The New England Journal of MedicineChilblain Lupus Erythematosus

Cold-Induced Inflammation: Chilblain Lupus Erythematosus as a Rare Manifestation of Systemic Lupus Erythematosus

A case report published in a recent medical journal details the presentation and management of chilblain lupus erythematosus, an uncommon variant of cutaneous lupus erythematosus. The case involves a 29-year-old woman with systemic lupus erythematosus who developed distinctive skin lesions following cold exposure. This report, with images of the condition, highlights the clinical features, diagnostic approach, and treatment strategies for this rare condition, offering valuable insights for dermatologists and rheumatologists.

Key Points:

• Patient: 29-year-old woman with systemic lupus erythematosus
• Presenting symptoms: 2-week history of itchy, painful rash on nose and hands
• Trigger: Cold weather exposure; no sun exposure reported
• Physical examination findings:
  • Erythematous macules and papules with punched-out ulcers on the nose
  • Scattered papules on the palms
  • Edematous erythrocyanosis of fingertips with lateral ulcerations
• Laboratory results:
  • Positive for antinuclear antibodies, anti–double-stranded DNA antibodies, rheumatoid factor, anti-Ro antibodies, and antiphospholipid antibodies
  • Hypergammaglobulinemia present
  • Negative cryoglobulin and cold agglutinin tests
• Histopathology: Vacuolar interface dermatitis and perivascular lymphocytic infiltrate
• Diagnosis: Chilblain lupus erythematosus
• Treatment:
  • Glucocorticoids
  • Azathioprine
  • Hydroxychloroquine
  • Nifedipine
  • Cold avoidance counseling
• Outcome: Skin lesions abated at 2-week follow-up

HCN Medical Memo
Chilblain lupus erythematosus is a rare but important differential diagnosis in patients with systemic lupus erythematosus presenting with cold-induced skin lesions. Early recognition and appropriate management, including cold avoidance and targeted therapies, can lead to rapid improvement in symptoms.

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