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Cutaneous lupus erythematosus (CLE) is a disfiguring and potentially disabling disease that causes significant morbidity in patients. Antimalarials are an important class of medication used to treat this disease and have been the first-line systemic therapy since the 1950s. Quinacrine, in particular, is used as an adjuvant therapy to other antimalarials for improved control of CLE. Quinacrine is currently unavailable in the USA, which has taken away an important component of the treatment regimen of patients with CLE. This paper reviews the evidence of available local and systemic therapies in order to assist providers in choosing alternative treatments for patients who previously benefited from quinacrine therapy.

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4. Laryngoscope, 128:885-888, 2018.

Cutaneous lupus erythematosus (CLE) is a chronic autoimmune disease that can present with a variety of skin manifestations and have a dramatic effect on a patient’s quality of life. Effective treatment options for this disease are limited, and the efficacy of these treatments is often supported by 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. Here, we provide clinical examples of the challenges involved in treating refractory CLE, examine the evidence currently available for treatment options, and provide 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.

ObjectiveThe lack of standardised outcomes and outcome measures for cutaneous lupus erythematosus (CLE) represents a substantial barrier to clinical trial design, comparative analysis and approval of novel investigative treatments. We aimed to develop a working core outcome set (COS) for CLE randomised controlled trials and longitudinal observational studies.MethodsWe conducted a multistage literature review of CLE and SLE studies to generate candidate domains and outcome measures. Domains were narrowed to a working core domain set. Outcome measures for core domains were identified and examined.ResultsProposed core domains include skin-specific disease activity and damage, investigator global assessment (IGA) of disease activity, symptoms (encompassing itch, pain and photosensitivity), health-related quality of life (HRQoL) and patient global assessment (PtGA) of disease activity. Recommended physician-reported outcome measures include the Cutaneous Lupus Erythematous Disease Area and Severity Index (CLASI) and Cutaneous Lupus Activity IGA (CLA-IGA). For the domains of symptoms, HRQoL and PtGA of disease activity, we were unable to recommend one clearly superior instrument.ConclusionThis work represents a starting point for further refinement pending formal consensus activities and more rigorous evaluations of outcome measure quality. In the interim, the proposed working COS can serve as a much-needed guide for upcoming CLE clinical trials.

Bullous pemphigoid (BP) is a rare, chronic antibody-mediated autoimmune blistering disease primarily affecting the elderly, with an age of onset over 60. Current treatment options are limited and involve the use of corticosteroids and immunosuppressants, but their long-term use is associated with significant morbidity and mortality. In Japan, human intravenous immunoglobin is approved for the treatment of corticosteroid-refractory BP. However, no treatment option is approved by the Food and Drug Administration for the management of BP. Therefore, developing effective therapies free of debilitating side effects is imperative. In this review, we summarize the main immunologic pathways involved in the pathogenesis of BP, with an emphasis on the role of eosinophils, immunoglobulins, cytokines such as the interleukin (IL)-4 and IL-5, and complements. We further discuss the latest advances with novel therapeutic targets tested for the management of BP. Ongoing efforts are needed to run well-designed controlled trials and test the efficacy and safety of investigational drugs while providing much-needed access to these medications for refractory patients who will not otherwise be able to afford them as off-label prescriptions.

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