Cutaneous Lupus Erythematosus: Overview and Treatment

    January 2017 in “ Springer eBooks
    Christopher B. Hansen, David Fiorentino, Richard D. Sontheimer
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    TLDR The document concludes that Cutaneous Lupus Erythematosus has different forms, is influenced by genetic and environmental factors, and can be treated with various medications, but more targeted therapies are needed.
    The document from 2017 provides a comprehensive overview of Cutaneous Lupus Erythematosus (CLE), detailing its subtypes, epidemiology, clinical features, pathogenesis, and treatment options. It explains that CLE can be classified into acute (ACLE), subacute (SCLE), and chronic (CCLE) forms, with ACLE being strongly associated with systemic lupus erythematosus (SLE) and presenting as a "butterfly rash." SCLE is often photosensitive, and CCLE, particularly Discoid LE (DLE), can lead to scarring and pigmentary changes. The document notes that DLE affects the scalp in 60% of patients and can be misdiagnosed due to similarities with squamous-cell carcinoma. It also discusses the genetic predispositions for CLE, such as HLA haplotypes and polymorphisms in the TNF-α promoter, and environmental triggers like UV light, which is strongly implicated in the disease. Treatment options include sun protection, corticosteroids, calcineurin inhibitors, and systemic agents like antimalarials, with thalidomide being effective for resistant cases but having a high relapse rate and risk of neuropathy. Other treatments mentioned are lenalidomide, traditional immunosuppressives, IVIG, and monoclonal antibodies, with future therapies potentially targeting T lymphocytes or inhibiting TLR signaling. The document also addresses the relationship between CLE and SLE, the importance of distinguishing between lupus-specific and non-specific skin diseases, and treatment for patients unresponsive to antimalarials.
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