Schnitzler Syndrome: It’s Not Always Easy to Pinpoint

Do you have hives (or a rash) that will not go away? And maybe inflammation of your joints, recurrent fevers, and perhaps enlarged lymph nodes?

If you answered yes, you may have Schnitzler Syndrome. And those aren’t even all of the symptoms! This rare disease is caused by a monoclonal IgM gammopathy–or the proliferation of a particular plasma cell that makes M-proteins accumulate in the blood. Schnitzler Syndrome is in the class of autoinflammatory disorders that result from a compromised immune system.

The rash is usually not itchy, but for almost half of those who develop Schnitzler Syndrome, it becomes itchy after a few years. Patients often complain about bone pain, usually occurring in the hips and lower legs. Luckily, this syndrome usually runs its course, but an unlucky few go on to develop cancer, thought to be from the overproduction of white blood cells.

Because symptoms vary widely from patient to patient, diagnosing Schnitzler Syndrome is often difficult. But once a diagnosis is made, patients are treated with non-steroidal anti-inflammatory medications. In more severe cases, various steroidal avenues are pursued.


If you have Schnitzler Syndrome and would like to investigate the possibility of participating in a clinical trial at the NIH Clinical Center in Bethesda, MD, see the information below.

Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]

For information about clinical trials sponsored by private sources, contact:

Has Schnitzler Syndrome affected your life? Share your experience with the Patient Worthy community!

Erica Zahn

Erica Zahn

Erica Zahn is passionate about raising awareness of rare diseases and disorders and helping people connect with the resources that may ease their journey. Erica has been a caregiver, and is a patient, herself, so she completely relates to the rare disease community--on a deeply personal level.

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