| Summary: | Orofacial granulomatosis (OFG) describes a disease entity with recurrent or persistent edema within the orofacial region. Microscopically, non-necrotizing granulomas form in the affected tissues in the absence of any recognized systemic disease. The etiologies of OFG are still debatable and poorly understood. Hypotheses are abundant as to the pathogenesis, which include genetic causes, allergic reactions to food or dental materials, infectious agents, and immunologic dysfunction. OFG also accounts for two other disease processes in a spectrum of nonspecific granulomatous diseases. Management of OFG is often challenging as there exists no singular medication which has been tested or approved for the treatment of the disease. We present an unusual case successfully treated with azithromycin pulse therapy, a literature review on the recent therapeutic options and an algorithm for the treatment of OFG.
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