Schnitzler syndrome

The classic feature of Schnitzler syndrome is the chronic, urticarial rash that does not generally present as itchy (pruritic), but can progress to being more pruritic. The rash generally is present on the torso, arms and legs, but is not often seen on the head, neck, palms of the hands, or soles of the feet. The rash may be seen daily, or only a few times a year.

At least 90% of patients can have recurrent fevers that can present daily, or only a few times a year. The fevers often can be over 40ºC, and can last for 1-2 days. Chills are not common, and the rash does NOT always occur along with the fever.

Fevers are often accompanied by headaches and extreme fatigue. Changes in temperature (xold weather, weather changes, or even going from the heat to a cold, air-conditioned room), stress and/or exercise can trigger flares.

Many patients have joint pains, especially in the hips, knees, wrist and ankles. Some patients may have arthritis, but joint destruction has not been reported. Less than half of the patients with this disease have bone pain, usually in the tibia (one of the lower leg bones) and the iliac bone. Patients suffer from felling ill, and fatigues, and can experience weight loss. Around 45% of patients may have enlarged lymph nodes, enlarged liver and/or spleen.

Of note, Schnitzler Syndrome is one of the few autoinflammatory diseases with a notable risk for some forms of cancer, which includes: a <20% risk of lymphoma, IgM myeloma, or Waldenströms macroglobulinemia (lymphoplasmacytic lymphoma). It is important for doctors to monitor labs and symptoms for these conditions.

For more information about diagnostic critieria, please go to our medically edited page about Schnitzler Syndrome on our comprehensive database of autoinflammatory diseases at autoinflammatory-search.org.

Schnitzler Syndrome was featured in the New York Times on February 14, 2018 in this great article by Dr Lisa Sanders MD, "Her Various Symptoms Seemed Unrelated. Then One Doctor Put It All Together.".