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Chilblain Lupus Erythematosus

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Skin Manifestations in Rheumatic Disease

Abstract

Chilblain lupus is a typical, albeit rare, form of chronic cutaneous lupus erythematosus. With its name derived from similarities to chilblains (perniones), these painful, bluish-red acral manifestations usually do not pose major diagnostic difficulties, with differential diagnoses including true perniones, lupus pernio (sarcoidosis), and ischemia. However, recent findings, linking familial chilblain lupus to TREX1, and thus the innate immune system, have had significant impact on our understanding of the disease. Therefore, this chapter will not only review the clinical side, but also our current understanding of the pathogenic events preceding disease.

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Acknowledgment

We thank P. Wissel, J. BĂĽckmann, and Professor T. A. Luger, University Muenster, Germany, for providing the clinical figures.

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Authors and Affiliations

  1. Children’s Hospital, University Medical Centre Carl Gustav Carus at the Technical University of Dresden, Fetscherstrasse 74, 01037, Dresden, Germany

    Min Ae Lee-Kirsch M.D.

  2. Division of Rheumatology, Department of Medicine III, University Medical Centre Carl Gustav Carus at the Technical University of Dresden, Fetscherstrasse 74, 01037, Dresden, Germany

    Martin Aringer M.D.

  3. Department of Dermatology, University of Muenster, Von-Esmarch-Strasse 58, D-48149, Muenster, Germany

    Annegret Kuhn M.D.

Authors
  1. Min Ae Lee-Kirsch M.D.
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  2. Martin Aringer M.D.
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  3. Annegret Kuhn M.D.
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Corresponding author

Correspondence to Martin Aringer M.D. .

Editor information

Editors and Affiliations

  1. Division of Rheumatology, University of Florence Department of Biomedicine, Florence, Italy

    Marco Matucci-Cerinic

  2. Division of Rheumatology, UCLA Medical Center Department of Medicine, Los Angeles, California, USA

    Daniel Furst

  3. Department of Dermatology, Stanford University School of Medicine, Redwood City, California, USA

    David Fiorentino

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Lee-Kirsch, M.A., Aringer, M., Kuhn, A. (2014). Chilblain Lupus Erythematosus. In: Matucci-Cerinic, M., Furst, D., Fiorentino, D. (eds) Skin Manifestations in Rheumatic Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-7849-2_2

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  • DOI: https://doi.org/10.1007/978-1-4614-7849-2_2

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-7848-5

  • Online ISBN: 978-1-4614-7849-2

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