An evidence based approach to refractory cutaneous lupus erythematosus

Cutaneous lupus erythematosus (CLE) is a chronic autoimmune disease that can present with a variety of skin manifestations and have a dramatic effect on patients' quality of life. Effective treatment options for this disease are limited, and the efficacy of these treatments is often supported with low levels of evidence. This makes the treatment of refractory disease especially challenging, as it is difficult to achieve a consensus on the appropriate progression of treatment beyond first and second-line treatment options. The treatment of refractory CLE often involves some degree of immunosuppression, which carries some risk for patients and requires a thoughtful approach to the selection of medications. Some treatments that have proven to be effective in systemic disease may not be as effective in cutaneous disease, making it difficult to extrapolate from the available evidence on systemic lupus erythematosus (SLE). Ultimately, the increased use of objective skin measurements in SLE clinical trials is necessary to understand drug efficacy in CLE and develop new treatments for this challenging disease. This manuscript provides clinical examples of the challenges involved in treating refractory CLE, examines the evidence currently available for treatment options, and provides an algorithmic approach to the treatment of refractory disease based on this evidence. Novel therapies under development for CLE are also discussed, as they may soon be part of the accepted treatment regimen for refractory CLE.