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Acta Dermatovenerologica Croatica, Vol. 28 No. 1, 2020.

Review article

Incomplete Schnitzler Syndrome

Mattias A.S. Henning ; Department of Dermatology, Zealand University Hospital, Faculty of Health and Medical Sciences, University of Copenhagen, Denmark
Gregor B.E. Jemec ; Department of Dermatology, Zealand University Hospital, Faculty of Health and Medical Sciences, University of Copenhagen, Denmark
Kristina S. Ibler ; Department of Dermatology, Zealand University Hospital, Faculty of Health and Medical Sciences, University of Copenhagen, Denmark

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Abstract

Schnitzler syndrome (SS) is a rare autoinflammatory disease that
presents with chronic urticaria and monoclonal immunoglobulin (Ig) M or G,
accompanied by fever, abnormal bone remodeling, skin biopsy with a neutrophilic
dermal infiltrate, leukocytosis, or elevated C-reactive protein. It is usually
refractory to antihistamines and immunosuppression. We present a case report
of clinical SS without monoclonal Ig with robust response to interleukin-1 inhibitor
anakinra. This suggests the possible existence of an incomplete form of
SS and underlines the risk of false negative diagnosis in individuals with such
“incomplete SS”.

Keywords

diagnosis, interleukin 1 receptor antagonist protein, Schnitzler syndrome, urticaria

Hrčak ID:

246800

URI

https://hrcak.srce.hr/246800

Publication date:

18.1.2020.

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