The Schnitzler syndrome: Chronic urticaria and monoclonal gammopathy – an autoinflammatory syndrome?

Abstract

Summary Schnitzler syndrome describes the simultaneous occurrence of monoclonal gammopathy and chronic urticaria with at least two additional minor symptoms (arthralgia, bone pain, fever of uncertain origin, hepato‐ or splenomegaly, lymphadenopathy, increased erythrocyte sedimentation rate, leukocytosis/thrombocytosis or increased bone density). Schnitzler syndrome is not wellknown and very likely under‐recognized. Comprehensive diagnostic examinations are necessary to rule out other diseases, especially those of hematologic origin. Close interdisciplinary collaboration is mandatory. The etiology of Schnitzler syndrome is unclear, but the rapid response to the interleukin‐1 receptor inhibitor anakinra underlines the pivotal role which the proinflammatory cytokine interleukin‐1 may play in the pathophysiology of this potentially autoinflammatory disorder.

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

“…Cutaneous findings include either angioedematous and urticarial lesions, or erythematous, pruritic papules and nodules d Gleich syndrome is also characterized by recurrent episodes of angioedema and urticaria, eosinophilia, elevated serum IgM, fever, and increased body weight; however, it is not associated with internal organ damage Schnitzler syndrome is a rare and often misdiagnosed disease that is characterized by the simultaneous occurrence of monoclonal IgM gammopathy and chronic urticaria with at least two additional minor symptoms: arthralgia, bone pain, fever of uncertain origin, hepato-or splenomegaly, lymphadenopathy, increased erythrocyte sedimentation rate, leukocytosis/thrombocytosis, and increased bone density. 132,133 Monoclonal gammopathy can be detectable even some years after the onset of the cutaneous manifestations. No spontaneous remissions have been reported to date.…”

Urticarial Syndromes

“…An urticarial rash and usually IgM but rarely IgG monoclonal gammopathy are present with a chronic patern in all the patients. Recurrent fever, bone or joint pain, increased bone density, hepato-or splenomegaly, lymphadenopathy, and elevated acute-phase reactants are also accepted as minor criteria [58]. Approximately, 300 cases have been identiied in the literature [57].…”

“…The most commonly developing lymphoproliferative disease is Waldenstrom macroglobulinemia. Treatment is usually unsatisfactory, but high doses of corticosteroids, systemic antihistamines, oral cyclosporine, intravenous pulse cyclophosphamide, and peloxacin mesylate are the therapeutic agents used [58].…”

Autoinflammatorische Syndrome/Fiebersyndrome

“…Zusätzlich kommen Fieberschübe, Hepatosplenomegalie, Lymphadenopathie sowie Gelenk-und Knochenschmerzen vor. Die Hauptkomplikation besteht in der Entwicklung sekundärer hämatologischer Malignome wie lymphoproliferativer Erkrankungen oder der Makroglobulinämie Waldenström [37].…”