Thalidomide has shown excellent results for severe cutaneous lupus erythematosus (CLE), but its prescription is limited by potentially severe adverse events.

To assess the overall rate of response to thalidomide in CLE with respect to CLE subtypes and the occurrence rate of relevant adverse events on the basis of previously published studies.

We performed a systematic review and meta-analysis of studies published in MEDLINE, Embase, and the Cochrane Library between 1965 and January 2017. The proportions of responders and rates of adverse events were extracted from individual studies and pooled using random effects or fixed models.

Among 548 patients from 21 included studies, the overall rate of response to thalidomide was 90% (95% confidence interval [CI], 85-94), with similar response rates between CLE subtypes. Conversely, the pooled rate of thalidomide withdrawal related to adverse events was 24% (95% CI, 14-35) including confirmed peripheral neuropathy in 16% (95% CI, 9-25) and thromboembolic events in 2% (95% CI, 1-3). The pooled rate of relapse after thalidomide withdrawal was 71% (95% CI, 65-77) compared with 34% (95% CI, 25-44) with a maintenance dose.

We found important statistical heterogeneity across included studies.

Considering the frequent occurrence of adverse events, prescription of thalidomide should be restricted to patients with severely refractory CLE or who are at high risk for severe scarring.