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

Çocukluk Çağı Behçet Hastalığında Mukoza ve Cilt Tutulumu

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

Kaynakça

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