Bechet’s Disease in Absence of Oral Aphthosis: Utility of Updated Criteria

Abstract

Background : Behçet disease (BD) is a chronic, relapsing, systemic vasculitis of unknown etiology with the clinical features of oral and genital ulcers, cutaneous vasculitic lesions, ocular, articular, vascular, gastrointestinal, neurologic, urogenital and cardiac involvement. Mucocutaneous lesions are considered hallmarks of the disease, and often precede other manifestations. Therefore, their recognition may permit earlier diagnosis and treatment with beneficial results for prognosis. Oral aphthosis is the main manifestation of BD and was reported in 97.3% of patients. Objective : In a case study we investigate the prevalence, demographic and clinical parameters of Behçet cases without oral aphthosis, and the interest of the new ICBD criteria. Methodology: In a retrospective study carried out in the internal medicine department of the university hospital Ibn Rochd de casablanca over an 18-year period, involving 563 patients with BD selected according to the new criteria proposed by the ICBD, we study the different characteristics of Behçet cases without oral aphthosis. Results: Among 563 BD patients, 9 patients (1.6%) had no oral aphthosis. The sex ratio was 3.5 men/women. The mean age of onset was 28.3 years. Initial manifestations included genital aphthosis in (77.7%), ocular involvement (11.1%), and vascular involvement (11 .1%). During the course of the disease, 66.6% of patients had pseudofolliculitis lesions, and 22.2% had a positive pathergy test. Joint involvement was present in 1/3 of patients. 6 patients had ocular involvement. Vascular involvement was present in 66.6% of cases. Pleuropulmonary and central neurological parenchymal involvement were observed in only 1 patient. Therapeutic measures included bolus and oral corticosteroids, azathioprine and cyclophosphamide, in addition to colchicine. Progression was good in 66.66% of patients, with therapeutic resistance in 11.1%, and the onset of AA amyloidosis in 1 patient. Conclusion: Bechet's disease is mainly diagnosed on the basis of clinical signs and symptoms, which often leads to delays in diagnosis. Revision of the criteria has improved sensitivity by making oral aphthosis a non-mandatory criterion, enabling diagnosis at an early stage and thus improving prognosis.