Development of Behçet’s disease on maintenance therapy for autoimmune hepatitis

Behçet's disease is a clinical diagnosis with variable presentations. Liver involvement is rare in the absence of vascular complications. We describe a patient diagnosed with Hashimoto's thyroiditis and autoimmune hepatitis on azathioprine who developed extensive aphthous ulcers approximately 10 years later. She was initially diagnosed with an overlap autoimmune connective tissue disorder in the setting of a positive anti-Ro antibody. Symptoms did not resolve with prednisone, hydroxychloroquine or mycophenolate mofetil. She subsequently developed pseudofolliculitis, erythema nodosum-like lesions, large cutaneous ulcers and pathergy phenomenon at sites of venipuncture consistent with Behçet's disease. She was successfully treated with infliximab, azathioprine, colchicine and apremilast. This case represents an unusual sequence of autoimmune conditions and a diagnostic challenge.