How not to miss autoinflammatory diseases masquerading as urticaria

Authors

  • K. Krause,

    1. Autoinflammation Reference Center Charité, Charité-Universitätsmedizin Berlin, Berlin, Germany
    2. Department of Dermatology and Allergy, Charité-Universitätsmedizin Berlin, Berlin, Germany
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  • C. E. Grattan,

    1. St John's Institute of Dermatology, St Thomas' Hospital, London, UK
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  • C. Bindslev-Jensen,

    1. Department of Dermatology and Allergy Center, Odense University Hospital, Odense, Denmark
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  • M. Gattorno,

    1. UO Pediatria II, G. Gaslini Institute, Genova, Italy
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  • T. Kallinich,

    1. Autoinflammation Reference Center Charité, Charité-Universitätsmedizin Berlin, Berlin, Germany
    2. Pediatric Pneumology and Immunology, Charité-Universitätsmedizin Berlin, Berlin, Germany
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  • H. D. de Koning,

    1. Department of General Internal Medicine, Nijmegen Institute for Infection, Inflammation and Immunology (N4i), Centre for Immunodeficiency and Autoinflammation (NCIA), Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    2. Department of Dermatology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
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  • H. J. Lachmann,

    1. National Amyloidosis Centre, University College London Medical School, London, UK
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  • D. Lipsker,

    1. Faculté de Médecine, Hôpitaux universitaires de Strasbourg, Université de Strasbourg et Clinique Dermatologique, Strasbourg, France
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  • A. A. Navarini,

    1. Department of Dermatology, University Hospital of Zurich, Zurich, Switzerland
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  • A. Simon,

    1. Department of General Internal Medicine, Nijmegen Institute for Infection, Inflammation and Immunology (N4i), Centre for Immunodeficiency and Autoinflammation (NCIA), Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
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  • C. Traidl-Hoffmann,

    1. Department of Dermatology and Allergy Biederstein, Technische Universität, Munich, Germany
    2. ZAUM – Center for Allergy and Environment, Technische Universität Munich, Helmholtz Center Munich, Munich, Germany
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  • M. Maurer

    Corresponding author
    1. Department of Dermatology and Allergy, Charité-Universitätsmedizin Berlin, Berlin, Germany
    • Autoinflammation Reference Center Charité, Charité-Universitätsmedizin Berlin, Berlin, Germany
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  • Edited by: Hans-Uwe Simon

Correspondence

Marcus Maurer, Department of Dermatology and Allergy, Charité – Universitätsmedizin Berlin, Charitéplatz 1, D-10117 Berlin, Germany.

Tel.: +49 30 450 518 043

Fax: +49 30 450 518 972

E-mail: marcus.maurer@charite.de

Abstract

Urticarial skin reactions are one of the most frequent problems seen by allergists and clinical immunologists in daily practice. The most common reason for recurrent wheals is spontaneous urticaria. There are, however, several less common diseases that present with urticarial rash, such as urticarial vasculitis and autoinflammatory disorders. The latter include cryopyrin-associated periodic syndrome and Schnitzler's syndrome, both rare and disabling conditions mediated by increased interleukin-1 secretion. Apart from the urticarial rash, patients are suffering from a variety of systemic symptoms including recurrent fever attacks, arthralgia or arthritis and fatigue. Autoinflammatory diseases are often associated with a diagnostic delay of many years and do not respond to antihistamines and other treatments of urticaria. Also, the chronic inflammation may lead to long-term complications such as amyloidosis. It is therefore important not to miss these diseases when diagnosing and treating patients with chronic recurrent urticarial rash. Here, we present clinical clues and tips that can help to identify autoinflammatory disorders in patients presenting with chronic urticarial rash and discuss their clinical picture and management.

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