Snapshot A 35-year-old Middle Eastern man presents to his dermatologist for painful oral and genital ulcers. He reports that he also is fatigued, has joint pain and stiffness in the morning. He is currently sexually active with two women but reports that all parties had sexually transmitted infection testing prior to engaging in any sexual activities. Physical exam reveals multiple aphthous ulcers and genital ulcers. He also has palpable purpura on his lower extremities that is concerning for vasculitis. He is sent for additional laboratory workup and given topical corticosteroids and colchicine. Introduction Clinical definition a systemic small and large vessel vasculitis Epidemiology incidence most common in Asia, Mediterranean regions, and the Middle East demographics male > female 20-40 years of age risk factors geographic area family history Pathogenesis thought to be an autoimmune process that is triggered by an environmental or infectious source, especially in a genetically predisposed person mechanism involves increased cytokines, proliferation of T-cells, and hyperactive neutrophils Associated conditions erythema nodosum Presentation Symptoms arthritis enthesitis can have vascular thrombosis or aneurysms from vasculitis Physical exam recurrent oral aphthous ulcers genital ulcers bilateral ocular lesions slit lamp testing nongranulomatous uveitis anterior uveitis skin lesions papules and pustules acneiform nodules erythema nodosum cutaneous ulcers pathergy is common hyper-reactivity of the skin that occurs in response to minimal trauma formation of skin lesions and ulcers cranial nerve palsies may occur Studies Labs ↑ inflammatory markers erythrocyte sedimentation rate C-reactive protein leukocytes autoantibodies typically absent Biopsy indications used to confirm diagnosis can differentiate Behcet syndrome from Crohn disease findings leukocytoclastic vasculitis or lymphocytic vasculitis thrombosis Making the diagnosis most cases are clinically diagnosed Differential Herpes genitalis Syphilis Inflammatory bowel disease Treatment Management approach based on affected organs and discussion of side effects with patients Medical topical corticosteroids indication isolated oral or genital ulcers colchicine indications erythema nodosum arthritis other systemic immunosuppression indications other organ involvement, including ocular, gastrointestinal, and neurologic drugs corticosteroids cyclosporine infliximab cyclophosphamide azathioprine Complications Blindness from ocular disease Thrombosis