Persistent cutaneous lupus erythematosus: A pathway toward systemic disease?

Area of Science:

• Immunodermatology
• Rheumatology
• Autoimmune Diseases

Background:

• Cutaneous lupus erythematosus (CLE) is an autoimmune condition primarily affecting the skin.
• While often considered localized, a subset of CLE patients, particularly those with refractory or relapsing disease, risk progression to systemic lupus erythematosus (SLE).
• The heterogeneity in CLE subtypes and increasing treatment resistance underscore the need for better prediction of disease evolution and refined therapeutic strategies.

Purpose of the Study:

• To review current knowledge on refractory/recurrent CLE clinical characteristics and management.
• To analyze epidemiological data and risk factors for CLE progression to SLE.
• To delineate shared and distinct immunopathogenic pathways between CLE and SLE.

Main Methods:

• Literature review synthesizing clinical, epidemiological, and mechanistic data.
• Analysis of immunopathogenic pathways including type I interferon signaling, neutrophils, B cells, and T cell immunity.
• Integration of clinical insights with emerging mechanistic data for risk stratification.

Main Results:

• Evidence suggests a subset of CLE patients face a heightened risk of systemic progression.
• Shared and distinct immunopathogenic mechanisms link CLE and SLE, involving key immune players.
• Refractory and recurrent CLE present significant management challenges.

Conclusions:

• Early risk stratification is essential for identifying CLE patients at risk of SLE progression.
• Targeted immunomodulation strategies are crucial for preventing systemic involvement in high-risk individuals.
• Improved understanding of CLE-SLE transition offers new perspectives for better long-term patient outcomes.