Schnitzler’s Syndrome: Monoclonal Gammopathy Associated with Chronic Urticaria

Abstract

Schnitzler’s syndrome (SS) is defined by monoclonal gammopathy and chronic urticaria combined with at least two of the following features: fever, arthralgia or arthritis, bone pain, hepato- and/or splenomegaly, palpable lymph nodes, elevated ESR, and leukocytosis. We report a 49-year-old man with monoclonal IgM gammopathy and a 4-year history of recurrent urticarial rash, unexplained fever and arthralgias. The skin biopsy from an acute lesion revealed perivascular lymphocytic infiltrates consisting of CD4+ and CD8+ T lymphocytes. To our knowledge, this is the first report of an immunophenotypic characterization of skin infiltrates in SS. A lower CD4+/CD8+ ratio of circulating T lymphocytes was also detected. SS usually has a benign course, but in 15% of patients a lymphoproliferative disorder develops.

Narrative Review: The Systemic Capillary Leak Syndrome

“…Purified monoclonal paraprotein from 5 SCLS patients failed to bind cultured endothelial cells or induce any cytotoxicity (12, 52–53). Although the amount of monoclonal Ig produced in MGUS is usually small, indicating few clonal plasma cells, it may produce a toxic monoclonal paraprotein that causes pathology either by aggregating and depositing in tissue, such as in light chain amyloidosis, or by binding to autologous common antigens (i.e., acting as an autoantibody) as in cold agglutinin disease, mixed cryoglobulinemia, vonWillebrand syndrome, paraproteinemic neuropathy, and Schnitzler syndrome (54-57). …”

Cancer Risk in Patients With Chronic Urticaria

“…26 Most of these patients present with gammopathy and hematologic disorder in their 40s to 60s after 4 to 9 years of chronic urticaria. [27][28][29] To our knowledge, no previous studies have examined the effect of age at onset of chronic urticaria on cancer occurrence. Our study demonstrated that younger patients (aged Ͻ40 years) tend to have the greatest risk of cancer development, especially for hematologic cancers.…”

Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria

“…133 Schnitzler syndrome usually has a benign course and patients show no increased mortality during the follow-up periods, but there is a 10-year 15% risk of developing a lymphoproliferative disorder, most notably Waldenström macroglobulinemia. 133,134 Skin manifestations of Schnitzler syndrome include persistent urticarial lesions, frequently with figurate lesions, leaving a brown hyperpigmentation. Histologic examination reveals a perivascular mixed inflammatory infiltrate with leukocytoclasis, nuclear dust without fibrinoid necrosis, and extravasated red blood cells.…”