Background: Behçet’s disease (BD) is a rare chronic auto-inflammatory systemic disease with non-specific oral manifestations, categorised as generalised variable vessel vasculitis that requires an interdisciplinary approach to diagnose due to its phenotypic heterogeneity. Whilst the oral lesions that reoccur in BD underpin the complex diagnostic process, the crucial role of dental professionals is highlighted in a case report summarised herein. We present a case of a 47-year-old male referred to the Oral Medicine Department by a rheumatologist after previous hospitalization for thrombosis of the iliac vein and inferior vena cava. He had elevated inflammatory C-reactive protein biomarker and an increased erythrocyte sedimentation rate. Recurrent episodes of folliculitis, oral and genital ulcers were reported. Clinical examination revealed multiple ulcerations in the oral mucosa. The complementary, histopathological analysis performed to rule out other disorders, based on excisional biopsy, showed non-keratinised stratified squamous epithelium with areas of exocytosis and ulceration. The connective tissue presented an intense mixed inflammatory infiltrate, congested blood vessels, haemorrhage, vasculitis, and HLA-B genotyping identified the expression of HLA-B15, further supporting the BD diagnosis. Treatment was initiated with colchicine, prednisolone, and weekly subcutaneous administration of methotrexate and resulted in the complete remission of oral lesions and no recurrence of other manifestations. Conclusions: This BD case report emphasizes the importance of a multidisciplinary approach in diagnosing BD, including the use of histopathological assessment and genetic profiling. It highlights the significance of thorough intraoral assessment and referral to a multidisciplinary team for diagnosis. The oral manifestations of BD as the primary symptoms often indicate underlying major systemic pathologies. The authors stress the need for a structured diagnostic algorithm to facilitate timely and effective management of BD.

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