Book review

A Modern Look at Schnitzler Syndrome – A Literature Review

Krzysztof Kanabaj orcid.org/0000-0001-8383-1181 ; Medical University of Gdansk, Department of Dermatology, Venereology and Allergology, Gdańsk, Poland
Barbara Bulińska orcid.org/0009-0002-6510-4444 ; Medical University of Gdansk, Department of Dermatology, Venereology and Allergology, Gdańsk, Poland *
Małgorzata Sokołowska-Wojdyło ; Medical University of Gdansk, Department of Dermatology, Venereology and Allergology, Gdańsk, Poland

* Corresponding author.

Abstract

Schnitzler syndrome (SS) is an extremely rare acquired systemic disease
that shares many similarities with various hereditary autoinflammatory

syndromes. It presents as chronic non-pruritic urticarial rash, mono-
clonal gammopathy, and systemic symptoms, such as recurrent fever,

arthralgia, myalgia, bone pain, bone lesions, and enlargement of the
spleen and liver. The specific feature associated with SS is its spectacular
response to treatment using anti-interleukin-1 (anti-IL-1) agents, such as
anakinra or canakinumab. If it remains untreated, the disease can have
a devastating effect on the patient’s quality of life as well as increased
mortality due to systemic complications. Herein, we will summarize the
most recent findings in the pathogenesis, diagnosis, and management
of SS.

Keywords

Schnitzler syndrome; chronic urticaria; autoinflammatory syndromes

Hrčak ID:

334834

URI

https://hrcak.srce.hr/334834

Publication date:

20.6.2024.

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