SummaryCutaneous lupus erythematosus can manifest with or without systemic involvement resulting in a broad clinical spectrum. Disease pathogenesis is often characterized by a loss of tolerance to endogenous antigens and a chronic relapsing activation of the innate and adaptive immune system. Research in recent years has expanded our pathogenic understanding of the disease. However, therapeutic options remain limited. Biologics directed against BLyS or the type I interferon receptor can be used for patients with cutaneous involvement and systemic lupus erythematosus, sometimes with an excellent response.Clinical trials are difficult to conduct due to the symptomatic variability of the disease. However, as cutaneous manifestations are increasingly recorded as a primary endpoints, we hope that multiple therapeutic targets will lead to better treatment options for SLE in the near future.