Original articleClinical and histopathologic review of Schnitzler syndrome: The Mayo Clinic experience (1972-2011)
Section snippets
Methods
This study was approved by our institutional review board. Patients were evaluated from January 1, 1972, through July 31, 2011.
Diagnosis of Schnitzler syndrome was established on the basis of documentation of an urticarial rash and monoclonal gammopathy (major criteria) plus at least 2 of the following minor criteria: fevers (recurrent or intermittent), arthralgias or arthritis, bone pain, lymphadenopathy, hepatomegaly and/or splenomegaly, elevated erythrocyte sedimentation rate, leukocytosis,
Results
We initially identified a preliminary cohort of 72 patients. Those who did not meet the above diagnostic criteria were excluded. The final cohort included 20 patients (65% male; mean age of 59 years [range, 37-84 years]). All fulfilled the 2 major criteria and 3 to 7 minor criteria (fevers [85%], arthralgias [70%], leukocytosis [70%], increased erythrocyte sedimentation rate [70%], bone pain [50%], lymphadenopathy [40%], and organomegaly [5%]). Sixteen patients (80%) fulfilled at least 3 minor
Discussion
The major diagnostic hallmarks of Schnitzler syndrome are urticarial skin eruptions and monoclonal gammopathy (IgM κ light chain in >90%).1, 3 Recurrence of urticarial lesions varies in frequency. Most commonly involving the trunk and extremities, and sparing the head and neck, the typical eruption consists of rose-colored, slightly elevated papules and wheals that may become confluent (Fig 3).1 Although classically nonpruritic, the urticarial lesions were associated with pruritus in 60% of our
Summary
To our knowledge, this study represents the largest case series of Schnitzler syndrome reported from a single institution. Diagnosis requires a high index of suspicion and consideration of clinical, serologic, and histopathologic data to exclude other diseases in the differential diagnosis. The cutaneous histopathology may be categorized as a NUD but also may resemble common urticaria, with a mild, mainly mononuclear cell infiltrate. Development of a hematologic malignancy is the most serious
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Funding sources: None.
Conflicts of interest: None declared.