Cutaneous Lupus Erythematosus: Current Treatment Options

Abstract

Opinion statement Cutaneous lupus erythematosus (CLE) is a heterogeneous autoimmune skin disease that can present with or without systemic lupus erythematosus (SLE). Managing CLE is important in order to reduce potential and established areas of damage, as well as improve quality of life (QOL). Non-drug therapy should be used in every case, which includes photoprotection, smoking cessation, and avoiding drugs that can trigger or exacerbate the disease. Topical corticosteroids and topical calcineurin inhibitors are often used in addition to lifestyle changes. Antimalarials are first-line systemic therapies, with hydroxychloroquine being the drug of choice. Quinacrine can be added to hydroxychloroquine for greater efficacy in hydroxychloroquine-refractory patients, and chloroquine can be used in place of hydroxychloroquine in patients who are unable to tolerate hydroxychloroquine. Second-line therapies include oral retinoids, immunosuppressives, immunomodulators, biologics, and pulsed dye laser. Systemic steroids may be necessary when bridging therapies but should normally be avoided due to their side effects. There is a paucity of high quality evidence with regard to management of cutaneous lupus, making it challenging to determine an appropriate treatment in refractory cases. Trials on existing therapies as well as new therapies are necessary in order to better treat patients with CLE.

The CLASI, a validated tool for the evaluation of skin disease in lupus erythematosus: a narrative review

“…In patients taking antimalarial and antimetabolite medications, an improvement in disease activity resulted in an improvement in QoL, even if the disease did not entirely subside (8). Medications that meaningfully improve QoL may not exhibit near or complete clearance of disease activity but still be an important improvement for disease management (57).…”

“…Several therapeutic interventions are currently used to treat CLE. Although the majority of these interventions are typically well-tolerated and can be effective, those used for refractory disease can cause serious side effects (8). Despite this, in the past fifty years, only one new drug has been approved for SLE and no drug has been approved for CLE (8,61).…”

“…Although antimalarials are generally well-tolerated and provide significant relief to a large subset of patients, quinacrine has unfortunately become largely unavailable due to import restrictions on the powder that was subsequently compounded. For patients unable to tolerate antimalarials, or in recalcitrant cases, other systemic agents (i.e., mycophenolate mofetil, azathioprine, methotrexate, dapsone, sulfasalazine, oral retinoids, thalidomide/lenalidomide, and systemic corticosteroids) should be considered (8). Although these medications can be effective, they can have significant side effects, making it difficult for providers to prescribe these medications (2,(8)(9)(10).…”

Advancing understanding, diagnosis, and therapies for cutaneous lupus erythematosus within the broader context of systemic lupus erythematosus

“…There is a need for safe and effective therapies for these refractory patients. Despite this, no medications have been approved for CLE in over 50 years; this is largely due to problems associated with lupus trial design 35 . In a recent proposal for optimizing lupus clinical trials, Merrill et al highlighted challenges to developing new treatments 9 .…”

“…Second, the use of background therapies has led to high placebo response rates. In order to decrease the chances of this occurring, it is recommended that patients with lower placebo response rates, particularly patients with refractory DLE or SCLE, be enrolled in clinical trials, as these patients do not respond well to background medications 35 . Notably, however, patients with isolated cutaneous disease generally require fewer background medications compared with patients with multi-organ involvement who may be systemically ill.…”