Schnitzler syndrome

Etiology

• acquired disorder associated with MGUS

Epidemiology

• 1% among patients with MGUS

Pathology

• rash with neutrophilic infiltrate

Clinical manifestations

• IgM monoclonal gammopathy
• chronic urticaria
• fever (75%)
• fatigue (75%)
• arthralgia (68%), bone pain (63%)
  • daily attacks of arthritis & bone pain
• night sweats (25%)
• lymphadenophathy (44%)
• hepatosplenomegaly
• peripheral neuropathy (54%)

* a long delay between the onset of symptoms & diagnosis, often up to 5 years, is common

Laboratory

• no diagnostic tests*
• complete blood count (CBC)
  • leukocytosis
• serum protein electrophoresis
  • IgM-kappa monoclonal gammopathy (94%)

Complications

• development of lymphoproliferative disease is rare
• may evolve into Waldenstrom's macroglobulinemia

Management

• interleukin 1-beta inhibitor
  • rilonacept
  • canakinumab (Ilaris)
  • anakira

More general terms

• autoimmune disease
• syndrome