Oral health status in children with inherited dystrophic epidermolysis bullosa
Abstract
Background/Aim. Epidermolysis bullosa is a group of rare, genetic connective tissue diseases that cause blisters in the skin and mucosal membranes. The aim of this study was to evaluate the oral health status of patients with epidermolysis bullosa dystrophica and level of knowledge and opinion of parents about the implementation of preventive measaures and quality of dental care of affected children. Methods. This study included a group of 17 patients from Serbia suffering from dystrophic epidermolysis bullosa and matched control group. Dental caries status was assessed using the Klein-Palmer index. Oral hygiene status was verified with oral hygiene indices, simplified plaque index, and calculus index as described by Green and Vermillion. The gingiva was assessed as healthy or inflamed (gingivitis) on the basis of any changes in color, shape, size and surface texture. The condition of oral mucosa has been registered on the basis of inspection of the lips, tongue, a floor of the mouth, mouth vestibule and palate. The level of knowledge and the impressions of parents about the application of preventive measures were investigated through two questionnaires specifically designed for this study. Results. In both dentitions, there was the highest percentage of caries teeth. In primary dentition average value of the modified plaque index was 1.4 ± 1.14 and modified calculus was 0.7 ± 1. On permanent teeth average plaque index was 2 ± 0.4, and average calculus 1.6 ± 0.6. Statistically, significant higher values were found in permanent dentition in percentage distribution of decayed, missing, filled teeth and also for plaque and calculus indices between affected children and the control group. Most common findings on mucosa were microstomia (76.5%) and ankyloglossia (88.2%). Conclusion. The absence of protocol between the treating physician and the dentist and not sufficiently informed parents are leading to inadequate dental care. The implementation of preventive measures is of most importance to decrease the risk of severe complications that are difficult to be managed.
References
Lanschuetzer CM. Definition. In: Fine JD, Hintner H, editors. Life with Epidermolysis Bullosa (EB): Etiology, Diagnosis, Multidisciplinary Care and Therapy.Wien: Springer Verlag; 2009. p. 3−5.
National organization for rare disorders. Rare diseases. In: Association for epidermolysis bullosa “DEBRA”, 1st. Beograd. 2014. p. 103.
Lanschuetzer CM, Fine JD. Classification and molecular basis of hereditary epidermolysis bullosa. In: Fine JD, Hintner H, editors. Life with Epidermolysis Bullosa (EB): Etiology, Diagnosis, Multidisciplinary Care and Therapy. Wien: Springer Verlag; 2009. p. 6−23.
Fine JD, Eady RA, Bauer EA, Bauer JW, Bruckner-Tuderman L, Heagerty A, et al. The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. J Am Acad Dermatol 2008; 58(6): 931−50.
Wright JT, Fine JD, Johnson L. Dental caries risk in hereditary epidermolysis bullosa. Pediatr Dent 1994; 16(6): 427−32.
Fine J, Mellerio JE. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: Part II. Other organs. J Am Acad Dermatol 2009; 61(3): 387−402; quiz 403−4.
Ashton GH. Kindler syndrome. Clin Exp Dermatol 2004; 29(2): 116−21.
Intong L, Murrell F. Inherited epidermolysis bullosa: New diagnostic criteria and classification. Clin Dermatol 2012; 30(1): 70−7.
Fine JD, Mellerio JE. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: Part I. Epithelial associated tissues. J Am Acad Dermatol 2009; 61(3): 367−384.
Wright JT, Fine JD, Johnson L. Hereditary epidermolysis bullosa: Oral manifestations and dental management. Pediatr Dent 1993; 15(4): 242−8.
Krämer S, Serrano MC, Zillmann G, Gálvez P, Araya I, Yanine N, et al. Oral Health Care for Patients with Epidermolysis Bullosa: Best Clinical Practice Guidelines. Int J Paediatr Dent 2012; 22(Suppl1): 1−35.
Azrak B, Kaevel K, Hofmann L, Gleissner C, Willershausen B. Dystrophic epidermolysis bullosa: oral findings and problems. Spec Care Dentist 2006; 26(3): 111−5.
Martin L, Elke N. Intraoral disease. In: Fine JD, Hintner H, editors. Life with Epidermolysis Bullosa (EB) Etiology, Diagnosis, Multidisciplinary Care and Therapy. Wien: Springer-Verlag; 2009. p. 150−66.
World Health Organization. Oral health surveys. Basic methods. 4th ed. Geneva: World Health Organization; 1999.
Wright JT, Fine JD, Johnson LB. Oral soft tissues in hereditary epidermolysis bullosa. Oral Surg Oral Med Oral Pathol 1991; 71(4): 440−6.
Dwivedi S, Mittal M, Vashisth P, Jaiswal D, Arora S. Oral Hygiene Pattern observed in Primary School Children as Reported by Their Mother: A Longitudinal Study. World J Dent 2012; 3(4): 308−12.
Greene J, Vermillion J. The simplified oral hygiene index. J Am Dent Assoc 1964; 68: 7−13.
Siqueira MA, de Souza Silva J, Silva FW, Díaz-Serrano KV, Freitas AC, Queiroz AM. Dental treatment in a patient with epidermolysis bullosa. Spec Care Dentist 2008; 28(3): 92−5.
EB Handbook Austria. Available from: http://www.ebhandbuch.org/fileadmin/media_data/5_EBHandbuch/7_pdf_s_ENGLISCH/Teeth_DDEB.pdf
Tješić-Drinković D. The role of nutrition in epidermolysis bullosa. The second regional symposium on hereditary epidermolysis bullosa. The satellite symposium of the 5th congress of Croatian dermatovenerologist with international participation; Zagreb; 2014 May 7; 2014
Wright T. Epidermolysis bullosa and Oral Health: Problems and solutions. Available from: http://ebnurse. org/assets/files/Dental_Care_by_Tim_Wright_DDS.pdf
Dental health foundation. Available from: http://www.dentalhealth.ie/dentalhealth/teeth/fluoridetoothpastes.htm