A 69-year-old woman with periodic fever, facial swelling, and neck pain.

We presented a case of a 69-year-old woman who experienced monthly episodes of facial swelling and nonpruritic, erythematous rash on her face, accompanied by high fever, nausea, headache, and neck pain over 1 year. Her symptoms started with myalgia, arthralgia, fever and neck stiffness, and headache, and then angioedema occurred, which was painful to touch. She underwent multiple iatrogenic diagnostic and therapeutic procedures that did not lead to the correct diagnosis. Subsequently, relevant immunology laboratory tests were conducted after a careful history and physical examination, which led to the diagnosis. This case illustrated the need for a detailed history and thorough immunologic assessment, and the requirement to maintain a broad differential diagnosis.