Dear Editor, Cutaneous lupus erythematosus (CLE) comprises various subtypes with a shared autoimmune basis and cytokine profile. CLE variants include acute CLE (ACLE), subacute CLE (SCLE) and chronic CLE (CCLE), which encompass discoid lupus erythematosus (DLE), chilblain lupus erythematosus (CHLE) and mucosal discoid lupus erythematosus (MDLE). Most CLE are exclusively cutaneous, but up to 70% of systemic lupus erythematosus (SLE) cases develop CLE. 1 CCLE significantly impacts quality of life due to potential scarring and dyspigmentation. Currently, there are few approved CLE-specific treatments. Most therapies, including immunosuppressants, antimalarials, thalidomide and lenalidomide, were originally developed for SLE and are used off-label. 2 Anifrolumab, an anti-IFN-α receptor subunit 1 (IFNAR1) monoclonal antibody, gained EMA (European Medicines Agency) approval in February 2022 for moderate to severe SLE. Secondary end points from trials of anifrolumab for SLE have demonstrated important benefits in CLE, achieving a 50% CLASI-A (Cutaneous Lupus Erythematosus Disease Area and Severity Index Activity) score reduction in 49% of patients, compared to 25% with placebo. 3 Since then, real-world evidence further supports its efficacy in the literature. 1,2,[4][5][6][7][8][9][10][11][12] We present a challenging CHLE case that exhibited rapid response to anifrolumab, along with a literature review on its real-life effectiveness.A 19-year-old Caucasian female presented with a history of paediatric-onset SLE characterized by lymphopenia and positive antinuclear autoimmunity (ANA), antiribonucleoprotein (anti-RNP) and anticardiolipin antibodies. Cryoglobulins and TREX1 mutation tests were negative. The patient experienced significant oral aphthosis and CHLE manifestations (Figure 1a), severely affecting acral areas of her fingers, hands, upper limbs, nose, ears and lips (CLASI-A score of 33, severe disease). The patient had previously undergone treatments with systemic corticosteroids, methotrexate, azathioprine, doxycycline, apremilast, colchicine, dapsone, hydroxychloroquine, mepacrine, nifedipine, sulfasalazine and tofacitinib. In response to the refractory nature of her condition, she began receiving intravenous anifrolumab 300 mg every 4 weeks, resulting in a complete response (CLASI-A score of 1) after the first infusion. No adverse effects were observed (Figure 1b).