Chilblain Lupus Erythematosus

  • Min Ae Lee-Kirsch
  • Martin AringerEmail author
  • Annegret Kuhn


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.


Systemic Lupus Erythematosus Cutaneous Lupus Erythematosus Nucleic Acid Metabolism Chilblain Lupus Cell Cycle Checkpoint Activation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



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


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Copyright information

© Springer Science+Business Media, LLC 2014

Authors and Affiliations

  • Min Ae Lee-Kirsch
    • 1
  • Martin Aringer
    • 2
    Email author
  • Annegret Kuhn
    • 3
  1. 1.Children’s HospitalUniversity Medical Centre Carl Gustav Carus at the Technical University of DresdenDresdenGermany
  2. 2.Division of Rheumatology, Department of Medicine IIIUniversity Medical Centre Carl Gustav Carus at the Technical University of DresdenDresdenGermany
  3. 3.Department of DermatologyUniversity of MuensterMuensterGermany

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