Çocukluk Çağı Behçet Hastalığında Mukoza ve Cilt Tutulumu

Yıl 2022, Cilt: 44 Sayı: 3, 323 - 329, 14.03.2022

Zahide Ekici Tekin , Elif Çelikel , Fatma Aydın , Tuba Kurt , Müge Sezer , Nilüfer Tekgöz , Cüneyt Karagöl , Serkan Coşkun , Melike Kaplan , Banu Acar

https://doi.org/10.20515/otd.994094

Öz

Behçet hastalığı, her tip ve boyuttaki damarları etkileyerek çoklu organ tutulumu ile seyreden kronik, otoinflamatuar bir vaskülittir. Her sistemi etkileyebilse de hastalık için tanı koydurucu ve karakteristik olan tutulum mukoza ve cilttir.Bu çalışma ile çocukluk çağı Behçet hastalığı tanısı ile merkezimizde takip edilen hastaların mukokutonöz tutulum oranlarının, tutulum şiddetlerinin, tedavi yanıtlarının bildirilmesi ve diğer organ tutulumları arasındaki ilişkinin ortaya konulması hedeflenmiştir. Retrospektif olarak düzenlenen bu çalışma Ocak 2007 ile Haziran 2021 tarihleri arasında 16 yaşından önce Behçet hastalığı tanısı almış olguları içermektedir. Hastaların elektronik dosyalarından demografik, klinik özellik ve tedavileri not edildi. Mukokutanöz tutulumları detaylı şekilde gruplandırıldı. Çalışmaya dahil edilen 79 hastanın %57’si kızdı. Tekrarlayan oral aft tüm hastalarda (%100), genital ülser 57 hastada (%72,7) ve kutanöz tutulum 37 hastada (%46,8) tespit edildi. Diğer organlarda etkilenme olmadan izole mukokutanöz tutulum 22 hastada (%27,8) gözlendi. Kutanöz tutulum, 30 hastada (%38) papülopüstüler lezyon, 8 hastada (%10,1) eritema nodozum benzeri lezyon, 4 hastada (%5,1) folikülit ve 2 hastada (%2,5) yüzeyel tromboflebit şeklindeydi. Paterji testi uygulanan 73 hastanın (%92,4) 22’sinde (%27,8) pozitifti. Çalışmamızda majör ROA ve majör GÜ sıklığı kızlarda daha fazla görüldü (p=0,059, p=0,046). Mukokutanöz tutulum için lokal tedavilere ek olarak tüm hastalara kolşisin başlandı. Çocukluk çağı Behçet hastalığı mukokutanöz bulguları ile ilgili olan çalışmamızda tekrarlayan oral aft, genital ülser ve cilt bulguları en sık görülen 3 bulgudur. Majör tekrarlayan oral aft ve genital ülser kız hastalarda daha sık gözlemlendi. Mukokutanöz bulgular Behçet hastalığında tanıya giden yolda ilk basamak olup dikkatle değerlendirilmelidir.

Anahtar Kelimeler

Çocukluk çağı Behçet hastalığı, deri, genital ülser, oral aft, tedavi

Destekleyen Kurum

yok

Proje Numarası

yok

Mucosal and Cutaneous Involvement of Behcet’s Disease in Children

Öz

Behçet's disease is a chronic, autoinflammatory vasculitis with multi-organ involvement affecting vessels of all types and sizes. Although it can affect any system, the diagnostic and characteristic involvement of the disease is mucosa and skin.In this study, it was aimed to show the rate, severity and treatment response of mucocutaneous involvement of children who were diagnosed Behçet's disease in our center. This retrospective study included cases diagnosed with Behçet disease before the age of 16 between January 2007 and June 2021. Demographic, clinical features and treatments were noted from the electronic files of the patients. Mucocutaneous involvements patients were evaluated in detail. Of the 79 patients included in the study 57% were girls. Recurrent oral aphthae were detected in all patients (100%), genital ulcers in 57 patients (72.7%), and cutaneous involvement in 37 patients (46.8%). Isolated mucocutaneous involvement was observed in 22 patients (27.8%) without other organ involvement. Cutaneous involvements were papulopustular lesion in 30 patients (38%), erythema nodosum-like lesion in 8 patients (10.1%), folliculitis in 4 patients (5.1%), and superficial thrombophlebitis in 2 patients (2.5%). Pathergy test was positive in 22 (27.8%) of 73 patients (92.4%). In present study, the frequency of major recurrent oral aphthae and major genitale ulceration was higher in girls (p=0.059, p=0.046). Colchicine was started in all patients in addition to local treatments for mucocutaneous involvement. In our study, recurrent oral aphthae, genital ulcer and skin lesions were the most common involvements respectively. Major recurrent oral aphtha and genital ulcer were observed more frequently in female patients. As a conclution, mucocutaneous lesions are the early step of diagnosing Behçet's disease and should be evaluated carefully.

Anahtar Kelimeler

Skin; childhood Behçet

Proje Numarası

yok

Kaynakça

• 1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculit-ides. Arthritis Rheum. 2013; 65: 1-11.
• 2. Yildiz M, Koker O, Adrovic A, et al. Pediatric Be¬hçet’s disease - clinical aspects and current concepts. Eur J Rheumatol. 2019; 7: 1-10.
• 3. Yurdakul S, Hamuryudan V, Yazici H. Behçet syndrome. Curr Opin Rheumatol. 2004;16(1):38-42. doi: 10.1097/00002281-200401000-00008.
• 4. Kone-Paut I (2016) Behcet’s disease in children, an overview. Pediatr Rheumatol. 14(1):10. https ://doi.org/10.1186/s12969-016-0070-z.
• 5. Criteria for diagnosis of Behcet’s disease. Inter-national Study Group for Behcet’s Disease. Lancet. 1990; 335: 1078-80.
• 6. The International Criteria for Behcet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014; 28: 338-47.
• 7. Kone-Paut I, Shahram F, Darce-Bello M, et al. Consensus classification criteria for paediatric Behcet’s disease from a prospective observational co-hort: PEDBD. Ann Rheum Dis. 2016; 75: 958-64.
• 8. Lawton G, Bhakta BB, Chamberlain MA, et al. The Behcet’s disease activity index. Rheumatology. 2004;43:73-78.
• 9. Fabiani C, Vitale A, Orlando I, et al. Quality of life impairment in Behçet's disease and relationship with disease activity: a prospective study. Intern Emerg Med. 2017;12(7):947-955. doi: 10.1007/s11739-017-1691-z. Epub 2017 Jun 15. PMID: 28620840.
• 10. Gezgin Yıldırım D, Bakkaloğlu SA, Hasanreisoglu M, et al. Disease activity and outcomes in juvenile Behçet's disease: 10 years' experience of a single centre. Clin Exp Rheumatol. 2020;38:105-111.
• 11. Butbul Aviel Y, Batu ED, Sözeri B, et al. Characteristics of pediatric Behçet's disease in Turkey and Israel: A cross-sectional cohort comparison. Semin Arthritis Rheum. 2020;50:515-520. doi: 10.1016/j.semarthrit.2020.01.013.
• 12. Atmaca L, Boyvat A, Yalcindag FN, et al. Behçet disease in children. Ocul Immunol Inflamm. 2011;19:103-7.
• 13. Sungur GK, Hazirolan D, Yalvac I, et al. Clinical and demographic evaluation of Behçet disease among different paediatric age groups. Br J Ophthalmol. 2009;93:83-7.
• 14. Hamzaoui A, Jaziri F, Ben Salem T, et al. Comparison of clinical features of Behçet disease according to age in a Tunisian cohort. Acta Med Iran. 2014;52:748-51.
• 15. Nanthapisal S, Klein NI, Ambrose N, et al. Paediatric Behçet’s disease: a UK tertiary centre expe¬rience. Clin Rheumatol. 2016;35:2509-16.
• 16. Gallizzi R, Pidone C, Cantarini L, et al. A national cohort study on pediatric Behcet’s disease: cross-sectional data from an Italian registry. Pediatr Rheumatol Online J. 2017;15:84.
• 17. Shahram F, Nadji A, Akhlaghi M, et al. Paediatric Behçet's disease in Iran: report of 204 cases. Clin Exp Rheumatol. 2018;36:135-140. Epub 2018 Jun 19. PMID: 29998839.
• 18. Bang D, Yoon KH, Chong HO, et al. Epidemiological and clinical features of Behçet’s disease in Korea. Yonsei Med J. 1997; 38:428-36. doi: 10.3349/ymj.1997.38.6.428.
• 19. Nakamura K, Tsunemi Y, Kaneko F, et al. Mucocutaneous Manifestations of Behçet's Disease. Front Med. 2021;7:613432. doi: 10.3389/fmed.2020.613432. PMID: 33598466; PMCID: PMC7882475.
• 20. Hatemi G, Christensen R, Bang D, et al. 2018 update of the EULAR recommendations for the management of Behcet’s syndrome. Ann Rheum Dis. 2018; 77:808-18.