Schnitzler Syndrome

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Acronym:
SCHNITZLER

SAID group:
Schnitzler Syndrome

Gene:
Currently unknown.

Inheritance:
Unknown.

Ethnicity:
Affects all races, but most cases are in Europe. [13] More men than women are affected.

Frequency:
Unknown. Over 150 known cases, mostly in Europe. [13]

Timing of symptoms:
12-36 hours. Rash is present first. Intermittent fevers often occur separately from the rash. [13]

Age of onset:
Most cases start in middle age, at over 35-50 years old. The youngest patient was 13 yrs old. Symptoms start with the rash. [13]

Skin cutaneous:
Maculopapular rash and plaques (sometimes itchy) on the chest and limbs. Dermis has neutrophillic infiltrate. Dermographism. [13]

Neurologic:
Intermittent fevers can rise >40°C. Chills are uncommon. Fatigue and headaches are common with fevers. Temperature changes (weather), stress and/or exercise can trigger flares. [13]

Auditory:
Uncommon. [13]

Ophthalamic:
Not noted. [13]

Cardiopulmonary:
Not noted. [13]

Abdominal:
GI symptoms are uncommon. Hepatomegaly, splenomegaly or hepatosplenomegaly is common. [13]

Lymphatic:
<20% with lymphoma, IgM myeloma, or Waldenströms macroglobulinemia (aka lymphoplasmacytic lymphoma). >45% with lymphadenopathy. Splenomegaly. [13]

Joints bones muscles cartilage:
80% have muscle, bone and/or joint pain, or arthritis. Bone pain is most common in the iliac and tibia. <40% have bone lesions. Some with osteocondensation and sclerotic bone marrow involvement in the legs. [13]

Vasculitis:
Vasculitis noted in 20% of patients. [13]

Amyloidosis:
A few patients have developed secondary amyloidosis. [13]

Abnormal labs:
Monoclonal IgM and/or IgG gammopathy. High: ESR, CRP. Leukocytosis. Complement normal to elevated. 50% with inflammatory anemia. [13]

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