Schnitzler syndrome is a very rare idiopathic disease characterized above all by: chronic urticaria, monoclonal gammopathy (immunoglobulin M or G types) and proinflammatory state of
the organism. Moreover, it should be considered in differential diagnosis of fever of unknown origin, chronic disease anemia, pains of the musculoskeletal system as well as lymphadenopathy. Due to recurring nature of inflammatory response of the organism, predisposition for excessive stimulated interleukin-1β secretion and certain similarities with
cryopyrinopathies (neutrophilic urticarial dermatosis in skin biopsy, almost universal treatment efficacy of interleukin-1 receptor antagonist – anakinra, perhaps also NLRP3 gene mutation), Schnitzler syndrome is being increasingly classified as an acquired autoinflammatory disorder. In our work, we stress a multidisciplinary approach to Schnitzler syndrome from dermatologist’s, hematologist’s, oncologist’s, internist’s, rheumatologist’s, immunologist’s and pathophysiologist’s points of view. Regarding potentially life-threatening complications
however (risks of transformation into a lymphoproliferation and development of secondary amyloidosis), we aim to address as many medical specialties as possible. With this objective in mind, we have added to the article an illustrated supplementary material including clinical findings, results from imaging methods and laboratory tests as well as a histopathological examination of a skin biopsy.