Schnitzler syndrome

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What are the features of Schnitzler syndrome?

Patients with Schnitzler syndrome can suffer from a number of different symptoms, listed below. Sometimes these occur together, but they can also alternate. These symptoms persist for years.

Skin lesions

The hallmark of Schnitzler syndrome is a chronic, recurrent urticarial rash (see photograph), which is in most cases the first symptom to occur. Other names for urticaria are ‘hives’ or ‘nettlerash’.

For more information on urticaria and its many possible (and many more frequent) causes see the website of the AAIR, and the website of the International Chronic Urticaria Society.

In the beginning, the rash is usually not itching, but it may become mildly itching. It is most often spread over the trunk, arms and legs. Palms, soles, head and neck areas are usually not affected. Some patients report a daily skin rash, others far less frequently.

Periodic fever

Recurrent fever is the second most common symptom, affecting approximately 90% of patients. The frequency of fever episodes differs greatly among patients, ranging from daily to twice a year. The episodes usually resolve within a few hours, but can persist for up to 24-48 hours. Peaks over 40ºC are common, though chills are rare. The fever and skin rash do not necessarily occur at the same time.

Pain in joints

About 80% of patients suffer from joint pains. This most often affects large joints, including hips, knees, wrists and ankles. In some cases, clear inflammation of joints is present, with swelling, redness and a hot feeling of the joint. But no joint destruction has been reported.

Bone pain

Bone pain has been reported in about 45% of patients, and it typically affects the lower part of the legs. Less frequently the pain may be located in the upper leg, forearm, back or collarbone.

Other symptoms

Many patients with Schnitzler syndrome suffer from fatigue and general malaise. Weight loss is also common. Lymph nodes may be swollen.

Last update/check: June 30, 2007

Skin lesions in Schnitzler syndrome

Information for patients