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Schnitzler syndrome

Evidence: Unconfirmed

Names

Schnitzler’s syndrome
Chronic urticaria with gammapathy
Chronic urticaria with macroglobulinemia
Chronic urticaria with gammopathy

Description

Schnitzler syndrome is a disease in which periodic episodes of inflammation occur. It is considered an autoinflammatory disease, not an autoimmune disease, because the immune system does not mistakenly attack healthy cells. Instead, Schnitzler syndrome is believed to arise from a problem with the immune system itself. It is uncertain what causes Schnitzler syndrome, but it does not appear to be hereditary.

Patients with Schnitzler syndrome typically experience chronic rash, relapsing fevers, pain and inflammation in the joints, enlarged lymph nodes, and an excess of certain proteins in the blood.  The condition is chronic, generally without worsening, though 10-15% of people with Schnitzler syndrome experience cancerous or non-cancerous overproduction of certain types of white blood cells. There is no cure, but treatments may relieve symptoms in some individuals. 

Bloggers

None found.

Prevalence

 US Cases: Few than 300 cases reported [de Koning 2014].

Typical Age of onset

The mean age of disease onset is 51 years old.

Symptoms

  • Red rash with raised patches of skin (urticaria) on the trunk, arms, and legs

  • Recurrent fevers

  • Enlarged internal organs such as the liver, lymph nodes and/or spleen

  • Blood abnormalities

  • Joint pain and inflammation

  • Fatigue

  • Weight loss

  • Bone pain

  • Muscle ache