This article presents a case of a 39-year-old woman with spontaneous lip swelling with gingival erythema. The case report discusses orofacial granulomatosis, a condition that often poses diagnostic and management challenges. Additionally, the article emphasizes the importance of establishing a definitive diagnosis of this disease, which has similar clinical and histological features to other oral and systemic conditions. Finally, a successful management approach is discussed that uses combination therapy consisting of intralesional steroid injections and oral hydroxychloroquine.

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