Chilblains in immune mediated inflammatory diseases: a review

Shirish Dubey, Nilay Joshi, Olivia Stevenson, Caroline Gordon, John Reynolds

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Chilblains were first described over a hundred years ago as cutaneous inflammatory lesions, typically on the digits, occurring on cold exposure. Chilblains can be primary, or secondary to number of conditions such as infections, including COVID-19, and immune mediated inflammatory disorders (IMIDs) with systemic lupus erythematosus (SLE) being the commonest. Chilblain Lupus Erythematosus (CHLE) was first described in 1888 as cold induced erythematous lesions before the terms ‘chilblains’ or ‘perniosis’ were coined. Diagnostic criteria exist for both chilblains and CHLE. Histopathologically, CHLE lesions show interface dermatitis with perivascular lymphocytic infiltrate. Immunofluorescence demonstrates linear deposits of immunoglobulins and complement in the dermo-epidermal junction. This narrative review focuses on chilblains secondary to immune mediated inflammatory disorders, primarily the epidemiology, pathogenesis and treatment of CHLE.
Original languageEnglish
Article numberkeac231
JournalRheumatology (Oxford, England)
Early online date12 Apr 2022
Publication statusE-pub ahead of print - 12 Apr 2022


  • Chilblains
  • Chilblain Lupus Erythematosus
  • CHLE
  • immune mediated inflammatory diseases
  • IMID


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