A 48-year-old man with typical features of Schnitzler Syndrome is presented, followed by a discussion of the pathogenesis and clinical aspects of this rare disease. Some of the challenges we and others have faced in diagnosing and treating this disease are also discussed. Clinical pearls and pitfalls are emphasized to aid clinicians from varying specialties in recognizing this syndrome and providing appropriate therapy. Schnitzler Syndrome is associated with high morbidity and increased risk of lymphoproliferative disorders. Accurate diagnosis is vital in restoring quality of life and ensuring appropriate long-term monitoring.
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