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Neutrophilic dermatoses (NDs) refer to a group of cutaneous conditions histologically characterized by the dense accumulation of neutrophils in the skin in the absence of infection. NDs have been associated with underlying autoimmune connective tissue disorders (CTDs) such as systemic lupus erythematosus (SLE), Sjogren's syndrome, and dermatomyositis. We describe a case of neutrophilic dermatoses as a manifestation of a SLE flare.

Discoid lupus erythematosus (DLE) is a devastating autoimmune disease with few therapies available. For patients with little to no symptom improvement with initial treatment, the literature surrounding further treatment options and their efficacy remains limited. Here we report a 46-year-old patient with lupus and refractory DLE, who failed numerous medications since her initial diagnosis in 2014. She had a robust response to lenalidomide with further improvement after adding anifrolumab (ANI), in conjunction with the standard of care hydroxychloroquine. Furthermore, she was able to taper off steroids without interval flares. The patient has not experienced any major infections since the initiation of treatment. No previous case reports describing outcomes of lenalidomide and ANI have been reported, yet the combinational approach has potential. Future clinical trials are needed to investigate the safety of the combination of lenalidomide and ANI in lupus patients with refractory DLE. [ABSTRACT FROM AUTHOR]

Introduction: Dermatomyositis, systemic and cutaneous lupus erythematosus have a significantly higher prevalence in women than men, emphasizing the relevance of exploring the relationship between sex hormones and autoimmune skin diseases. This review analyzes the interplay between sex hormones and these two skin diseases. Materials and methods: We performed an extensive literature search using the PubMed database from July to August 2023. Search terms included ‘contraceptives’, ‘pregnancy’, ‘hormone replacement’, ‘tamoxifen’, and ‘aromatase inhibitors’. Results and Discussion: This comprehensive literature review shows that there remains considerable debate regarding the use of hormonal contraceptives and hormonal replacement therapy in individuals with autoimmune skin conditions. Nonetheless, it is well established that their use is contraindicated in patients with antiphospholipid syndrome or when antiphospholipid antibodies are positive. Individuals experiencing disease flares and uncontrolled symptoms should also avoid these interventions. Pregnancy planning should be timed to coincide with well-managed disease states to minimize obstetric and neonatal complications. Hormonal breast cancer treatment requires close skin monitoring. Conclusion: Pregnancy, menopause, contraceptive use, hormone replacement therapy, and breast cancer treatment drugs result in substantial shifts in hormone levels. Additionally, hormone levels are altered by aromatase inhibitors and anti-estrogen medications. These fluctuations can modulate mechanisms influencing autoimmune skin abnormalities. [ABSTRACT FROM AUTHOR]

Abstract There is a need for training dermatologists in skin of colour's common and less common dermatoses due to the increasing diversity of the world population. The aim of this review is to highlight on cutaneous manifestations of systemic diseases in Afro‐Caribbean people, especially sarcoidosis, dermatomyositis, systemic sclerosis, systemic lupus.

TEN like Lupus erythematosus is an uncommon life-threatening variant of Lupus erythematosus. It is usually associated with flares of systemic lupus erythematosus and also because of widespread skin erosions, it can cause acute skin failure. It is often confused with drug induced TEN, however the management of both the diseases is different and hence correct diagnosis becomes crucial. In this study we aimed to assess the clinical characteristics and outcome of TEN like LE in the Indian population. All patients satisfying ACR/EULAR 2019 criteria for SLE and clinically diagnosed with TEN like LE were retrospectively reviewed. A total of 6 patients were identified. All patients were female. Except 1 patient who presented de-novo, the others had pre-existing symptoms of connective tissue disease. Half of the patients had palmoplantar involvement. Mucosal involvement was only mild. Majority had systemic involvement in the form of nephritis followed by arthralgia, autoimmune hepatitis and autoimmune hemolytic anemia. [ABSTRACT FROM AUTHOR]

In rare instances, patients with SLE may exhibit atypical clinical manifestations, such as Hypocomplementemic Urticarial Vasculitis, which can pose diagnostic challenges. Here, we present a case report of a 28-year-old female with a history of SLE with lupus nephritis clase IV who developed HUV-like symptoms, ultimately leading to a diagnosis of C1q Vasculitis. This case underscores the importance of considering C1q Vasculitis in SLE patients presenting with HUV-like features and highlights Rituximab as a promising therapeutic option for managing this rare condition. [ABSTRACT FROM AUTHOR]

Cutaneous lupus erythematosus (CLE) comprises dermatologic manifestations that may occur independently or with systemic lupus erythematosus (SLE). Despite advancements in refining CLE classification, establishing precise subtype criteria remains challenging due to overlapping presentations and difficulty in distinguishing morphology. Current treatments encompass preventive measures, topical therapies, and systemic approaches. Hydroxychloroquine and glucocorticoids are the sole US Food and Drug Administration (FDA)‐approved medications for CLE, with numerous off‐label treatments available. However, these treatments are often not covered by insurance, imposing a significant financial burden on patients. The exclusion of most CLE patients, particularly those without concurrent SLE, from trials designed for SLE has resulted in a lack of targeted treatments for CLE. To develop effective CLE treatments, validated outcome measures for tracking patient responsiveness are essential. The Cutaneous Lupus Erythematosus Disease Area and Severity Index is widely utilized for its reliability, validity, and ability to differentiate between skin activity and damage. In contrast, the FDA mandates the use of the Investigator's Global Assessment, a five‐point Likert scale related to lesion characteristics, for skin‐related therapeutic trials. It requires the disease to resolve or almost completely resolve to demonstrate improvement, which can be difficult when there is residual erythema or incomplete clearance that is meaningfully improved from a patient perspective. Various classes of skin lupus medications target diverse pathways, allowing tailored treatment based on the patient's lupus inflammatory profile, resulting in improved outcomes. Promising targeted therapeutic drugs include anifrolumab (anti‐type 1 interferon), deucravacitinib (allosteric tyrosine kinase 2 inhibitor), litifilimab (plasmacytoid dendritic cell‐directed therapy), iberdomide (cereblon‐targeting ligand), and belimumab (B‐cell directed therapy). Despite the significant impact of CLE on quality of life, therapeutic options remain inadequate. While promising treatments for cutaneous lupus are emerging, it is crucial to underscore the urgency for skin‐focused treatment outcomes and the implementation of validated measures to assess therapeutic effectiveness in clinical trials. [ABSTRACT FROM AUTHOR]

Abstract Objective To investigate the association between ultraviolet light index (UVI), as a marker for UV exposure, and seasonality with rash and systemic disease activity in youth with childhood-onset systemic lupus (cSLE) from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. Methods We reviewed data on rash and disease activity from Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K) scores from cSLE CARRA Registry participants with visits between 2010 and 2019 and obtained zipcode level UVI data from the National Oceanic and Atmospheric Administration (NOAA). Our main exposures were UVI and season during the month of visit and one month prior to visit. We used mixed-effects logistic regression models to examine associations between regional UVI (by zipcode)/season and odds of rash and severe SLEDAI-2 K score (≥ 5 vs. 0–4), adjusting for age, sex, race and income. Results Among 1222 participants, with a mean of 2.3 visits per participant, 437 visits (15%) had rash and 860 (30%) had SLEDAI-2 K score ≥ 5. There were no associations between UVI during the month prior to visit or the month of the visit and odds of rash or elevated systemic activity. However, fall season was associated with increased odds of rash (OR = 1.59, p = 0.04), but not increased disease activity. Conclusion This study found no association between UVI and rash or UVI and disease activity. However, further studies directly measuring UV exposure and accounting for patient-level protective behavioral measures may help to better understand the complex relationship between sun exposure and SLE disease activity.

Abstract A 39‐year‐old man presented with an acneiform facial eruption, bilateral vegetating erythematous plaques on the inguinal folds, focal palmoplantar keratoderma, keratotic violaceous plaques of the dorsum of both hands, and whitish striated lesions on the oral mucosa. Laboratory work‐up showed high titre of ANAs, anti‐SSA(Ro) and anti‐SSB(La) and hypocomplementemia (low C4). Three skin biopsies from different locations were suggestive of cutaneous lupus erythematosus (CLE). Treatment with hydroxychloroquine 400 mg/day plus topical and systemic corticosteroids led to significant clinical improvement after 2 months. Lupus erythematosus comedonicus is a form of discoid CLE and clinically characterized by acneiform lesions. Vegetating lesions, named as lupus erythematosus vegetans, are extremely rare in CLE. The combination of both manifestations in our patient may be better described as ‘lupus erythematous comedonicus and vegetans’.

AbstractIntroduction Dermatomyositis, systemic and cutaneous lupus erythematosus have a significantly higher prevalence in women than men, emphasizing the relevance of exploring the relationship between sex hormones and autoimmune skin diseases. This review analyzes the interplay between sex hormones and these two skin diseases.Materials and methods We performed an extensive literature search using the PubMed database from July to August 2023. Search terms included ‘contraceptives’, ‘pregnancy’, ‘hormone replacement’, ‘tamoxifen’, and ‘aromatase inhibitors’.Results and Discussion This comprehensive literature review shows that there remains considerable debate regarding the use of hormonal contraceptives and hormonal replacement therapy in individuals with autoimmune skin conditions. Nonetheless, it is well established that their use is contraindicated in patients with antiphospholipid syndrome or when antiphospholipid antibodies are positive. Individuals experiencing disease flares and uncontrolled symptoms should also avoid these interventions. Pregnancy planning should be timed to coincide with well-managed disease states to minimize obstetric and neonatal complications. Hormonal breast cancer treatment requires close skin monitoring.Conclusion Pregnancy, menopause, contraceptive use, hormone replacement therapy, and breast cancer treatment drugs result in substantial shifts in hormone levels. Additionally, hormone levels are altered by aromatase inhibitors and anti-estrogen medications. These fluctuations can modulate mechanisms influencing autoimmune skin abnormalities.

Objective: To investigate the association between ultraviolet light index (UVI), as a marker for UV exposure, and seasonality with rash and systemic disease activity in youth with childhood-onset systemic lupus (cSLE) from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. Methods: We reviewed data on rash and disease activity from Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K) scores from cSLE CARRA Registry participants with visits between 2010 and 2019 and obtained zipcode level UVI data from the National Oceanic and Atmospheric Administration (NOAA). Our main exposures were UVI and season during the month of visit and one month prior to visit. We used mixed-effects logistic regression models to examine associations between regional UVI (by zipcode)/season and odds of rash and severe SLEDAI-2 K score (≥ 5 vs. 0–4), adjusting for age, sex, race and income. Results: Among 1222 participants, with a mean of 2.3 visits per participant, 437 visits (15%) had rash and 860 (30%) had SLEDAI-2 K score ≥ 5. There were no associations between UVI during the month prior to visit or the month of the visit and odds of rash or elevated systemic activity. However, fall season was associated with increased odds of rash (OR = 1.59, p = 0.04), but not increased disease activity. Conclusion: This study found no association between UVI and rash or UVI and disease activity. However, further studies directly measuring UV exposure and accounting for patient-level protective behavioral measures may help to better understand the complex relationship between sun exposure and SLE disease activity. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Nuclear matrix protein (NXP‐2) positive amyopathic dermatomyositis (DM) may present without classic symptoms like muscle weakness, dysphagia, and edema, and mimic conditions like cutaneous lupus. Given DM's association with malignancy and interstitial lung disease, prompt and accurate diagnosis is important. Testing for myositis‐specific antibodies aids diagnosis in ambiguous cases. [ABSTRACT FROM AUTHOR]

Canine cutaneous lupus erythematosus (CCLE) is a well‐described, yet uncommon, autoimmune disease which can present clinically with different variants. This case report describes the clinical and histopathological presentation, and treatment response, of CCLE affecting a novel location, the interdigital skin, in two unrelated greyhounds. [ABSTRACT FROM AUTHOR]

Cutaneous lupus erythematosus (CLE) can present with a wide variety of dermatologic manifestations and is broken down into acute, subacute, and chronic subtypes. Tumid lupus, a variant of CLE, is rarely associated with systemic lupus erythematosus. Lesions of CLE and tumid lupus can mimic multiple other dermatologic conditions. We present a case of a patient with tumid lupus who was initially treated for erythema migrans and systemic symptoms suspected to represent Lyme disease. This article is intended for nurse practitioners, nurses, and other clinicians practicing in dermatology, primary care, urgent care, and emergency medicine settings in the care of patients presenting with annular skin lesions. [ABSTRACT FROM AUTHOR]

Cutaneous lupus erythematosus (CLE) is an autoimmune skin disease that can manifest itself with a variety of skin symptoms. Periorbital erythema, a rare variant of CLE, presents challenges in terms of diagnosis and treatment. Here, we report a case of CLE presenting with periorbital erythema and edema. A 42-year-old female patient presented with complaints of erythema, edema, and scaling on the right eyelid that started four months ago. A skin biopsy was performed on the lesioned skin of the eyelid to differentiate dermatomyositis, cutaneous lupus erythematosus, sarcoidosis, lupus vulgaris, and cutaneous lymphoma. Histopathological examination revealed focal hyperkeratosis and parakeratosis on the surface of the epidermis, vacuolar degeneration in the basal layer of the epidermis, lymphocyte exocytosis with necrotic keratinocytes, edema in the dermis, melanophages, and perivascular, periadnexal lymphocytic reaction. Laboratory tests showed negative antinuclear antibody and anti-dsDNA, but positivity for anti-Ro-52. In the absence of any other complaints, the patient was diagnosed with cutaneous lupus erythematosus presenting with periorbital erythema based on clinical, histopathological, and laboratory findings. Hydroxychloroquine 200 mg/day, topical corticosteroid, and topical tacrolimus were administered. Two months later, significant improvement in the lesions was observed. In conclusion, it should be kept in mind that periorbital erythema can develop as a rare variant of CLE and can be misdiagnosed as contact dermatitis, dermatomyositis, sarcoidosis, or cutaneous lymphoma. Additionally, the ANA and anti-dsDNA antibodies are often found to be negative in these cases. In establishing the diagnosis, firstly considering the disease, followed by histopathological examinations and laboratory tests, is crucial. [ABSTRACT FROM AUTHOR]

Key Clinical Message Nuclear matrix protein (NXP‐2) positive amyopathic dermatomyositis (DM) may present without classic symptoms like muscle weakness, dysphagia, and edema, and mimic conditions like cutaneous lupus. Given DM's association with malignancy and interstitial lung disease, prompt and accurate diagnosis is important. Testing for myositis‐specific antibodies aids diagnosis in ambiguous cases.

Cutaneous lupus erythematosus (CLE), the main manifestation of systemic lupus erythematosus (SLE), is driven by type I interferons (IFNs) and often only partially responds to conventional therapies. Treatment of seven SLE patients with the monoclonal antibody anifrolumab induced fast and sustained remission of previously refractory CLE lesions, beginning within the first weeks of treatment. Decline in CLASI-A score was paralleled by a reduction in IFN score determined by mRNA expression of seven IFN-stimulated genes (ISGs) in blood. These data suggest that a subset of ISGs could be a valuable biomarker in CLE. [ABSTRACT FROM AUTHOR]

Background: Anti‐tumour necrosis factor alpha (anti‐TNF) agents are a highly effective treatment for inflammatory bowel disease (IBD). Skin lesions, including psoriasiform, eczematous and lupoid eruptions, may paradoxically result from anti‐TNF use and cause significant morbidity leading to discontinuation of therapy. There are no consensus guidelines on the management of these lesions. Aims: This systematic review considers the existing evidence regarding cutaneous complications of anti‐TNF therapy in IBD and the development of an algorithm for management. Methods: A systematic review was performed by searching Medline (Pubmed) and Embase for articles published from inception to January 2021. The following search terms were used 'anti‐tumour necrosis factor alpha', 'infliximab', 'adalimumab', 'certolizumab', 'golimumab', 'inflammatory bowel disease', 'Crohn disease', 'Ulcerative colitis', 'psoriasis', 'psoriasiform', 'dermatitis', 'lupus', 'skin lesion' and 'skin rash'. Reference lists of relevant studies were reviewed to identify additional suitable studies. Results: Thirty‐four studies were included in the review. Eczema can generally be managed with topical agents and the anti‐TNF can be continued, while the development of lupus requires immediate cessation of the anti‐TNF and consideration of alternative immunomodulators. Management of psoriasis and psoriasiform lesions may follow a step‐wise algorithm where topical treatments will be trialled in less severe cases, with recourse to an alternative anti‐TNF or a switch to an alternative class of biological agent. Conclusion: Assessment of anti‐TNF skin lesions should be performed in conjunction with a dermatologist and rheumatologist in complex cases. High‐quality prospective studies are needed to clarify the validity of these algorithms in the future. [ABSTRACT FROM AUTHOR]

Acute cutaneous lupus erythematosus (ACLE) is a variant of acute autoimmune disease with distinctive cutaneous manifestations. In addition to its typical manifestations, it can also manifest as bullous lesion, which can be difficult to distinguish clinically from other dermatological disorders. We reported a 40-year-old lady who presented with painful vesicobullous eruptions on malar region since 1 week ago. Patient refused to be examined further and not compliance to the treatment given. She then consumed traditional medicine and admitted to the hospital 2 weeks later with extensive vesicobullous lesions in the face, scalp, trunk and extremities. Patient showed complete remission and excellent clinical response after corticosteroid treatment. This report showed the importance of early diagnosis as well as prompt treatment can result in a better prognosis. [ABSTRACT FROM AUTHOR]

Cutaneous lupus erythematosus (CLE), the main manifestation of systemic lupus erythematosus (SLE), is driven by type I interferons (IFNs) and often only partially responds to conventional therapies. Treatment of seven SLE patients with the monoclonal antibody anifrolumab induced fast and sustained remission of previously refractory CLE lesions, beginning within the first weeks of treatment. Decline in CLASI-A score was paralleled by a reduction in IFN score determined by mRNA expression of seven IFN-stimulated genes (ISGs) in blood. These data suggest that a subset of ISGs could be a valuable biomarker in CLE.

Key Clinical Message: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease that affects several organs including the skin. Clinical cutaneous symptoms of SLE come in a broad range, consisting of both non‐specific and specific cutaneous lesions. Except for cases of amicrobial pustulosis of the folds, generalized pustular psoriasis, acneiform eruptions, pustular vasculitis, Wells' syndrome, subcorneal pustular dermatosis, and neutrophilic dermatosis, there are no reports of pustular lesions linked to SLE. The unusual cutaneous features of our patient were annular plaques with pustules and crusts on the margins. [ABSTRACT FROM AUTHOR]

Background: Cutaneous lupus erythematosus is an umbrella term for a group of autoimmune connective tissue disorders affecting the skin. Discoid lupus erythematosus (DLE) is the chronic condition and most common form of cutaneous lupus erythematosus. Aims: Current therapies of DLE are challenging and not completely satisfactory, highly expensive, off‐label, or poorly available (like antimalarials due to COVID‐19 outbreaks). Nicotinamide, also called niacinamide, is a water‐soluble form of vitamin B3 (niacin). Its multiple effects let us think that nicotinamide could be a therapy for lupus‐associated skin lesions. Methods: We performed a prospective randomized double‐blind clinical trial on 60 subjects diagnosed with Discoid lupus erythematosus using topical Nicotinamide 2% and 4% preparations in form of cream and gel on skin and scalp lesions. Control group was included using only cream/gel base as placebo control. Results: Obtained data showed that topical Nicotinamide can be used for the treatment of DLE as adjuvant to other treatment regimens with good cosmetic results and minimal side effects. Topical 4% Nicotinamide is superior to 2% preparation in response but associated with a higher incidence of irritation. Conclusion: Topical Nicotinamide can be used for the treatment of DLE as an adjuvant to other treatment regimens with good cosmetic results and minimal side effects. Further trials with long‐term therapy, follow‐up period, and bigger sample sizes are required. [ABSTRACT FROM AUTHOR]

Key Clinical Message Systemic lupus erythematosus (SLE) is a systemic autoimmune disease that affects several organs including the skin. Clinical cutaneous symptoms of SLE come in a broad range, consisting of both non‐specific and specific cutaneous lesions. Except for cases of amicrobial pustulosis of the folds, generalized pustular psoriasis, acneiform eruptions, pustular vasculitis, Wells' syndrome, subcorneal pustular dermatosis, and neutrophilic dermatosis, there are no reports of pustular lesions linked to SLE. The unusual cutaneous features of our patient were annular plaques with pustules and crusts on the margins.

Summary: Background: Autoimmune skin diseases can expedite various systemic sequelae involving other organs. Although limited to the skin, cutaneous lupus erythematosus (CLE) was noted to be associated with thromboembolic diseases. However, small cohort sizes, partially discrepant outcomes, missing data on CLE subtypes, and incomplete risk assessment limits these findings. Methods: The Global Collaborative Network of TriNetX provides access to medical records of more than 120 million patients worldwide. We used TriNetX to elucidate the risk for cardiac and vascular diseases after diagnosis of CLE, and its subtypes chronic discoid (DLE) and subacute cutaneous lupus erythematosus (SCLE). We included 30,315 CLE, 27,427 DLE, and 1613 SCLE patients. We performed propensity-matched cohort studies determining the risk to develop cardiac and vascular diseases (ICD10CM:I00-99) following diagnosis of CLE, DLE, or SCLE. Patients with systemic lupus erythematosus were excluded. Findings: We document that CLE and its subtype DLE but less so SCLE are associated with a higher risk for various cardiac and vascular diseases. This included predominantly thromboembolic events such as pulmonary embolism, cerebral infarction, and acute myocardial infarction, but also peripheral vascular disease and pericarditis. For example, the hazard ratio of arterial embolism and thrombosis was 1.399 (confidence interval: 1.230–1.591, p

We describe a case of a male patient with systemic lupus erythematosus (SLE) and lupus nephritis. A patient who was initially diagnosed with multibacillary leprosy, an infectious disease, with clinical symptoms for two years. However, after hospitalization and investigation, his diagnosis was revoked and replaced with SLE. The aim of this study is to emphasize the importance of knowing the most important and significant clinical changes in SLE and thus allowing an accurate diagnosis, preventing disease progression with target organ involvement, and allowing better clinical management. [ABSTRACT FROM AUTHOR]

Inflammation driven by immune-mediated mechanisms can lead to a wide variety of skin manifestations. Cutaneous lupus erythematosus (CLE), for example, is an autoimmune connective tissue disease that may be limited to the skin or be part of the widespread multiorgan involvement seen in systemic lupus erythematosus. Clinical findings are divided into lupus-specific (acute, subacute, and chronic) and nonspecific skin lesions. The diagnosis of CLE requires a thorough physical examination and, in some cases, skin biopsy and laboratory evaluation for any underlying systemic involvement. CLE treatment is dependent on the cutaneous manifestations and severity of disease. In addition to lifestyle measures, particularly avoidance of sunlight, topical and systemic therapies have proven beneficial. Prognosis is varied by disease severity, although chronic CLE notoriously results in cosmetic damage. Early recognition and management of CLE is important as it may also be the presenting manifestation of systemic lupus erythematosus. Patient care includes education and close collaboration with primary care providers, dermatologists, and rheumatologists. [ABSTRACT FROM AUTHOR]

Lupus mastitis is a rare clinical manifestation associated with systemic lupus erythematosus or discoid lupus erythematosus. It is necessary to make a correct diagnosis to differentiate it from inflammatory breast cancer. The histological study shows involvement of the adipose tissue of the breast with histopathological findings of cutaneous lupus erythematosus. Direct immunofluorescence detects the lupus band at the dermal-epidermal junction. The treatment of choice is hydroxychloroquine. We present a case of unilateral lupus mastitis in a patient with no previous diagnosis of lupus with complete remission after the use of hydroxychloroquine and topical corticosteroids. [ABSTRACT FROM AUTHOR]

Cutaneous lupus erythematosus (CLE) is a chronic autoimmune skin disease with potential for systemic involvement, disfigurement, and significant disease burden. The relationships of demographics and socioeconomic status with patients with CLE are emerging topics with important clinical implications. The primary objective of our study is to perform a literature review of studies that have investigated demographic and socioeconomic factors amongst patients with CLE and determine whether these factors influence diagnosis frequency, disease severity and outcomes or health related quality of life. We searched multiple databases to identify literature addressing CLE and concepts such as race, ethnicity, gender, income, education level and geographic location. Information regarding primary research objective was extracted from all full text articles, and a summary of findings was prepared. We found that race and ethnicity can influence CLE diagnosis frequency and disease outcomes. Chronic cutaneous lupus (CCLE) occurs more frequently in Black patients, often with higher overall disease damage. Differences between genders exist in CLE in terms of health-related quality of life, as female gender was a risk factor for worse quality of life in several studies. Lower income, low educational attainment, and lack of health insurance all contribute to poorer overall outcomes in CLE patients. This review will help inform physicians about populations at risk for potentially worse outcomes to guide treatment decisions for patients with CLE and provide important information to design interventions that address modifiable social determinants of health in this population.

OBJECTIVE: To describe the frequency and the clinical and immunological characteristics of systemic lupus erythematosus applying the 2019 EULAR/ACR classification criteria. MATERIALS AND METHODS: A retrospective study was done describing the frequency and the clinical and immunological characteristics of patients with systemic lupus erythematosus care in the internal medicine and rheumatology service from 2014 to 2020. The cut-off points for the individual diagnosis of lupus was ≥ 10 points. The presence of antinuclear antibodies was mandatory. RESULTS: There were included 132 patients, 116 women and 16 men with a 7:1 ratio. The median age was 35 years (range: 12 to 58); 77% had arthritis, 71% leukopenia, 59% acute cutaneous lupus, 41% oral ulcers and 32% fever. Nephropathy was observed in 28%, determined by proteinuria or renal biopsy; in 13 cases the histopathological study revealed type III/IV nephritis and in one case type II/V nephritis (ISN/RPS); 78% had anti-DNA native and 20% anti-Sm/RNP. The decrease in complement was found in 22% of the cases. CONCLUSIONS: The new classification criteria of EULAR/ACR 2019 for systemic lupus erythematosus are based on previous criteria, except for fever. All our patients fulfilled 10 or more points to meet the classification criteria. [ABSTRACT FROM AUTHOR]

Introduction: Dermatomyositis, systemic and cutaneous lupus erythematosus have a significantly higher prevalence in women than men, emphasizing the relevance of exploring the relationship between sex hormones and autoimmune skin diseases. This review analyzes the interplay between sex hormones and these two skin diseases., Materials and Methods: We performed an extensive literature search using the PubMed database from July to August 2023. Search terms included 'contraceptives', 'pregnancy', 'hormone replacement', 'tamoxifen', and 'aromatase inhibitors'., Results and Discussion: This comprehensive literature review shows that there remains considerable debate regarding the use of hormonal contraceptives and hormonal replacement therapy in individuals with autoimmune skin conditions. Nonetheless, it is well established that their use is contraindicated in patients with antiphospholipid syndrome or when antiphospholipid antibodies are positive. Individuals experiencing disease flares and uncontrolled symptoms should also avoid these interventions. Pregnancy planning should be timed to coincide with well-managed disease states to minimize obstetric and neonatal complications. Hormonal breast cancer treatment requires close skin monitoring., Conclusion: Pregnancy, menopause, contraceptive use, hormone replacement therapy, and breast cancer treatment drugs result in substantial shifts in hormone levels. Additionally, hormone levels are altered by aromatase inhibitors and anti-estrogen medications. These fluctuations can modulate mechanisms influencing autoimmune skin abnormalities.

Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by diverse clinical manifestations affecting multiple organs and systems. The understanding of genetic factors underlying the various manifestations of SLE has evolved considerably in recent years. This review provides an overview of the genetic implications in some of the most prevalent manifestations of SLE, including renal involvement, neuropsychiatric, cutaneous, constitutional, musculoskeletal, and cardiovascular manifestations. We discuss the current state of knowledge regarding the genetic basis of these manifestations, highlighting key genetic variants and pathways implicated in their pathogenesis. Additionally, we explore the clinical implications of genetic findings, including their potential role in risk stratification, prognosis, and personalized treatment approaches for patients with SLE. Through a comprehensive examination of the genetic landscape of SLE manifestations, this review aims to provide insights into the underlying mechanisms driving disease heterogeneity and inform future research directions in this field., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Cutaneous lupus erythematosus (CLE) is a chronic inflammatory skin disease characterized by a diverse cadre of clinical presentations. CLE commonly occurs in patients with systemic lupus erythematosus (SLE), and CLE can also develop in the absence of systemic disease. Although CLE is a complex and heterogeneous disease, several studies have identified common signaling pathways, including those of type I interferons (IFNs), that play a key role in driving cutaneous inflammation across all CLE subsets. However, discriminating factors that drive different phenotypes of skin lesions remain to be determined. Thus, we sought to understand the skin-associated cellular and transcriptional differences in CLE subsets and how the different types of cutaneous inflammation relate to the presence of systemic lupus disease. In this study, we utilized two distinct cohorts comprising a total of 150 CLE lesional biopsies to compare discoid lupus erythematosus (DLE), subacute cutaneous lupus erythematosus (SCLE), and acute cutaneous lupus erythematosus (ACLE) in patients with and without associated SLE. Using an unbiased approach, we demonstrated a CLE subtype-dependent gradient of B cell enrichment in the skin, with DLE lesions harboring a more dominant skin B cell transcriptional signature and enrichment of B cells on immunostaining compared to ACLE and SCLE. Additionally, we observed a significant increase in B cell signatures in the lesional skin from patients with isolated CLE compared with similar lesions from patients with systemic lupus. This trend was driven primarily by differences in the DLE subgroup. Our work thus shows that skin-associated B cell responses distinguish CLE subtypes in patients with and without associated SLE, suggesting that B cell function in skin may be an important link between cutaneous lupus and systemic disease activity. [ABSTRACT FROM AUTHOR]

Background: Patients with skin of color (P-SOC) are disproportionately burdened by lupus and often have worse disease outcomes than white patients. This is partly because educational materials underrepresent P-SOC, thereby promoting unconscious bias and clinical deficiencies among practitioners. Purpose: We sought to measure providers' confidence in diagnosing the cutaneous manifestations of lupus (i.e., lupus-related rashes) in P-SOC and to assess which factors influenced their confidence. Research Design: We created and distributed a survey that gathered information about participants' personal characteristics, clinical specialty, training, and current practice as well as measuring their confidence assessing lupus-related rashes in various skin tones. Study Sample: Practitioners from the fields of rheumatology, dermatology, and internal medicine in the greater St. Louis area (Missouri, USA) participated in the survey. Analysis: We compared practitioners' mean confidence levels assessing lupus-related rashes in patients with fair skin and P-SOC with a linear mixed effects model and used univariate and multivariate linear regression models to determine if the aforementioned factors correlated with confidence. Results: Participants' mean confidence in diagnosing lupus-related rashes in P-SOC was significantly lower than assessing such findings in patients with fair skin (p =.009). Several factors correlated with confidence level at a univariate level; however, the multivariate model revealed experience as the only factor significantly associated with confidence (p =.001). Conclusions: Providers report significantly less confidence assessing lupus-related rashes in P-SOC than in patients with fair skin. Our analysis demonstrates that experience positively correlates with confidence and suggests that interventions which enhance practitioners' exposure to and experience with these rashes in P-SOC can improve clinical confidence as well as patient outcomes. [ABSTRACT FROM AUTHOR]

Cutaneous lupus erythematosus (CLE) is a chronic inflammatory skin disease characterized by a diverse cadre of clinical presentations. CLE commonly occurs in patients with systemic lupus erythematosus (SLE), and CLE can also develop in the absence of systemic disease. Although CLE is a complex and heterogeneous disease, several studies have identified common signaling pathways, including those of type I interferons (IFNs), that play a key role in driving cutaneous inflammation across all CLE subsets. However, discriminating factors that drive different phenotypes of skin lesions remain to be determined. Thus, we sought to understand the skin-associated cellular and transcriptional differences in CLE subsets and how the different types of cutaneous inflammation relate to the presence of systemic lupus disease. In this study, we utilized two distinct cohorts comprising a total of 150 CLE lesional biopsies to compare discoid lupus erythematosus (DLE), subacute cutaneous lupus erythematosus (SCLE), and acute cutaneous lupus erythematosus (ACLE) in patients with and without associated SLE. Using an unbiased approach, we demonstrated a CLE subtype-dependent gradient of B cell enrichment in the skin, with DLE lesions harboring a more dominant skin B cell transcriptional signature and enrichment of B cells on immunostaining compared to ACLE and SCLE. Additionally, we observed a significant increase in B cell signatures in the lesional skin from patients with isolated CLE compared with similar lesions from patients with systemic lupus. This trend was driven primarily by differences in the DLE subgroup. Our work thus shows that skin-associated B cell responses distinguish CLE subtypes in patients with and without associated SLE, suggesting that B cell function in skin may be an important link between cutaneous lupus and systemic disease activity.

Vesiculobullous lesions can be developed by less than 5% of patients with systemic lupus erythematosus (SLE). Among these lesions, bullous systemic lupus erythematosus (BSLE) is one of the rarest and most challenging cutaneous presentations of the disease. Its frequency in the largest series of 5149 patients with cutaneous lupus was 0.19%, and the majority of cases are reported as sporadic observation or small series not exceeding ten cases. We report the original observation of BSLE revealing SLE in 32-year-old Tunisian Woman. This atypical presentation of cutaneous lupus deserves to be known by clinicians and discussed in front of any bullous dermatosis, particularly in young woman.

Cutaneous manifestations of systemic lupus erythematosus (SLE) are important diagnostic criteria as described by the revised American College of Rheumatology and Systemic Lupus International Collaborating Clinics. Patients usually present with various lupus-specific cutaneous manifestations such as malar rash, discoid rash, photosensitivity, and alopecia. Acneiform presentation of SLE is extremely rare. Review of the literature using PubMed has found only nine other reports so far. Here, we report a case who was initially treated as acne and later diagnosed as SLE.

It is now two decades since Rituximab was first used in the treatment of patients with systemic lupus erythematosus. There have been many challenges but in spite of failing to meet its primary endpoints in two clinical trials it is widely used for many aspects of lupus, its side-effects and the possibility that combining it with Benlysta may be of value. We also consider the proposal that it may provide a useful initial therapy. In this review, we consider the place of Rituximab in the treatment of lupus and anticipate how developments in fully-humanized anti-CD20 monoclonals may well extend the "therapeutic life" of B-cell depletion as a viable treatment option. [ABSTRACT FROM AUTHOR]

Background: Leptin is an adipokine that has an important role in body weight regulation and endocrine function. Leptin also acts as a pro‑inflammatory cytokine by inducing the production of Th1 cytokines. The aim of our study was to estimate the serum leptin levels in systemic lupus erythematosus (SLE) patients and analyze its associations with clinical manifestations of lupus and disease activity. Materials and Methods: This study was conducted on 80 patients who satisfied Systemic Lupus International Collaborating Clinics 2012 criteria for SLE and 40 healthy controls. Demographic, clinical, and laboratory parameters were recorded. SLE disease activity index (SLEDAI) was scored for all SLE patients. Serum leptin levels (ng/dl) were estimated by enzyme‑linked immunosorbent assay and the results were analyzed by SPSS. Results: Baseline characteristics were comparable in both cases and controls. SLE patients had higher serum leptin levels as compared to controls (mean 16.01 vs. 5.86 ng/ml) which were statistically significant (P < 0.00001). Cutaneous lupus (85%) was the most common manifestation observed in patients with elevated leptin levels. Patients with lupus nephritis had high serum leptin levels (P = 0.04) in comparison to patients with nonrenal lupus. Serum leptin levels correlated positively with higher body mass index (BMI), higher uric acid levels, and lower high‑density lipoproteins (HDL) cholesterol levels (P < 0.0001). Serum leptin levels showed no correlation with disease activity markers such as erythrocyte sedimentation rate, C‑reactive protein, anti‑double‑stranded deoxyribonucleic acid titers, complements C3 and C4, and SLEDAI. Conclusion: Patients with SLE had high serum leptin levels. High leptin levels were seen predominantly in mucocutaneous lupus and lupus nephritis. Serum leptin levels correlated positively with BMI and serum uric acid and negatively with serum HDL cholesterol levels but showed no correlation with disease activity markers. [ABSTRACT FROM AUTHOR]

Neutrophilic dermatoses (NDs) refer to a group of cutaneous conditions histologically characterized by the dense accumulation of neutrophils in the skin in the absence of infection. NDs have been associated with underlying autoimmune connective tissue disorders (CTDs) such as systemic lupus erythematosus (SLE), Sjogren's syndrome, and dermatomyositis. We describe a case of neutrophilic dermatoses as a manifestation of a SLE flare., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflict of interest with respect to the research, authorship, and/or publication of this article.

Thalidomide is effective in patients with refractory cutaneous lupus erythematosus (CLE). However, the mechanism of action is not completely understood, and its use is limited by its potential, severe side-effects. Immune cell subset analysis in thalidomide’s CLE responder patients showed a reduction of circulating and tissue cytotoxic T-cells with an increase of iNKT cells and a shift towards a Th2 response. We conducted an RNA-sequencing study using CLE skin biopsies performing a Therapeutic Performance Mapping System (TMPS) analysis in order to generate a predictive model of its mechanism of action and to identify new potential therapeutic targets. Integrating RNA-seq data, public databases, and literature, TMPS analysis generated mathematical models which predicted that thalidomide acts via two CRBN-CRL4A- (CRL4CRBN) dependent pathways: IRF4/NF-ҡB and AMPK1/mTOR. Skin biopsies showed a significant reduction of IRF4 and mTOR in post-treatment samples by immunofluorescence. In vitro experiments confirmed the effect of thalidomide downregulating IRF4 in PBMCs and mTOR in keratinocytes, which converged in an NF-ҡB reduction that led to a resolution of the inflammatory lesion. These results emphasize the anti-inflammatory role of thalidomide in CLE treatment, providing novel molecular targets for the development of new therapies that could avoid thalidomide’s side effects while maintaining its efficacy.