Cutaneous Lupus Erythematosus: An Update on Pathogenesis, Diagnosis and Treatment

Abstract

Cutaneous lupus erythematosus (CLE) includes a broad range of dermatologic manifestations, which may or may not be associated with systemic disease. Recent studies in this area continue to shape our understanding of this disease and treatment options. Epidemiologic studies have found an incidence of CLE of 4.30 per 100,000, which approaches similar analysis for systemic lupus erythematosus (SLE). Although there have been extensive efforts to define SLE, the classification of CLE and its subgroups remains a challenge. Currently, diagnosis relies on clinical and laboratory findings as well as skin histology. The Cutaneous Lupus Area and Severity Index™ (CLASI™) is a validated measure of disease activity and damage. CLE pathogenesis is multifactorial and includes genetic contributions as well as effects of ultraviolet (UV) light. Immune dysregulation and aberrant cell signaling pathways through cytokine cascades are also implicated. Patient education and avoidance of triggers are key to disease prevention. Antimalarials and topical steroids continue to be the standard of care; however, immunosuppressants, thalidomide analogs and monoclonal antibodies are possible systemic therapies for the treatment of recalcitrant disease.

TWEAK/Fn14 Activation Participates in Ro52-Mediated Photosensitization in Cutaneous Lupus Erythematosus

“…Currently, photosensitivity in CLE is believed to be a consequence of ultraviolet irradiation based on genetic background (6). Ultraviolet B (UVB) irradiation upregulates Ro52 in cutaneous keratinocytes (7), which may further trigger the production of autoantibodies to Ro52 (8).…”

High Prevalence of Metabolic Syndrome in Patients with Discoid Lupus Erythematosus: A Cross-Sectional, Case-Control Study

“…As known, a critical and most important part of assessment of DLE patients is evaluating and monitoring for early detection of SLE development [1–3]. Furthermore, especially in recent years, evaluating the significant accompanying comorbidities in terms of morbidity and mortality such as CVDs and cerebrovascular accidents (which are more frequent in SLE) has been highlighted in skin-restricted lupus patients [4–6].…”

“…DLE occurs more frequently in women in their fourth and fifth decades of life, which appears to be well-demarcated, erythematous, scaly, keratotic plaques and eventually leading to disfiguring scarring and skin atrophy. Patients with DLE generally have a more benign disease course as compared to patients with other CLE subtypes, with only a reported 5–10% developing SLE throughout their disease course [1–3]. …”

“…The mechanism has not been fully elucidated, but increasing evidence suggests the role of ultraviolet irradiation, immune dysregulation, aberrant cell signaling pathways through the cytokine cascades (T helper-1 mediators including IFN- α , IL-1, IL-6, IL-8, IL-10, and TNF- α ), apoptosis, necrosis, autoantibodies, plasmacytoid dendritic cells, T cells, B cells, and vascular changes [1–3]. Although SLE and DLE are clinically different diseases, there are multiple genetic similarities between the two groups to consider a common pathogenesis [2, 3].…”

Cutaneous lupus erythematosus: updates on pathogenesis and associations with systemic lupus

“…Subtypes of CLE are currently grouped on the basis of histology, lesion duration, clinical findings, and laboratory abnormalities [4, 5] and are summarized in Table 1. [6–9]…”