Update on cutaneous lupus erythematosus pathogenesis, diagnosis and management

Cutaneous lupus erythematosus (CLE) encompasses a spectrum of skin manifestations that may occur as an isolated dermatological condition or in association with systemic lupus erythematosus (SLE). This review provides an updated synthesis of current knowledge on CLE, including its classification, pathogenesis, clinical presentation and diagnostic strategy. It will discuss recent advances in pathogenesis, particularly the central role of type I interferons and interferon-producing cells. Diagnostic evaluation involves clinical assessment, histopathology and immunological testing, with specific focus on CLE mimickers. In recent years, validated tools such as the Cutaneous Lupus Area and Severity Index (CLASI) have facilitated standardized assessment in clinical trials. Management of CLE requires a multifaceted approach incorporating general measures, topical therapies, antimalarials, systemic immunosuppressants and emerging biologics. Treatment should be tailored based on CLE subtype, severity, scarring risk and the presence or absence of systemic involvement. Antimalarials, particularly hydroxychloroquine, remain the cornerstone of systemic therapy. Second-line or third-line agents such as methotrexate, retinoids, dapsone, thalidomide and lenalidomide are recommended in refractory cases. Biological therapies, including belimumab and anifrolumab, are approved in the setting of SLE. Promising results from recent trials of targeted therapies including inhibitors of plasmacytoid dendritic cells, TLR7/8 and TYK2 are paving the way for novel treatment strategies in CLE.