Cutaneous Lupus Erythematosus: An Update on Pathogenesis and Future Therapeutic Directions

American Journal of Clinical Dermatology, Journal Year: 2023, Volume and Issue: 24(4), P. 521 - 540

Published: May 4, 2023

Lupus erythematosus comprises a spectrum of autoimmune diseases that may affect various organs (systemic lupus [SLE]) or the skin only (cutaneous [CLE]). Typical combinations clinical, histological and serological findings define clinical subtypes CLE, yet there is high interindividual variation. Skin lesions arise in course triggers such as ultraviolet (UV) light exposure, smoking drugs; keratinocytes, cytotoxic T cells plasmacytoid dendritic (pDCs) establish self-perpetuating interplay between innate adaptive immune system pivotal for pathogenesis CLE. Therefore, treatment relies on avoidance UV protection, topical therapies (glucocorticosteroids, calcineurin inhibitors) rather unspecific immunosuppressive immunomodulatory drugs. Yet, advent licensed targeted SLE might also open new perspectives management The heterogeneity CLE be attributable to individual variables we speculate prevailing inflammatory signature defined by either cells, B pDCs, strong lesional type I interferon (IFN) response, above suitable predict therapeutic response treatment. pretherapeutic assessment infiltrate could stratify patients with refractory T-cell-directed (e.g. dapirolizumab pegol), B-cell-directed belimumab), pDC-directed litifilimab) IFN-directed anifrolumab). Moreover, Janus kinase (JAK) spleen tyrosine (SYK) inhibitors broaden armamentarium near future. A close interdisciplinary exchange rheumatologists nephrologists mandatory optimal best strategy.

Language: Английский

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Cutaneous lupus erythematosus: a review of etiopathogenic, clinical, diagnostic and therapeutic aspects

Anais Brasileiros de Dermatologia, Journal Year: 2023, Volume and Issue: 98(3), P. 355 - 372

Published: March 1, 2023

Cutaneous lupus erythematosus is an autoimmune disease of varied clinical expression, which may present as exclusively cutaneous or be one the multiple manifestations systemic erythematosus. Its classification includes acute, subacute, intermittent, chronic and bullous subtypes, are usually identified based on features histopathological laboratory findings. Other non-specific associated with related to activity. Environmental, genetic immunological factors play a role in pathogenesis skin lesions Recently, considerable progress has been made elucidating mechanisms involved their development, allows for foreseeing future targets more effective treatments. This review proposes discuss main etiopathogenic, clinical, diagnostic therapeutic aspects erythematosus, aiming update internists specialists from different areas.

Language: Английский

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Circulating and skin biopsy-present cytokines related to the pathogenesis of cutaneous lupus erythematosus

Autoimmunity Reviews, Journal Year: 2022, Volume and Issue: 22(2), P. 103262 - 103262

Published: Dec. 20, 2022

Language: Английский

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Role of Anifrolumab in Refractory Cutaneous Manifestations of Lupus Erythematosus: A Case Series and Literature Review

Cureus, Journal Year: 2023, Volume and Issue: unknown

Published: May 27, 2023

Lupus erythematosus (LE) is an autoimmune disease that presents either as a systemic (SLE) or isolated skin (CLE). Currently, there no FDA-approved medication specifically for CLE, and treated with the same approach SLE. We present two refractory cases of SLE severe cutaneous manifestations unresponsive to first-line therapy anifrolumab. First, 39-year-old Caucasian female known history subacute CLE presented clinic her symptoms. Her current regimen was hydroxychloroquine (HCQ), mycophenolate mofetil (MMF), s/c belimumab improvement. Belimumab discontinued, she started on anifrolumab significant Another, 28-year-old medical referred rheumatology elevated anti-nuclear antibody (ANA) ribonucleoprotein (RNP) titers. She diagnosed SLE, HCQ, belimumab, MMF but failed produce reasonably good outcome. Hence discontinued added instead The treatment spectrum wide, which includes antimalarial oral corticosteroids (OCS), immunosuppressants (Methotrexate-MTX, MMF, azathioprine-AZT). Anifrolumab, type 1 IFNα receptor subunit (IFNAR1) inhibitor, has been recently approved by FDA moderate while standard in August 2021. Early use may result improvement patients.

Language: Английский

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Refractory Dermatitis Evolving Into Lupus Spectrum Disease in the Setting of Dupilumab Use

Cureus, Journal Year: 2025, Volume and Issue: unknown

Published: Feb. 24, 2025

Cutaneous lupus erythematosus (CLE) encompasses a broad range of clinical and histopathologic variants that can overlap with other dermatologic entities, complicating accurate diagnosis. We report the case 42-year-old male patient who initially presented diffuse pruritic eruption presumed to be atopic dermatitis, for which dupilumab was initiated. Within following weeks, developed fever unknown origin diarrhea, raising concern an atypical drug-related reaction or unmasked autoimmune process. Subsequent biopsies demonstrated evolving features, including superficial deep perivascular dermatitis suggestive drug eruption. In addition, dermal mucin deposition mixed neutrophilic lymphocytic infiltrates is cutaneous lupus, such as tumid lupus-related urticarial dermatosis. Despite negative direct immunofluorescence fluctuating autoantibodies, partial sustained improvement occurred hydroxychloroquine therapy. The patient's variable serologic profile (including intermittent positivity antiribonucleoprotein anti-Smith), transient lesions, histopathology highlight difficulties in definitively categorizing subtypes. While causal link between lupus-like disease remains unproven, temporal association raises possibility T helper type 1/T 2 immune modulation may unmask subclinical conditions. This underscores importance repeated clinicopathologic correlation multidisciplinary surveillance patients presenting treatment-refractory dermatitis. Ongoing rheumatologic evaluation critical early detection systemic involvement, especially when etiologies are suspected. Hydroxychloroquine cornerstone therapy many CLE provide substantial improvement, even complex overlapping scenarios.

Language: Английский

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Structural Hierarchy in Collagenous Tissues and the Effects of Genetic Mutations

Published: Jan. 1, 2025

Language: Английский

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An update on the management of refractory cutaneous lupus erythematosus

Frontiers in Medicine, Journal Year: 2022, Volume and Issue: 9

Published: Sept. 23, 2022

Management of cutaneous lupus erythematosus (CLE) involves a combination preventive measures, topical and systemic drugs, fairly similar for the different subtypes. Although guidelines exist, to date, no specific drugs have been specifically licensed CLE. Antimalarials remain first-line treatment, but many patients do not respond, making refractory challenge clinicians. The choice alternative medication should be based on effectiveness, safety cost. Most available CLE adapted from (SLE) treatment existing literature is limited small studies evidence often lacks. As knowledge pathogenesis both SLE improving, promising new therapies are emerging. In this review, we discuss medications, focusing novelties under development

Language: Английский

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Tumid Lupus Erythematosus (TLE): A Review of a Rare Variant of Chronic Cutaneous Lupus Erythematosus (cCLE) with Emphasis on Differential Diagnosis

Diagnostics, Journal Year: 2024, Volume and Issue: 14(7), P. 780 - 780

Published: April 8, 2024

Tumid lupus erythematosus (TLE) has been the subject of heated debate regarding its correct nosographic classification. The definition TLE changed over time, varying according to different studies performed. In this review, we address initial TLE, changes that have taken place in understanding and placement within classification cutaneous (CLE), with a focus on clinical, histopathological, immunophenotypical, differential diagnosis aspects.

Language: Английский

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Dermatological Manifestation of SLE Patients, Living in Aseer Region

Journal of Family Medicine and Primary Care, Journal Year: 2024, Volume and Issue: 13(4), P. 1249 - 1253

Published: April 1, 2024

Systemic lupus erythematosus (SLE) is an autoimmune disease that affects both men and women differently has a variety of multisystemic symptoms. One the diseases most often affected target organs skin. Different ethnic racial groupings may display variations in incidence, clinical heterogeneity, severity depending on environmental, cultural, or genetic factors. This study conducted to determine prevalence SLE's cutaneous symptoms their relationship organ involvement.

Language: Английский

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Tumor Necrosis Factor-Alpha: Ally and Enemy in Protean Cutaneous Sceneries

International Journal of Molecular Sciences, Journal Year: 2024, Volume and Issue: 25(14), P. 7762 - 7762

Published: July 16, 2024

Skin is the forestage for a series of many-sided functions tumor necrosis factor-alpha (TNF-α), proinflammatory cytokine with staggering versatility and sizable implications tissue homeostasis, immune responses, angiogenesis, apoptosis, local systemic inflammation. An aberrant TNF-α-mediated crosstalk has been linked to pathogenesis acute chronic skin inflammatory diseases, indeed, TNF-α dysregulation can contribute development progression psoriasis, vitiligo, damage following exposition ultraviolet light radiations, cutaneous lupus erythematosus, acne vulgaris. Therapies that target are conspicuously used in treatment different disorders, aiming modulate vivo triggered by many cells, including keratinocytes, mast or Langerhans reduce inflammation taking place within skin. Herein, we focus on key relationships between distinct non-neoplastic physiologic conditions, showing natural induction may have protective significance but overproduction be harmful even lethal. Many questions remain unraveled therapeutic practice, caution should exercised due eventual backlashes exerted maintaining health provoking disease.

Language: Английский

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