Comparison of patients with isolated cutaneous lupus erythematosus versus systemic lupus erythematosus with cutaneous lupus erythematosus as the sole clinical feature: A monocentric study of 149 patients

Background: Cutaneous lupus erythematosus (CLE) may present as an isolated entity or be classified as Systemic lupus erythematosus (SLE) by the presence of laboratory abnormalities, including cytopenia, low complement levels, and/or autoantibodies (CLE with laboratory SLE). Objective: To compare isolated CLE and CLE with laboratory SLE and to validate an existing 3 -item score with age < 25 years (1 point), phototypes V to VI (1 point), antinuclear antibodies >= 1:320 (5 points) to predict the risk of progression from CLE to severe SLE (sSLE). Methods: Monocentric cohort study including consecutive patients with CLE. CLE with laboratory SLE was defined by 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for SLE score of >= 10 points at baseline with CLE as the sole clinical feature. Results: Of the 149 patients with CLE, 20 had CLE with laboratory SLE. The median follow-up duration was 11.3 years (IQR: 5.1-20.5). Ten patients (7%) had sSLE developed. In survival analysis, the risk of progression to sSLE was higher among CLE with laboratory SLE (hazard ratio = 6.69; 95% CI: 1.93-23.14, P < .001) compared to isolated CLE. In both groups, none of the patients with a risk score # 2 had sSLE developed. Limitations: Monocentric study with a limited number of patients. Conclusions: CLE with laboratory patients with SLE have a higher risk of progression to sSLE than isolated CLE.