SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferences

View all Images (11)

Chilblain lupus erythematosus
Other Resources UpToDate PubMed

Chilblain lupus erythematosus

Contributors: Regina Parker, Susan Burgin MD
Other Resources UpToDate PubMed


Chilblain lupus erythematosus (chilblain LE) is a subtype of chronic cutaneous lupus erythematosus (CCLE). Others in this group include discoid lupus erythematosus (DLE), lupus erythematosus tumidus (LE tumidus), and lupus profundus (lupus panniculitis).

Chilblain LE is characterized by circumscribed, dusky red papules and plaques on the dorsal and lateral aspects of the hands and feet, including the fingers and toes. Involvement of the heels, nose, and ears may also occur. The lesions may be itchy, painful, or asymptomatic and are precipitated by exposure to cold, damp conditions. Photosensitivity is not observed.

Most patients with chilblain LE have immunologic abnormalities, including polyclonal hypergammaglobulinemia, elevated rheumatoid factor, and/or antinuclear antibodies, in particular anti-Ro/SSA.

Chilblain LE may be sporadic or familial. Sporadic chilblain LE most commonly occurs in middle-aged women. This variant has been reported in association with anorexia, intestinal lymphoma, and pregnancy. An estimated 20% of cases of sporadic chilblain LE progress to systemic LE. The pathogenesis of sporadic chilblain LE has been related to vasoconstriction or microvascular injury secondary to cold exposure, as well as hyperviscosity and stasis secondary to immunologic abnormalities. A rarer form of familial chilblain LE may manifest within the first years of life. This form of the disease is associated with autosomal dominant, heterozygous mutations of the TREX1 gene, which encodes a 3-prime repair DNA exonuclease with high specificity for single-stranded DNA. The sporadic and familial forms of chilblain LE are clinically and histologically indistinguishable, despite their differing pathogenetic mechanisms.


L93.2 – Other local lupus erythematosus

238928005 – Chilblain lupus erythematosus

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

  • Idiopathic Chilblains (chilblains) – Lymphocytic vasculitis is seen on histopathology without vacuolar interface changes.
  • Pseudo-chilblains of COVID-19 infection
  • Acrocyanosis
  • Raynaud phenomenon – Presents as reversible white or blue discoloration of the distal digits triggered by cold exposure or stress.
  • Type 1 Cryoglobulinemia
  • Cryofibrinogenemia
  • Cholesterol emboli – Associated with a recent history of vascular intervention or thrombolytic therapy. Most often affects the toes and feet.
  • Thrombophilia (Protein C deficiency and Protein S deficiency, Antithrombin deficiency, prothrombin G202120A mutation, factor V Leiden deficiency) – May present with retiform purpura that may involve the distal digits.

Best Tests

Subscription Required

Management Pearls

Subscription Required


Subscription Required

Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

Subscription Required


Subscription Required

Last Reviewed:07/06/2020
Last Updated:01/12/2022
Copyright © 2024 VisualDx®. All rights reserved.
Chilblain lupus erythematosus
A medical illustration showing key findings of Chilblain lupus erythematosus : ANA positive, Hypergammaglobulinemia, Painful skin lesions
Copyright © 2024 VisualDx®. All rights reserved.