Schnitzler Syndrome Clinical Presentation

Updated: Mar 12, 2019
  • Author: Brian J Thomas, MD; Chief Editor: Dirk M Elston, MD  more...
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All patients with Schnitzler syndrome present with a chronic, recurrent, urticarial eruption. Pruritus is usually absent at the disease onset, but lesions may become mildly pruritic in approximately 45% of patients after 3-4 years. The skin eruption is usually the first symptom to occur, primarily affecting the trunk and the extremities, and sparing the palms, soles, and head and neck areas

Approximately 90% of Schnitzler syndrome patients experience recurrent fevers. Each febrile episode usually resolves within a few hours; however, fevers can persist for up to 24-48 hours. Episodes may occur daily or as infrequently as twice per year. Chills are rare. In some cases, the fever and rash are not related.

Concurrent with the fever, 80% of patients report relapsing arthralgias, 70% report bone pain (usually in the large joints), and myalgias. The bone pain mostly affects the iliac bone and the tibia. The femur, spine, forearms, and clavicle are less often involved. Additionally, fatigue and weight loss occur in a high percentage of patients.


Physical Examination

The urticarial rash of Schnitzler syndrome consists of pale-rose, slightly elevated papules and plaques. Individual lesions are 0.5-3 cm in diameter. New lesions appear daily. They last 12-24 hours and then disappear without sequelae. Angioedema is possible but is very rare. Lymphadenopathy may be found up to 50% of patients, hepatomegaly in 30% of patients, and splenomegaly in 10% of patients. Other signs and symptoms in Schnitzler syndrome include the following:

  • Recurrent fever

  • Arthralgia or arthritis

  • Bone pain

  • Lymphadenopathy

  • Hepatomegaly or splenomegaly

  • Leukocytosis

  • Elevated erythrocyte sedimentation rate (ESR)

  • Bone abnormalities (upon radiologic or histopathologic investigations)



Roughly 15-20% of patients develop an overt lymphoproliferative disorder. [14]

With Schnitzler syndrome, a lymphoplasmacytic malignancy, such as Waldenström macroglobulinemia, lymphoplasmacytic lymphoma, or IgM myeloma, may occur.

The development of AA amyloidosis is a concern in untreated patients with Schnitzler syndrome. [14]