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Schnitzler Syndrome - Causes, Diagnosis and Treatment

Published on Mar 05, 2022   -  4 min read


Schnitzler syndrome is a rare inflammatory disorder consisting of various signs and symptoms. A detailed description regarding its causes, diagnosis, and treatment is given below.



Schnitzler syndrome is a systemic disorder affecting the adult population and is more common among men. It is found to occur at the age of 51 years. A French dermatologist Liliane Schnitzler was the first person to report the various symptoms of this syndrome in 1972. Therefore this disease was named after him.

What Is Schnitzler Syndrome?

Schnitzler syndrome is associated with different signs and symptoms like skin rash, fever, bone pain, joint pain, and inflammation. It also has other characteristic signs like enlarged lymph nodes or organs, increased production of a specific protein called IgM in the blood, along with other abnormal blood findings. In a few patients, Schnitzler syndrome can progress to other lymphoproliferative disorders (abnormal and uncontrolled production of lymphocytes) like B-cell lymphoma.

What Causes Schnitzler Syndrome?

The actual cause of Schnitzler syndrome remains unknown. Some researchers have found that alterations in the level of cytokine (a specialized protein that helps in immune response) in the blood are associated with the occurrence of Schnitzler syndrome. A cytokine called interleukin-1 alpha (IL-1α) is found in increased amounts in patients with this syndrome responsible for the inflammatory symptoms. Gene mutations (changes in gene patterns), specifically in the gene NLRP3, cause Schnitzler syndrome in a few patients.

What Are the Clinical Features?

Schnitzler syndrome is a chronic disorder that consists of the following signs and symptoms:

What Are the Radiological Findings?

Radiography shows increased bone formation (hyperostosis) thickening of the bone's outer portion (cortex) in the lower leg, thigh, and other symptomatic areas.

How Is Schnitzler Syndrome Diagnosed?

What Are the Differential Diagnoses?

How Do You Treat Schnitzler Syndrome?


The symptoms do not progress to severe disorders in most patients, and it is not a life-threatening disorder. Its signs and symptoms may seem troublesome, but it shows a good prognosis when diagnosed and treated correctly.

Frequently Asked Questions


Can We Cure Schnitzler Syndrome?

Yes, Schnitzler syndrome can be cured entirely. In mild cases, NSAIDs (non-steroidal anti-inflammatory drugs) are used as the first line of treatment. In more severe cases, Schnitzler syndrome is successfully treated with Anakinra.


Does Schnitzler Syndrome Come Under Autoimmune Diseases?

Yes, Schnitzler syndrome is an autoimmune-autoinflammatory syndrome.


Is Schnitzler Syndrome Prevalent?

No, Schnitzler syndrome is not a very common syndrome. Only about 160 people are known to be affected by this disease throughout medical history.


How to Diagnose Schnitzler Syndrome?

Schnitzler syndrome can be diagnosed with a proper clinical evaluation and history taking. The physician looks at the rashes, and the characteristic features like urticarial rashes, fever, joint pains, abnormal bone morphologies show the presence of Schnitzler syndrome. Specific tests like erythrocytes sedimentation rate (ESR) will be elevated.


Is Schnitzler Syndrome a Hereditary Disorder?

Schnitzler syndrome is an autoimmune-autoinflammatory condition with no proof to be hereditary.


How Does a Person Get Affected by Schnitzler Syndrome?

The accurate cause of Schnitzler syndrome is unknown. A particular type of immunoglobulin called IgM is produced more in persons affected by Schnitzler syndrome. Abnormal amounts of IgM immunoglobulin could be a cause of Schnitzler syndrome. Therefore this condition is known as an autoimmune-autoinflammatory disease.


What Does a Schnitzler Syndrome Rash Look Like?

The urticarial rash, which is red rashes that resemble hives, is the typical rash seen in patients with Schnitzler syndrome.

Last reviewed at:
05 Mar 2022  -  4 min read




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