(chronic urticaria with gammapathy, chronic urticaria with macroglobulinemia)

Rare. Autoinflammatory pathology probably caused by a dysfunction of the inflammasomes. It often affects a man in his fifties. The diagnosis is often delayed. It is characterized by:

-        rash:  pink or red non itchy macules or plaques all over the body which disappear within 24 hours; biopsy shows a neutrophil infiltration of the dermis without vasculitis

-        recurrent episodes of fever (90 %)

-        bone (70 %) ( osteocondensing injuries and hyperostosis) or joint (large joints) pain

-        lymph nodes (45 %)

-        sometimes hepato-splenomegaly (30 %) or thrombocytosis (50 %)

-        monoclonal IgM.

Risk: appearance of a Waldenström disease, of a lymphoma or a  myeloma with IgM (risk of 15 %  at 10 years).

Many treatments have been attempted: the administration of anakinra, an IL-1 receptor antagonist, is the only effective one. Daily subcutaneous injection.

Anesthetic implications:

check the total blood count, do not interrupt the treatment with anakinra.

References : 

-         Henry B, Néel A, Barbarot S, Masseau A, Hamidou M. 
Le syndrome de Schnitzler. 
La Revue de Médecine interne. 2013 ; 34 : 224-9.

Updated: September 2018