Abstract:

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.

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