: Chilblain lupus erythematosus is a rare, cold-induced form of chronic cutaneous lupus that can occur in both genetic and sporadic forms. It is characterized by acral skin lesions and is commonly associated with autoimmunity and type I interferon pathway activation. Diagnosis is based on clinical features and histological findings. While topical and systemic therapies can be effective, the disease is often chronic and relapsing. We describe a case of a 48-year-old woman with chilblain lupus erythematosus who achieved almost complete clinical remission following treatment with hydroxychloroquine and methotrexate.