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.
Patient Groups
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
