Schnitzler Syndrome - Unraveling the Mystery of a Complex Condition

Abstract

Introduction: This review paper aims to explore the current state of knowledge regarding the underlying mechanisms of Schnitzler syndrome, its clinical manifestations, the diagnostic challenges, and to analyze current treatment approaches, along with potential complications related to the disease.

Materials and Methods: A comprehensive review of the literature was conducted using the PubMed and Google Scholar databases using the following keywords: "Schnitzler syndrome", "Schnitzler-like syndrome", "NETosis", "urticaria", "fever", "monoclonal gammopathy", "neutrophilic dermatosis", "anakinra", "treatment".

Summary: Schnitzler syndrome is a rare autoinflammatory disease that typically affects adults and presents with symptoms such as recurrent urticaria, fever, monoclonal gammopathy, and musculoskeletal pain. The exact cause is still not fully understood, but IL-1 antagonists have shown promising results, indicating IL-1's role in the disease's development. Despite the established diagnostic criteria, the disease still presents significant diagnostic challenges, often leading to delays in recognition or even underdiagnosis. Schnitzler syndrome, particularly if left untreated, can lead to complications such as the development of lymphoproliferative disorders, most commonly Waldenström's macroglobulinemia or, in rare cases, AA amyloidosis.

Conclusions: This review emphasizes the importance of a thorough examination, as its symptoms often resemble those of other diseases, which can lead to misdiagnosis.  Due to the complexity of the clinical presentation, effective collaboration among dermatologists, rheumatologists and immunologists is essential. Ongoing research is crucial to better understand the disease’s pathogenesis and to develop more effective and personalized treatment strategies.

Keywords: Schnitzler syndrome, neutrophilic urticarial dermatosis, monoclonal gammopathy