Incomplete Schnitzler Syndrome

Mattias A S Henning; Gregor B E Jemec; Kristina S Ibler

Incomplete Schnitzler Syndrome

Mattias A S Henning
, Gregor B E Jemec
, Kristina S Ibler

Dermatology

Research output: Contribution to journal › Article › Research › peer-review

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".

Original language	English
Pages (from-to)	38-40
Number of pages	3
Journal	Acta Dermatovenerologica Croatica
Volume	28
Issue number	1
Publication status	Published - Jul 2020

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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{"}.",

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AU - Henning, Mattias A S

AU - Jemec, Gregor B E

AU - Ibler, Kristina S

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N2 - 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".

AB - 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".

M3 - Article

C2 - 32650850

SN - 1330-027X

VL - 28

SP - 38

EP - 40

JO - Acta Dermatovenerologica Croatica

JF - Acta Dermatovenerologica Croatica

IS - 1

ER -

Incomplete Schnitzler Syndrome

Abstract

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