on oral mucosa and tongue, the gingiva was red, edematous and inflamed with plaque deposits on all his teeth. A panoramic view was taken and treatment plan was assessed. First, carious primary tooth was assessed, it was not restorable so extraction was done by using topical and local anesthesia that was injected deeply and slowly into the soft tissue to avoid trauma to the fragile oral tissues.
Oral hygiene instructions were given to improve his oral condition, by using soft tooth brush and topical application of 0.12% chlorohexidine gel with cotton bud for one week, with professional application of fluoride varnish with cotton bud once every 3 months. The patient will be seen in this clinic once every 3 months to control his oral health.
Epidermolysis Bullosa is a mucocutanous blister formation disorder of hereditary nature. These blisters appeared due to a minor trauma or no apparent trauma. EB has a wide variety of manifestations and complications, the major phenotypes have been described depending on the level of cleavage of the basement membrane at dermoepidermal junction which are; simplex, junctional and dystrophic.1
EB simplex usually appears with milder symptoms while junctional and dystrophic types may appear with severe symptoms and multiorgans involvement.10 EB simplex is a non-scaring dominant disorder although the type of inheritance is recessive in some subtypes11, the intraepidermal bullae occur due to cytolysis of the basal cells, while in junctional and dystrophic forms the separation occurs through the lamina lucida of the basement membrane and lamina densa.7
In both cases presented in this article, bullae were manifested at or shortly after birth involving the site of pressure or trauma, ulcers and erosions were observed in addition to microstomia in the oral cavity with obliteration of oral vestibule that complicated dental treatment. Nails were involved either dystrophic or absent in both cases.
Both of them have many bullae and scars involving their hands, elbows, soles and knees. The dental treatment of the patient in case 1 wasn't simple, while the patient in case 2 mainly required preventive measures. Restorative treatment and extraction of the patient in case 1 were undertaken by using topical anesthesia, then slow injection of local anesthesia to avoid blistering and scaring.
Preventive measures such as plaque related diseases of caries and periodontal disease, should be considered especially for those patients who have diminished oral opening and obliteration of the labial sulcus that make oral hygiene maintenance difficult, even minor trauma from tooth brush may cause oral bullae that lead to scaring.
In case 2 adequate oral hygiene was considered since he had a very poor oral hygiene with plaque deposits covering all his teeth. So he was advised to use a small headed soft tooth brush with topical application of 0.12% chlorohexidine gel for one week. Chlorohexidine adsorbed to oral mucosa surfaces where it exerts an antibacterial effect for many hours, which also reduces secondary infection of ulcerated areas and promotes healing. The use of fluoride to prevent dental caries is another vital point in prevention. Dental visits should be carried out once every 3 months.
Dentists should be aware of oral and dental manifestations of Epidermolysis Bullosa to provide the patient with proper restorative and preventive treatment. Maintaining the dentition and providing optimal oral health, will reduce the potential for soft tissue trauma and may allow better nutrition for the patient.
1 Swathi chacham, jakkampudi Nagasravani, Uppin Narayan Reddy, Jillalla Narsing Rao, S. Pratap Rao, Kumar A. S; Epidermolysis bullosa in a small for gestational age preterm male neonate with two affected siblings: A case report. Edorium J 2014; 5(10):690-94.
2 Altaf Naseem, Naila Mazher, E Apoorva, Bismah Gul, Soumia Anumula, wasim Akram, Waseem Khan; A rare case of Epidermolysis bullosa in a fifty day old male child with one affected siblingA case report. Sch J Med Case Rep, September 2015; 3(9B):896-900.
3 Patricia A. Lanier, William R. Posnick, Kevin J. Donly; Epidermolysis bullosadental management and anesthetic consideration: case report. Pediatric dent, 1990; 12(4):246-49.
4 A. P. Javid, Prashanth Shenal, Laxmikanth Chatra, K.M. Veena, Prasanna Kumar Rao, Rachana Prabhu; occurrence of Epidermolysis bullosa along with amylogenesis imperfecta in female patient of India. Dental R J, 2013; 10(6):813-16.
5 Christopher B. Olsen, laurence F. Bourke; Recessive dystrophic Epidermolysis bullosa: two case report with 20 year follow up. Australian Dent J, 1997; 42(1):1-7.
6 Asma AlJobeir; hereditary Epidermolysis bullosa: Report of two cases. Saudi Dent J, 2006; 18(3):156-61.
7 Thais M. Oliveira, Vivian t. Sakay, Liliani A. Candido, Salete M. B. Silva, Maria Aparecida A. M. Machado; clinical manifestation for Epidermolysis bullosa dystrophic. J Appl Oral Sci, 2008; 16(1): 81-85.
8 Katayoun Esfahanizade, Ali Reza Mahdavi, Ghassen Ansari, Masoud Fallahinejad Ghajari, Abdolreza Esfahanizadeh; Epidermolysis bullosa, dental and anesthetic management: A case report. J Dent shiraz Univ Med Sci, September 2014; 15(3):147-52.
9 R Somdeepac Reddy, D Yshrikhande, prashant Nigwekar, Shashank Panwar, Prathmesh Patil; Epidermolysis bullosa in new born: a rare case report. India J of basic and Appl Med Res, June 2014; 3(3):131-34.
10 Sandeep Kulkarni, Sumit Kar, Bhushan Madke, Digambar Dashatuar, Neha Singh; Epidermolysis bullosa destrophica-Acase report. Indian J of Res, 2013;2(3):278-79.
11 Purushotham D. R., Sunil B, Adarsh E, Ragamish K.V, Tamilselvan, Karthik Arigela; Epidermolysis bullosa in a new born: a case report. Sch. J App. Med. Sci., 2014; 2(IB):149-51
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|Publication:||Pakistan Oral and Dental Journal|
|Date:||Dec 31, 2016|
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