An Update on the Pathogenesis of Cutaneous Lupus Erythematosus and Its Role in Clinical Practice
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SYSTEMIC LUPUS ERYTHEMATOSUS (G TSOKOS, SECTION EDITOR)
An Update on the Pathogenesis of Cutaneous Lupus Erythematosus and Its Role in Clinical Practice Jay Patel 1,2 & Robert Borucki 1,2 & Victoria P. Werth 1,2,3
# This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply 2020
Abstract Purpose of Review Understanding the pathogenesis of cutaneous lupus erythematosus (CLE) is an important step in developing new medications and providing effective treatment to patients. This review focuses on novel research within CLE pathogenesis, as well as some of the medications being developed based on this knowledge. Recent Findings The subtle differences between systemic lupus erythematosus (SLE) and CLE pathogenesis are highlighted by differences in the circulating immune cells found in each disease, as well as the specific pathways activated by ultraviolet light. Plasmacytoid dendritic cells and the related type I interferon pathway are major components of CLE pathogenesis, and as such, therapies targeting components of this pathway have been successful in recent clinical trials. B cell–depleting therapies have shown success in SLE; however, their role in CLE is less clear. Understanding the differences between these manifestations of lupus allows for the development of therapies that are more effective in skin-specific disease. Summary Discovering key pathways in CLE pathogenesis is critical for understanding the clinical features of the disease and ultimately developing new and effective therapies. Keywords Cutaneous lupus erythematosus . Discoid lupus erythematosus . Pathogenesis . Targeted therapy
Introduction Cutaneous lupus erythematosus (CLE) is an autoimmune inflammatory skin disease that can present with a variety of cutaneous manifestations. While CLE can exist as an independent entity, it can also be associated with systemic lupus erythematosus (SLE). The recognition of CLE is important as cutaneous disease is a presenting sign of SLE in 20 to 25% of cases and ultimately is seen in 70 to 80% of SLE cases [1]. CLE has a clear female predominance of 3 or 4:1, a similar trend to the 8:1 female predominance in SLE [2•, 3]. In This article is part of the Topical Collection on Systemic Lupus Erythematosus * Victoria P. Werth [email protected] 1
Corporal Michael J. Crescenz VAMC, Philadelphia, PA, USA
2
Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
3
Department of Dermatology, Perelman Center for Advanced Medicine, Suite 1-330A, 3400 Civic Center Boulevard, Philadelphia, PA 19104, USA
addition to the risk of systemic involvement, the presence of cutaneous lesions has a significant impact on patients’ quality of life [4, 5]. CLE can be divided into three major subtypes including acute, subacute, and chronic CLE (ACLE, SCLE, CCLE). The most common variant of CCLE is discoid lupus erythematosus (DLE). While ACLE has the greatest chance of association with SLE (80 to 90% of cases), SCLE and
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