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

Pregledni rad

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

Puni tekst: engleski pdf 103 Kb

str. 38-40

preuzimanja: 356

citiraj

Sažetak

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”.

Ključne riječi

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

Hrčak ID:

246800

URI

https://hrcak.srce.hr/246800

Datum izdavanja:

18.1.2020.

Posjeta: 668 *