What is Schnitzler syndrome

Schnitzler syndrome is a rare autoinflammatory disorder characterized by chronic urticaria (hives) and monoclonal gammopathy (abnormal protein in the blood). It was first described by Dr. Liliane Schnitzler in 1972. The syndrome primarily affects adults, with symptoms typically appearing in middle age.

The key features of Schnitzler syndrome include:

• Chronic Urticaria: Persistent and recurring hives that can be itchy and painful.


• Monoclonal Gammopathy: Abnormal production of a specific type of protein (monoclonal IgM) by plasma cells in the bone marrow.


• Systemic Inflammation: Patients may experience fever, joint and bone pain, fatigue, enlarged lymph nodes, and organ inflammation.


• Other Symptoms: Some individuals may develop bone abnormalities, weight loss, muscle inflammation, and increased risk of infections.

Diagnosis of Schnitzler syndrome requires the presence of chronic urticaria along with at least two additional major criteria. Treatment often involves the use of anti-inflammatory medications, such as nonsteroidal anti-inflammatory drugs (NSAIDs) or corticosteroids, to manage symptoms. Additionally, targeted therapies like interleukin-1 inhibitors have shown promising results in controlling inflammation in some cases.