A Rare but Fascinating Disorder: Case Collection of Patients with Schnitzler Syndrome
Joint Authors
Hariman, Richard
Bashir, Maaman
Bettendorf, Brittany
Source
Issue
Vol. 2018, Issue 2018 (31 Dec. 2018), pp.1-4, 4 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2018-03-08
Country of Publication
Egypt
No. of Pages
4
Main Subjects
Abstract EN
Background.
Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy (IgM in more than 90% of the cases).
It is difficult to distinguish from other neutrophilic urticarial dermatoses, and diagnosis is based on the Strasbourg criteria.
Interleukin-1 is considered the key mediator, and interleukin-1 inhibitors are considered first line treatment.
Here, we present two cases of Schnitzler syndrome, both successfully treated with anakinra.
Objectives.
To increase awareness regarding clinical presentation, diagnosis, and treatment of this rare disorder.
Cases.
We describe the clinical features and disease course of two patients with Schnitzler syndrome, diagnosed using the Strasbourg criteria.
Both were treated with anakinra with remarkable response to therapy.
Conclusion.
Schnitzler syndrome is a rare and underdiagnosed disorder.
High suspicion should be maintained in patients with chronic urticaria-like dermatoses, intermittent fevers, and arthralgias.
A serum protein electrophoresis and immunofixation should be performed in these patients.
The diagnosis is important to recognize as Schnitzler syndrome is associated with malignancy.
A lymphoproliferative disorder develops in about 20% of patients at an average of 7.6 years after onset of symptoms.
Thus, patients warrant long-term follow-up.
IL-1 inhibitors are extremely effective in relieving symptoms and are considered first line therapy.
American Psychological Association (APA)
Bashir, Maaman& Bettendorf, Brittany& Hariman, Richard. 2018. A Rare but Fascinating Disorder: Case Collection of Patients with Schnitzler Syndrome. Case Reports in Rheumatology،Vol. 2018, no. 2018, pp.1-4.
https://search.emarefa.net/detail/BIM-1149877
Modern Language Association (MLA)
Bashir, Maaman…[et al.]. A Rare but Fascinating Disorder: Case Collection of Patients with Schnitzler Syndrome. Case Reports in Rheumatology No. 2018 (2018), pp.1-4.
https://search.emarefa.net/detail/BIM-1149877
American Medical Association (AMA)
Bashir, Maaman& Bettendorf, Brittany& Hariman, Richard. A Rare but Fascinating Disorder: Case Collection of Patients with Schnitzler Syndrome. Case Reports in Rheumatology. 2018. Vol. 2018, no. 2018, pp.1-4.
https://search.emarefa.net/detail/BIM-1149877
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1149877