Background/Purpose: Cutaneous lupus erythematosus (CLE), occurring with or without systemic lupus erythematosus (SLE), is a group of inflammatory skin diseases that can be very debilitating, causing significant psychological distress, and in some cases scarring. We sought to comprehensively present the evidence for different treatment modalities in patients with cutaneous manifestations of lupus erythematosus (LE).

Methods: Medline, Embase, Scopus and Cochrane CENTRAL were searched electronically from 1990 to March 2019, using keywords related to cutaneous lupus and synonyms and treatment. Articles retrieved were screened for relevance, including reference lists of retrieved reviews. We included clinical trials, observational studies or case series with 5 or more patients focussing on treatment of CLE, with or without SLE.

Results: The search identified 6637 studies, of which 104 were included. Each study commonly included a heterogenous mixture of CLE subtypes, with or without SLE. The 104 included studies investigated 11 different categories of treatment in 6811 patients. Treatments included topical calcineurin inhibitors (CNIs) (13 studies), sun protection (5 studies), R-salbutamol cream (2 studies), antimalarials (23 studies), synthetic disease modifying anti-rheumatic drugs (DMARDs)(10 studies), retinoids (2 studies), thalidomide/lenalidomide (22 studies), biologic therapies (12 studies), intravenous immune globulin (3 studies), laser (6 studies) and other therapies (6 studies). A suggested management algorithm based on these data, determined by disease severity, is summarised in Figure 1. General measures to be considered include smoking cessation, sun protection measures and optimisation of vitamin D levels. Moderate evidence exists for benefit with topical CNIs, particularly as a steroid sparing agent in areas at high risk of steroid complications (e.g. facial skin). There is moderate evidence for hydroxychloroquine, which is first-line in SLE patients, limited evidence to support other synthetic DMARDs, and moderate evidence supporting thalidomide but with significant risk of toxicity. Of biologic therapies, there are moderate data to support belimumab. Limited evidence exists for other therapies.

Conclusion: Many management options are available for CLE, including topical, systemic and biologic therapies, with a variable balance of efficacy and toxicity. There is a paucity of high-quality clinical trial data. Further trials are required to better understand optimal management of CLE, particularly in specific subgroups.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/management-of-cutaneous-manifestations-of-lupus-erythematosus-a-systematic-review/