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Schnitzler Syndrome

Pic d'aspect monoclonal en gamma Igm kappa


It was defined by Dr. Liliane Schnitzler, a French dermatologist in 1972 as the occurrence in adulthood of urticaria, fatigue, bone and/or joint pain and the discovery in the blood of an excess protein called gammopathy. monoclonal.


It preferentially affects people over 50 and is cosmopolitan: all countries in the world can be affected.


There is no genetic mutation found to date in Schnitzler syndrome.


Patients present with fatigue associated with hives-like rash, bone and joint pain.


The diagnosis is based on a clinical presumption, the exclusion of differential diagnoses (in particular hematological and infectious) and the presence of a monoclonal gammopathy associated with systemic symptoms such as fever, arthromyalgia, skin lesions such as urticaria, in an inflammatory setting. Diagnostic criteria have been defined under the name of Strasbourg criteria.


Evolution is not predictable. Some patients may present with a progression to a lymphoproliferative haematological disease.


The treatment is not codified to date, but may be based on analgesics and anti-Interleukin 1 biotherapies.

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