Büllozalı Çocukların Klinik Bulguları: Üç Kardeş Vaka Çalışması
Epidermolizis bülloza, (EB) deri ve mukozada travmayakarşı cevap olarak meydana gelen bül oluşumuyla karakterizeailesel geçişli bir vezikülobüllöz hastalıktır. EB hastalarınındental tedavilerinde kullanılan aletlerin yumuşak doku lezyonuoluşturması riski yüksektir ve özel önlem gerektirmektedir. Buvaka raporunda, ebeveynleri akraba olan, EB’lı üç kardeşin klinikve oral bulguları sunuldu. Birinci vaka deri lezyonları, mikrostomi,ankiloglossi ve kötü ağız hijyenine sahip 5 yaşındaki bir kızçocuğu idi. İkinci vaka olan 4 yaşındaki kız çocuğunda sadeceayak tırnağında deformite mevcuttu. Üçüncü vaka, deride çoksayıda kabarcığı bulunan ankiloglossi ve kötü ağız sağlığına sahip2 yaşındaki bir erkek çocuk idi. Bu çocukların ağız sağlığı, oralhijyenin sağlanması, koruyucu uygulamalar ve düzenli kontrollerile idame edildi.
Anahtar Kelimeler:
Epidermolizis büllosa, Ağız sağlığı, Oral bulgular
Clinical Findings of Children With Epidermolysis Bullosa: Case Reports of Three Siblings
Epidermolysis bullosa (EB) is a group of inherited bullousdisorders characterized by blistering of the skin and mucosa inresponse to trauma. EB patients require special precautions duringdental treatment because of the high risk of lesioning the softtissue when handling cutting instruments close to the skin andoral mucosa In this case report, clinical and oral findings of threesiblings with epidermolysis bullosa, whose parents were relatives,are presented. The first case was a 5-year-old girl with skin lesions,microstomia, anklyoglossia and poor oral health. The second casewas a 4-year-old girl having only deformity on her foot fingernailwithout any oral tissue involvement. The third case was a 2-yearoldboy with numerous dermal blisters, ankyloglossia and poordental status. Oral health of these children was maintained byimproving oral hygiene, preventive measures and regular visits.
Keywords:
Epidermolysis bullosa, oral health, oral manifestations,
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- Silva IG, Alves JMA, Mattos LMBB: Epidermolysis bullosa. HU Rev. 2000;26:221-227.
- Trent JT, Kirsner RS: Epidermolysis bullosa: identification and treatment. Adv Skin Wound Care. 2003;16:284-290
- Williams EF, Gannon K, Soon K: The experiences of young people with epidermolysis bullosa simplex: a qualitative study. J Health Psychol 2011;16:701-710.
- Komorowski L, Müller R, Vorobyev A, Probst C, Recke A, Jonkman MF, Hashimoto T, Kim SC, Groves R, Ludwig RJ, Zillikens D, Stöcker W, Schmidt E: Sensitive and specific assays for routine serological diagnosis of epidermolysis bullosa acquisita. J Am Acad Dermatol 2013;68:e89-95.
- Fine JD: Inherited epidermolysis bullosa. Orphanet J Rare Dis 2010;5:12-28.
- Kho YC1, Rhodes LM, Robertson SJ, Su J, Varigos G, Robertson I, Hogan P, Orchard D, Murrell DF: Epidemiology of epidermolysis bullosa in the antipodes: the Australasian Epidermolysis Bullosa Registry with a focus on Herlitz junctional epidermolysis bullosa. Arch Dermatol 2010;146:635-640.
- Browne F, Heagerty AHM, Martinez A, Mellerio J, Moss C: The epidemiology of epidermolysis bullosa in the U.K.: a 9 – year study. Br J Dermatol 2011;165(Suppl 1): 11.
- Reichart PA, Schmidt-Westhausen AM, Khongkhunthian P, Strietzel FP: Dental implants in patients with oral mucosal diseases – a systematic review. J Oral Rehabil 2016;43:388- 399.
- Prabhu VR, Rekka P, Swathi S: Dental and anesthetic management of a child with epidermolysis bullosa. J Indian Soc Pedod Prev Dent 2011;29:155-160.
- Pacheco W, Marques de Sousa Araugio R: Orthodontic treatment of a patient with recessive dystrophic epidermolysis bullosa: a case report. Spec Care Dentist 2008;28:136-139.
- Fine JD, Eady RA, Bauer EA, Briggaman RA, Bruckner- Tuderman L, Christiano A, Heagerty A, Hintner H, Jonkman MF, McGrath J, McGuire J, Moshell A, Shimizu H, Tadini G, Uitto J: Revised classification system for inherited epidermolysis bullosa: Report of the Second International Consensus Meeting on diagnosis and classification of epidermolysis bullosa. J Am Acad Dermatol 2000;42:1051-1066.
- Fortuna G, Aria M, Cepeda-Valdes R, Trevino MGM, Salas- Alanís JC: Pain in patients with dystrophic epidermolysis ullosa: Association with anxiety and depression. Psychiatry Investig 2017;14:746-753.
- Al-Abadi A, Al-Azri SA, Bakathir A, Al-Riyami Y, Qaboos S: Dental and anaesthetic challenges in a patient with dystrophic epidermolysis bullosa, University Med J 2016; 16:e495-499.
- Varki R, Sadowski S, Pfendner E, Uitto J: Epidermolysis bullosa. I. Molecular genetics of the junctional and hemidesmosomal variants. J Med Genet 2006;43:641-652.
- Fine JD, Bruckner-Tuderman L, Eady RA, Bauer EA, Bauer JW, Has C, Heagerty A, Hintner H, Hovnanian A, Jonkman MF, Leigh I, Marinkovich MP, Martinez AE, McGrath JA, Mellerio JE, Moss C, Murrell DF, Shimizu H, Uitto J, Woodley D, Zambruno G: Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification. J Am Acad Dermatol 2014;70:1103-1126.
- Pekiner FN, Yucelten D, Ozbayrak S, Sezen EC: Oralclinical findings and management of epidermolysis bullosa. J Clin Pediatr Dent 2005;30:59-65.
- Krämer SM, Serrano MC, Zillmann G, Gálvez P, Araya I, Yanine N, Carrasco-Labra A, Oliva P, Brignardello- Petersen R, Villanueva J: Oral health care for patients with epidermolysis bullosa-best clinical practice guidelines . Int J Paediatr Dent 2012;22(Suppl 1):1-35.
- Siqueira MA, DE Souza Silva J, Silva FW, Diaz-Serrano KV, Freitas AC, Queiroz AM: Dental treatment in a patient with epidermolysis bullosa. Spec Care Dentist 2008;28:92-95.
- Esfahanizade K, Mahdavi AR, Ansari G, Fallahinejad Ghajari M, Esfahanizadeh A: Epidermolysis bullosa, dental and anesthetic management: a case report. J Dent (Shiraz) 2014;15:147-152.
- Harris JC, Bryan RA, Lucas VS, Roberts GJ: Dental disease and caries related microflora in children with dystrophic epidermolysis bullosa. Pediatr Dent 2001;23:438-443.
- Mello BZ, Neto NL, Kobayashi TY, Mello MB, Ambrosio EC, Yaedú RY, Machado MA, Oliveira TM: General anesthesia for dental care management of a patient with epidermolysis bullosa: 24-month follow-up. Spec Care Dentist 2016;36:237-240.
- Galeotti A, D’Anto V, Gentile T, Galanakis A, Giancristoforo S, Uomo R, Romeo U: Er:YAG laser dental treatment of pateints affected by epidermolysis bullosa. Case Rep Den. 2014;2014:421783.
- Silva LC, Cruz RA, Abou-Id LR, Brini LN, Moreira LS: Clinical evaluation of patients with epidermolysis bullosa: review of the literature and case reports. Spec Care Dentist 2004;24:22-27.
- Scheidt L, Sanabe ME, Diniz MB: Oral manifestations and dental management of epidermolysis bullosa simplex. Int J Clin Pediatr Dent 2015;8:239-241.
- Intong LR, Murrell DF: Inherited epidermolysis bullosa: new diagnostic criteria and classification. Clin Dermatol 2012;30:70-77.
- Santos KKD, Difabio LFG, Santos MTBR, Soares Junior LAV: Effectiveness of oral lubricants in patients with epidermolysis bullosa. RGO 2011;59:209-213.
- Babaee N, Zabihi E, Mohseni S, Moghadamnia AA: Evaluation of the therapeutic effects of Aloe vera gel on minor recurrent aphthous stomatitis. Dent Res J 2012;9:381- 385.
- Habeeb F, Shakir E, Bradbury F, Cameron P, Taravati MR, Drummond AJ, Gray AI, Ferro VA: Screening methods used to determine the anti-microbial properties of Aloe vera inner gel. Methods 2007;42:315-320.
- Kummer TR, Nagano HC, Tavares SS, Santos BZ, Miranda C. Oral manifestations and challenges in dental treatment of epidermolysis bullosa dystrophica. J Dent Child 2013;80:97-100.
- Başlangıç: 2015
- Yayıncı: Marmara Üniversitesi
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