Schnitzler syndrome: clinical features and histopathology

Schnitzler syndrome is a rare and underrecognized syndrome characterized by chronic urticaria, a monoclonal protein, and a variety of other symptoms, including fever, bone pain, organomegaly, and evidence of an acute phase response. Biopsy of an involved area of the skin shows a neutrophilic infiltr...

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Bibliographic Details
Published inPathology and Laboratory Medicine International Vol. 7; no. default; pp. 39 - 46
Main Authors Dingli, David, Camilleri, Michael J
Format Journal Article Book Review
LanguageEnglish
Published Macclesfield Dove Medical Press Limited 01.01.2015
Taylor & Francis Ltd
Dove Medical Press
Subjects
Analysis
Anemia
Asthma
Biopsy
Bone marrow
Chronic illnesses
Cytokines
Fever
Histology, Pathological
Laboratories
Lymphatic system
Multiple myeloma
Pain
Patients
Peripheral neuropathy
Proteins
Risk factors
Urticaria
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Summary:Schnitzler syndrome is a rare and underrecognized syndrome characterized by chronic urticaria, a monoclonal protein, and a variety of other symptoms, including fever, bone pain, organomegaly, and evidence of an acute phase response. Biopsy of an involved area of the skin shows a neutrophilic infiltrate without evidence of vasculitis or hemorrhage. Although the etiology of the syndrome is unknown, current evidence suggests this is an autoinflammatory syndrome. Recognition of this syndrome is critical since it is highly responsive to anakinra. Keywords: neutrophilic urticarial dermatosis, autoinflammatory syndrome, monoclonal gammopathy, neutrophilic dermatosis, anakinra
ISSN:1179-2698
1179-2698
DOI:10.2147/PLMI.S69414