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Acute cutaneous lupus erythematosus. Classic malar “butterfly” rash of the forehead, chin, and malar cheeks. Note the relative sparing of the nasolabial folds.

Acute cutaneous lupus erythematosus. Classic malar “butterfly” rash of the forehead, chin, and malar cheeks. Note the relative sparing of the nasolabial folds.

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Lupus, Latin for “wolf,” is a term used to describe many dermatologic conditions, some of which are related to underlying systemic lupus erythematosus, while others are distinct disease processes. Cutaneous lupus erythematosus includes a wide array of visible skin manifestations and can progress to systemic lupus erythematosus in some cases. Cutane...

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... Cutaneous lupus erythematosus (CLE) is a form of lupus that affects the skin, causing lesions, rashes, and other changes, particularly in sun-exposed areas. This autoimmune disease occurs when the immune system mistakenly attacks healthy tissues, including the skin, resulting in inflammation and cellular damage [155]. Autoimmune skin diseases like CLE can be challenging to treat due to the need to suppress the immune response without compromising the patient's overall health [156]. ...
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Amphiphilic copolymers (ACs) are versatile systems with self-assembling and aggregating properties, enabling the formation of nanomaterials (NMs) such as micelles, vesicles, nanocapsules, and nanogels. These materials have been extensively explored for the delivery of various drugs and active compounds, enhancing the solubility and permeation of poorly water-soluble drugs into skin tissue. This improvement facilitates the treatment of skin diseases, including chronic conditions like cancer, as well as infections caused by bacteria, fungi, and viruses. This review summarizes recent applications of ACs in skin treatment, with a particular focus on their use in anti-cancer drug therapy. It covers the synthesis, classification, and characterization of ACs using various experimental techniques. Additionally, it discusses recent research on different drug delivery pathways using ACs, including encapsulation efficiency, release behavior, characteristics, applications, and responses to various chemical and physical stimuli (both in vivo and in vitro). Furthermore, this review provides a comprehensive analysis of the effects of ACs NMs on several skin diseases, highlighting their potential as alternative treatments.
... Used as a clinical tool, the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) standardizes the way disease activity is characterized and offers guidance for spotting a clinical change. [12][13][14] This study aimed to evaluate the prevalence of cutaneous manifestations of SLE and to find out the association with organ involvement. Patients with SLE have both unique and general cutaneous characteristics. ...
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A BSTRACT Introduction Systemic lupus erythematosus (SLE) is an autoimmune disease that affects both men and women differently and has a variety of multisystemic symptoms. One of the diseases most often affected target organs is the skin. Different ethnic and racial groupings may display variations in disease incidence, clinical heterogeneity, and severity depending on environmental, cultural, or genetic factors. This study is conducted to determine the prevalence of SLE’s cutaneous symptoms and their relationship to organ involvement. Materials and Methods Data were gathered for this study from the patient chart, the study design was the retrospective chart review after the consent of the patients and obtaining an ethical approval, The study was carried out in Aseer Central Hospital, Abha Saudi Arabia Results Out of a total of 100 patients 92% were females while 8% were males. The mean (SD) of the age of the respondent was 38.3 (8.5). 89.2 of the respondents had skin manifestations Conclusion A thorough understanding of SLE skin lesions will aid in the accurate identification of the condition and in the effective therapy of lupus patients. In order to more accurately diagnose cutaneous lesions in SLE patients, we need more dermatology and rheumatology clinics that combine expertise together.
... An initial diagnosis of cutaneous LE (CLE) is associated with a 5-18% risk of developing SLE over the next 3-5 years, and these patients usually present with a mild course of the disease [4][5][6][7]. Concurrently, CLE may occur as a separate entity in the absence of systemic symptoms, and such a variant is characterized by a 2-3-fold higher incidence compared to SLE [8,9]. Genetic and environmental factors play a key role in the pathogenesis of CLE, as in SLE [10]. ...
... Extensive and tense vesiculo-bullous lesions develop, particularly affecting areas exposed to UV radiation, i.e., face, neck, upper trunk, upper limbs, red zone of the lips and mucous membrane of the oral cavity [18,25]. However, bullous lesions may also appear on the skin of other parts of the body [9]. Bullae are located on erythematous-background or normal-looking skin and resolve without scarring or milia formation. ...
... lips and mucous membrane of the oral cavity [18,25]. However, bullous lesions may also appear on the skin of other parts of the body [9]. Bullae are located on erythematousbackground or normal-looking skin and resolve without scarring or milia formation. ...
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Lupus erythematosus (LE) is an autoimmune inflammatory disease with complex etiology. LE may present as a systemic disorder affecting multiple organs or be limited solely to the skin. Cutaneous LE (CLE) manifests with a wide range of skin lesions divided into acute, subacute and chronic subtypes. Despite classic forms of CLE, such as malar rash or discoid LE, little-known variants may occur, for instance hypertrophic LE, chilblain LE and lupus panniculitis. There are also numerous non-specific manifestations including vascular abnormalities, alopecia, pigmentation and nail abnormalities or rheumatoid nodules. Particular cutaneous manifestations correlate with disease activity and thus have great diagnostic value. However, diversity of the clinical picture and resemblance to certain entities delay making an accurate diagnosis The aim of this review is to discuss the variety of cutaneous manifestations and indicate the clinical features of particular CLE types which facilitate differential diagnosis with other dermatoses. Although in diagnostically difficult cases histopathological examination plays a key role in the differential diagnosis of LE, quick and accurate diagnosis ensures adequate therapy implementation and high quality of life for patients. Cooperation between physicians of various specialties is therefore crucial in the management of patients with uncommon and photosensitive skin lesions.
... The clinical manifestations of SLE may range from mild localised skin disease to a life-threatening illness, such as lupus nephritis or neuropsychiatric SLE. 1,2 The complex interaction among genetic, environmental, and hormonal factors is key to clinical heterogeneity in SLE manifestations. The prevalence and incidence of SLE have been increasing in recent years. ...
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Background Cutaneous involvement is common in systemic lupus erythematosus (SLE) patients and may be essential to the disease activity. This study aimed to describe cutaneous manifestations spectrum and determine the association of cutaneous lesions with the disease activity and systemic involvement among SLE patients in Malang, Indonesia. Methods A cross-sectional study was conducted using 54 SLE patients from rheumatology outpatient clinic at Saiful Anwar General Hospital Malang, Indonesia. Cutaneous features were classified according to Gilliam and Sontheimer classification of cutaneous lupus. Disease activity and clinical manifestations were documented according to Mexican-SLE disease activity index (Mex-SLEDAI). Results Among 54 subjects, 50% of the patients had cutaneous manifestations. Subacute cutaneous lupus erythematosus (SCLE) was observed in 11.1% of patients, and malar rash in 20.4%. Subjects with cutaneous lesions had significantly higher Mex-SLEDAI scores, especially those who had SCLE (p<0.001), malar rash (p=0.002), alopecia (p=0.002), and photosensitivity (p=0.032). Six patients (11.1%) had skin infections with higher disease activity (9[8–11]vs.2[0–4];p<0.001). SCLE was significantly associated with malar rash (OR 11.7[1.8–76.5]), vasculitis (OR 43.0[4.1–445.6]), and fatigue (OR 15.0[2.1–108.8]). Malar rash was associated with photosensitivity (OR 8.4[1.6–44.0]), while oral or nasal ulcer was associated with fatigue (OR 8.6 [1.4–54.6]). Vasculitis (OR 5.9[1.0–35.1]) and nephritis (OR 11.7 [1.8–76.5]) were associated with the presence of skin infection. Conclusion SCLE and malar rash are the most common cutaneous lesions among subjects. Subjects with cutaneous lesions have relatively higher disease activity. Several skin lesions are also associated with SLE patients’ systemic manifestations.
... Imaging scans, biopsies, laboratory testing, and clinical assessment are critical tools for diagnosing and treating cutaneous issues in patients. Early detection of cutaneous indications and diagnosis of systemic disorders are essential for prompt treatment and minimal consequences [5]. Dermatologists must work with other medical specialists to ensure patients get full treatment for their systemic illnesses. ...
... According to other studies, some systemic diseases have been associated with common skin signs. The symptoms of cutaneous lupus erythematosus and the need for a full diagnosis were investigated by Cooper et al. (2021). The variety of skin manifestations in systemic diseases was pointed out by Miulescu et al. in their 2020 assessment of pancreatic disorders [13]. ...
... SLE has several phenotypes and clinical manifestations involving various organs, including the joints, skin, kidneys, and organs of the neurological or hematological systems [1,2]. Cutaneous lupus erythematosus (CLE) can manifest as either a feature of SLE or an independent skin ailment [4,5]. The most prevalent symptoms of CLE include rashes, hair loss, blood vessel inflammation, ulcers, and increased sensitivity to light [6,7]. ...
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Background Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease affecting various organs with a wide range of clinical manifestations. Cutaneous lupus erythematosus (CLE) can manifest as a feature of SLE or an independent skin ailment. Health-related quality of life (HRQoL) is frequently compromised in individuals living with lupus. Understanding patients’ perspectives when living with a disease is crucial for effectively meeting their unmet needs. Social listening is a promising new method that can provide insights into the experiences of patients living with their disease (lupus) and leverage these insights to inform drug development strategies for addressing their unmet needs. Objective The objective of this study is to explore the experience of patients living with SLE and CLE, including their disease and treatment experiences, HRQoL, and unmet needs, as discussed in web-based social media platforms such as blogs and forums. Methods A retrospective exploratory social listening study was conducted across 13 publicly available English-language social media platforms from October 2019 to January 2022. Data were processed using natural language processing and knowledge graph tagging technology to clean, format, anonymize, and annotate them algorithmically before feeding them to Pharos, a Semalytix proprietary data visualization and analysis platform, for further analysis. Pharos was used to generate descriptive data statistics, providing insights into the magnitude of individual patient experience variables, their differences in the magnitude of variables, and the associations between algorithmically tagged variables. Results A total of 45,554 posts from 3834 individuals who were algorithmically identified as patients with lupus were included in this study. Among them, 1925 (authoring 5636 posts) and 106 (authoring 243 posts) patients were identified as having SLE and CLE, respectively. Patients frequently mentioned various symptoms in relation to SLE and CLE including pain, fatigue, and rashes; pain and fatigue were identified as the main drivers of HRQoL impairment. The most affected aspects of HRQoL included “mobility,” “cognitive capabilities,” “recreation and leisure,” and “sleep and rest.” Existing pharmacological interventions poorly managed the most burdensome symptoms of lupus. Conversely, nonpharmacological treatments, such as exercise and meditation, were frequently associated with HRQoL improvement. Conclusions Patients with lupus reported a complex interplay of symptoms and HRQoL aspects that negatively influenced one another. This study demonstrates that social listening is an effective method to gather insights into patients’ experiences, preferences, and unmet needs, which can be considered during the drug development process to develop effective therapies and improve disease management.
... La manera en que se manifiesta la enfermedad puede variar según la edad en que comienza; una presentación clínica inusual con síntomas raros puede dificultar el diagnóstico, especialmente cuando la enfermedad comienza en una edad temprana. En alrededor del 40% de los pacientes, el lupus eritematoso sistémico pediátrico puede presentarse de manera grave y afectar múltiples sistemas del cuerpo en el momento del diagnóstico, lo que a menudo requiere hospitalización inmediata e inclusive se ha encontrado reportes de la necesidad de terapia sustitutiva renal de emergencia (Cooper et al., 2021;Valenzuela et al., 2021). El LESc se caracteriza por su naturaleza diversa y no sigue un patrón específico de afectación, por lo tanto, es crucial analizar detenidamente la historia cronológica de todos los eventos relevantes y los antecedentes familiares de enfermedades autoinmunes. ...
Article
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El lupus eritematoso sistémico pediátrico (LESc) es una enfermedad multisistémica de origen autoinmune complejo, de etiología desconocida, caracterizada por la aparición de distintos anticuerpos séricos y diferentes resultados de laboratorio e imagen dependiendo el progreso de la enfermedad. Se narra el reporte de caso de una adolescente de 14 años que presenta alza térmica de 1 mes de evolución acompañada de erupción malar en forma de alas de mariposa, petequias intrabucales en paladar e infralingual, además de erupciones petequiales en regiones palmar y plantar, con leucopenia, trombocitopenia, disminución de los niveles del complemento sérico y anticuerpos DNA positivos, por lo que se cataloga como debut lúpico y se hospitaliza para su manejo integral donde permanece por 5 días hasta su resolución completa y se deriva a unidad de mayor complejidad para seguimiento con reumatología pediátrica. Se considera de importancia conocer las manifestaciones clínicas y patrones de crisis lúpica en la población pediátrica ya que al ser de baja frecuencia, poseen alta morbilidad y compromiso sistémico.
... SLE usually involves organs, including the skin [1] . Cutaneous manifestations are many and of wide spectrum [9] . In most cases, skin lesions in SLE occur in sun-exposed areas [10] . ...
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Unlabelled: Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease that affects many organs. In this report, we discuss the case of a patient with SLE who presented to an out-hospital clinic, complaining of fever and pain with genital ulcers. Negative evaluations for other causes of genital ulcers, indicated these ulcers as related to SLE. This case highlights the importance of including SLE ulcers in the differential diagnosis of genital ulcers. Learning points: Determining the cause of genital ulceration is a clinical challenge, especially in sexually active individuals.Genital ulcers associated with SLE are often underdiagnosed. Any patient with a history of SLE presenting with genital discomfort and/or pain must be evaluated for genital ulcers without delay.
... The identification of these subtypes is crucial, as they often occur in different clinical contexts, with diagnostic, prognostic, and therapeutic implications. 2 Exclusive CLE is two to three times more frequent than SLE, with an annual incidence of 4.3/100,000 in Europe and the US. 1---3 There is a predominance of LE in the female sex, where the incidence of adult SLE is 7 to 15 times higher and, for childhood SLE, 3 to 4 times higher. ...
... This female predominance is less evident in the isolated cutaneous forms of LE, with a ratio of 4:1, and it is even less significant, with a ratio of 3:1, for discoid LE (DLE), the most common form of CCLE. 2,4 There are also racial differences in the occurrence of CLE, with a 5.4-fold higher risk of CCLE in African-Americans when compared to Caucasians. 4 In New Zealand, when compared to the population of European origin, the indigenous Māori population shows a relative risk of 2.47 for the development of all CLE subtypes and 5.96 risks for CCLE. 5 The peak incidence of SLE occurs in middle age, but it occurs later in men. 6 Although it also affects children and the elderly, exclusive CLE is more common between the ages of 20 and 40 years, with a mean age at onset of 43 years, varying according to the subtype. ...
... 3 It is identified at the time of diagnosis in approximately 50% of SLE cases and can be triggered or exacerbated by acute sun exposure. 2 The localized form is the predominant one (90%---95%) and corresponds to the typical butterfly rash, which manifests as an erythematous and finely desquamative lesion, symmetrically affecting the malar regions and the nasal dorsum, generally sparing the nasolabial folds (Fig. 1A). Facial edema may also be present. ...
Article
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Cutaneous lupus erythematosus is an autoimmune disease of varied clinical expression, which may present as an exclusively cutaneous disease or be one of the multiple manifestations of systemic lupus erythematosus. Its classification includes acute, subacute, intermittent, chronic and bullous subtypes, which are usually identified based on clinical features and histopathological and laboratory findings. Other non-specific cutaneous manifestations may be associated with systemic lupus erythematosus and are usually related to disease activity. Environmental, genetic and immunological factors play a role in the pathogenesis of skin lesions in lupus erythematosus. Recently, considerable progress has been made in elucidating the mechanisms involved in their development, which allows for foreseeing future targets for more effective treatments. This review proposes to discuss the main etiopathogenic, clinical, diagnostic and therapeutic aspects of cutaneous lupus erythematosus, aiming to update internists and specialists from different areas.
... The generalized form of DLE has a higher potential of progressing to SLE compared to those with localized lesions (12,(19)(20)(21)(22). Potential risk factors for progression to systemic disease are suggested to be anemia, arthritis and positivity for ANA (19). DLE patients statistically have a lower risk of progression to or coexisting SLE compared to both ACLE and SCLE (23,24). ...
... This manifestation is usually triggered by UV irradiation although not exclusively. This so called "butterfly eruption" typically lasts from days to weeks and heals without scarring (24,32). The rare, generalized form is presented as a morbilliform widespread eruption (24,33). ...
... This so called "butterfly eruption" typically lasts from days to weeks and heals without scarring (24,32). The rare, generalized form is presented as a morbilliform widespread eruption (24,33). ACLE is often seen as a prodromal symptom of systemic disease and patients are usually positive for ANA (80%) and anti-dsDNA (30-40%) by this time (34). ...
Article
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Lupus erythematosus (LE) is an autoimmune inflammatory disease with a wide clinical spectrum from life-threatening multi-organ inflammation in systemic lupus erythematosus (SLE) to limited skin disease in cutaneous LE (CLE). The etiology of CLE is still not fully understood but a multifactorial genesis with genetic predisposition and certain environmental factors as triggers for the development are generally accepted features. Lesions can be induced and aggravated by UV-irradiation and smoking is linked to more severe forms of skin disease and to co-morbidity. Drugs, including many common medicines like antihypertensives, are known to induce subacute CLE (SCLE). The mechanisms involved have recently been shown to be part of the IFN-I pathway and new, specific treatments are currently in clinical trials. CLE is currently classified in subtypes based on clinical presentation and duration into acute CLE (ACLE), SCLE, and chronic CLE (CCLE). Distinct subtypes can be seen in individual patients or coexist within the same patient. Because of the confluent and overlapping picture between these subsets, serology, and histopathology constitute an important role guiding towards correct diagnose and there is ongoing work to update the classification. The Cutaneous Lupus Area Severity Index (CLASI) is a validated tool to measure activity and damage both in clinical trials but also for the clinician to evaluate treatment and follow the course of the disease among patients. CLE is known to have substantial impact on the life of those affected. Several tools have been proposed to measure QoL in these patients, currently Skindex-29 is probably the most used. Patient education is an important part of prevention of flares, including UV-protection and smoking cessation. First-line treatment includes topical corticosteroids as well as topical calcineurin inhibitors with the addition of systemic treatment with antimalarials in more severe or therapy resistant cases. Treatment specifically targeting CLE has been lacking, however novel potential therapies are in later phase clinical trials. In this review we aim to describe the different subsets of the cutaneous form in LE with focus on clinical aspects.