pci-32765 and Schnitzler-Syndrome

Schnitzler syndrome is a rare adult-onset acquired autoinflammatory disorder typically characterized by chronic urticarial rash and immunoglobulin M (IgM) (rarely IgG) monoclonal gammopathy. Its clinical symptoms usually respond well to interleukin-1 blockade therapy, which, however, does not impact the underlying monoclonal gammopathy. Herein, we described a female patient who presented with urticarial rash, recurrent fevers, and fatigue for 7 years. Laboratory investigations revealed IgMκ monoclonal protein and

Topics: Adenine; Adult; Antibodies, Monoclonal; Exanthema; Female; Humans; Piperidines; Schnitzler Syndrome; Urticaria

Topics: Adenine; Female; Humans; Interleukin-1; Lymphoma; Middle Aged; Piperidines; Schnitzler Syndrome

Topics: Adenine; Agammaglobulinaemia Tyrosine Kinase; Humans; Immunoglobulin M; Inflammasomes; NLR Family, Pyrin Domain-Containing 3 Protein; Piperidines; Protein Kinase Inhibitors; Schnitzler Syndrome

Topics: Adenine; Aged, 80 and over; Exanthema; Histocytochemistry; Humans; Male; Piperidines; Protein Kinase Inhibitors; Pyrazoles; Pyrimidines; Schnitzler Syndrome; Skin; Treatment Outcome