Subacute Lupus Research Articles

Case study of patient with diabetic nephropathy and emerging subacute cutaneous lupus erythematosus

Introduction: Diabetes is one of the chronic diseases spread worldwide. Diabetic kidney disease (DKD) also known as Diabetic Nephropathy, is a chronic, progressive disease that is the most common cause of end-stage renal failure. High BP with chronic blood glucose level damage tiny blood vessels in the kidney and their function. Cellular degradation in the nephron and podocytes of the renal glomeruli, contributes to the impairment of renal function. As diabetic nephropathy majorly seen in aged person precaution should be taken while selection of drug. Case presentation: The patient, in this report, is a 60-year-old male who was working as driver. Patient having diabetic history in their family. His mother has diabetes mellitus. He is with 10 years history of both hypertension and diabetes mellitus. Then in 2021 he was also infected by COVID-19 during pandemic. After few weeks of recovery patient start experiencing swelling feet and lower legs during night time. Blood test reveals elevated creatinine level and patient suffered rapid progression of DKD. The condition known as diabetic nephropathy. Despite aggressive treatment, the patient finally became dialysis dependent. Now past few weeks the patient is also suffered from newly developing skin disease. His skin become getting darker. Histomorphological features suggestive of Subacute Lupus Erythematosus. Results and discussion: Now patient is following regular medication and diet. Patient taking doctor’s monthly appointment and following maintenance dialysis three times a week.

Plasmacytoid Dendritic Cells Are Not Major Producers of Type 1 IFN in Cutaneous Lupus: An In-Depth Immunoprofile of Subacute and Discoid Lupus

The immunologic drivers of cutaneous lupus erythematosus (CLE) and its clinical subtypes remains poorly understood. We sought to characterize the immune landscape of discoid lupus erythematosus (DLE) and subacute CLE (SCLE) using multiplexed immunophenotyping. We found no significant differences in immune cell percentages between DLE and SCLE (p>0.05) with the exception of an increase in TANK binding kinase 1 (TBK1) in DLE (p<0.05). Unbiased clustering grouped subjects into two major clusters without respect to clinical subtype. Subjects with a history of smoking had increased percentages of neutrophils, disease activity, and endothelial granzyme B than non-smokers. Despite previous assumptions, plasmacytoid dendritic cells (pDCs) did not stain for type 1 interferon (IFN-1). Skin-eluted and circulating pDCs from CLE subjects expressed significantly less IFNα than healthy control pDCs upon toll like receptor (TLR) 7 stimulation ex vivo (p<0.0001). These data suggest that DLE and SCLE have similar immune microenvironments in a multiplexed investigation. Our aggregated analysis of CLE revealed that smoking may modulate disease activity in CLE via neutrophils and endothelial GZMB. Notably, our data suggest that pDCs are not the major producers of IFN-1 in CLE. Future in vitro studies to investigate the role of pDCs in CLE are needed.

Role of neutrophils in cutaneous lupus erythematosus.

There are various types of cutaneous lupus erythematosus (CLE), either with or without the association of systemic lupus erythematosus (SLE). In some of the subtypes of cutaneous lupus, histopathology reveals neutrophil infiltration in the lesional skin; however, the significance of neutrophils in CLE is not yet fully elucidated. Recent studies have shown that neutrophil extracellular traps (NETs) formation by activated neutrophils is observed in several types of CLE, including lupus panniculitis, subacute lupus erythematosus, and acute lupus erythematosus, although the number of reports is small. Excessive NETosis, due to either increased NETs formation or defective clearance of NETs, may play a role in the induction of autoimmunity and autoantibody production in SLE, as well as endothelial damage, thrombus formation, and vascular damage in the lesional skin. CLE is an excessive interferon-driven autoimmune disease. Plasmacytoid dendritic cells are located in lupus erythematosus skin and contribute to the etiology of skin lesions as a main producing cell of type I interferon. Neutrophils, monocytes, and keratinocytes also produce type I interferon via several triggers. Neutrophils play an important role in the innate immune response in SLE. In this review, several types of CLE with neutrophil infiltration, as well as the role of neutrophils are discussed.

Cutaneous Toll-like Receptor 9 Pre-Defines Hydroxychloroquine Dosage in Patients with Both Discoid and Subacute Lupus Erythematosus.

Background and Objectives: Cutaneous lupus erythematosus (CLE) presents clinically heterogeneous manifestations, partially explained by the different expression of Toll-like receptors (TLRs) type 8 and 9, located to endosomal compartments where they are poised to recognize microbial nucleic acids. This disease is empirically treated with hydroxychloroquine (HCQ), which is hallmarked with a safe and effective profile, but induces a slow and sometimes clinically insufficient therapeutic response. Currently, no biomarkers predictive of response are validated or even proposed in the scientific literature. We aimed to evaluate endosomal TLR type 7, 8 and 9 as predictive biomarkers of HCQ efficacy. Materials and Methods: We conducted a case-control study comparing CLE patients retrospectively assigned to three subgroups based on 3-6-month Cutaneous LE Disease Area and Severity Index (CLASI) reduction upon treatment with HCQ (I = <40% vs. II = 40-80% vs. III = >80%). Before HCQ, lesional skin specimens were collected in untreated CLE and through immunohistochemistry; TLR-7, -8 and -9 expression was evaluated in the epidermis and the lymphocytic infiltrate was evaluated in the dermis. Results: Sixty-six lesional skin biopsies were compared with healthy controls. CLE patients displayed lower epidermal expression of total TLR 8 and 9 as well as infiltrating TLR-8, TLR9 + lymphocytes compared to controls. High HCQ responders differed from low responders for TLR-9 positivity (high vs. low) and for the lymphocytic dermal infiltrate (high vs. low). Conclusions: TLR9 could be envisaged as a possible biomarker to predict HCQ response level and dosage in CLE patients.

ЛЕКАРСТВЕННО-ИНДУЦИРОВАННАЯ КРАСНАЯ ВОЛЧАНКА

Medicinal or drug-induced lupus erythematosus (DIL) is an autoimmune phenomenon, which is clinically and morphologically similar to idiopathic lupus erythematosus, that occurs in patients during the long-term use of drugs, the list of which currently includes more than 100 items. To date, the pathogenesis of DIL has not been fully deciphered, and diagnostic criteria have not been developed, which determines the difficulties of diagnosis and determination of therapeutic approaches. At the same time, the widespread introduction of new classes of drugs, in particular, TNF inhibitors and other genetically engineered biological drugs associated with the risk of developing a systemic form of DIL, the use of proton pump inhibitors and chemotherapy drugs that induce subacute lupus erythematosus, allow predicting an increase in the prevalence of DIL, including in children, which in its turn determines the relevance of the problem of DIL in pediatric practice. This bibliographical review summarizes the current data on drugs that are potential inducers of DIL, possible mechanisms of development and clinical forms of the disease, principles of diagnosis and therapy.

IL-1β and IL-17 in cutaneous lupus erythematous skin biopsies: could immunohistochemicals indicate a tendency towards systemic involvement?

BackgroundOnly a fraction of patients with cutaneous lupus erythematosus (CLE) will eventually progress toward systemic disease (SLE). ObjectiveTo find inflammatory biomarkers which could predict the progression of cutaneous lupus erythematosus (CLE) into systemic lupus erythematosus (SLE) using immunohistochemical (IHC) assays. MethodsImmunohistochemical markers for cytotoxic, inflammatory, and anti-inflammatory responses and morphometric methods were applied to routine paraffin sections of skin biopsies, taken from lesions of 59 patients with discoid lupus, subacute lupus, and lupus tumidus. For the diagnosis of SLE, patients were classified by both the American College of Rheumatology (ACR-82) and the Systemic Lupus International Collaborating Clinics (SLICC-12) systems. ResultsSkin samples from CLE/SLE+patients presented higher expression of IL-1β (ARC-82: p=0.024; SLICC-12: p=0.0143) and a significantly higher number of cells marked with granzyme B and perforin (ARC: p=0.0097; SLICC-12: p=0.0148). Biopsies from CLE/SLE- individuals had higher expression of IL-17 (ARC-82: p=0.0003; SLICC-12: p=0.0351) and presented a positive correlation between the density of granzyme A+and FoxP3+ cells (ARC-82: p=0.0257; SLICC-12: p=0.0285) and CD8+ cells (ARC-82: p=0.0075; SLICC-12: p=0.0102), as well as between granulysin-positive and CD8+ cells (ARC-82: p=0.0024; SLICC-12: p=0.0116). Study limitationsPatients were evaluated at a specific point in their evolution and according to the presence or not of systemic disease. The authors cannot predict how many more, from each group, would have evolved towards SLE in the following years. ConclusionsIn this cohort, immunohistochemical findings suggested that patients with a tendency to systemic disease will show strong reactivity for IL-1β, while those with purely cutaneous involvement will tend to express IL-17 more intensely.

Translated article] Melanocytic Nevus in Subacute Lupus Erythematosus: An Unexpected Association

Translated article] Melanocytic Nevus in Subacute Lupus Erythematosus: An Unexpected Association

Pattern of systemic lupus erythematosus in NIGERIA: a multicentre descriptive hospital-based study.

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease with manifestations ranging from mild to life-threatening organ dysfunction. There is wide variability in the reported incidence and prevalence rate globally, particularly in low and middle-income countries. Nigeria had very few isolated reports of SLE from private and public hospitals Therefore, we conducted this large multi-center descriptive study to determine the sociodemographic, clinical profile, laboratory patterns, and treatment among Nigerian lupus patients. A retrospective hospital-based study of all SLE patients seen over 4years (January 2017 to December 2020) was conducted at 20 rheumatology clinics spread across the 6 geopolitical zones of Nigeria. All patients 18years and above satisfying the American College of Rheumatology (ACR) 1997 and/or the Systemic Lupus International Collaboration Clinics (SLICC) 2012 classification criteria for SLE were enrolled. Patients with other Rheumatic and Musculoskeletal Diseases (RMDs) not in keeping with SLE and Patients with incomplete data were excluded. Data was analysed using SPSS version 23.0 software. A total of 896 patients with SLE were included in the final analysis with a mean age ± SD of 34.47 ± 11 and a female to male ratio of 8.1:1. Synovitis was reported by 61.6% of patients, while 51%, 19.9% and11.4% patients reported acute, sub-acute and chronic lupus rashes respectively. ANA was positive in 98.0% with titers ranging from 1:80 to 1:64,000. SLE is not rare in Nigeria. Most patients were female in their 3rd to 4th decades of life. There is a delayed presentation to a rheumatology facility. Arthritis and mucocutaneous manifestations were the most frequent presentation. Key Points •This study presents the first national data on SLE in Nigeria •This study showed that SLE is not rare in Nigeria in contrast to previous reports •There appear to be ethnic disparity in the frequency of lupus among Nigerians •Nigerians with lupus have very high titer of ANA.

Cutaneous Adverse Reactions of Immunotherapy in Patients with Advanced Melanoma.

Immune checkpoint blockers (ICBs) have been widely used during the last decade for the treatment of various tumors, including advanced and metastatic melanoma. While these agents have improved melanoma patients' survival rates, they have also been associated with various autoimmune toxicities, with the skin being most commonly affected. The severity of cutaneous toxicity can not only negatively affect patients' quality of life but can also limit the proper treatment of cancer. Thus, the role of the dermatologist is substantial in early detecting and promptly treating these adverse events. Maculopapular rash, psoriasiform, lichenoid dermatoses and bullous pemphigoid are the most frequent cutaneous adverse events that require immediate intervention. Other rare autoimmune toxicities, e.g., sarcoidosis, dermatomyositis or subacute lupus, have also been reported. In this review, we summarize the aspects of ICB-induced cutaneous toxicities in patients with melanoma, emphasizing their management and treatment options in clinical practice.

VEXAS syndrome mimicking lupus-like disease.

Dear Editor, We report on an 81-year-old male Caucasian patient, who initially presented with oral ulcerations and disseminated erythematous papules on the upper back, chest, arms and legs with histopathological evidence of lymphocytic infiltration and small vessel vasculitis matching subacute lupus erythematous (Fig. 1A and B). His skin manifestations improved with topical CS and HCQ at standard dosages. However, a complete remission of the lesions could not be achieved. Therefore, repeated skin biopsies were performed, showing features of subacute eczema or Sweet Syndrome. Fourteen months later, the patient developed recurrent episodes of oral ulcerations, painful auricular and nasal erythematous swelling and arthritis in hands and feet (Fig. 1C and D). A hand MRI showed a moderate to severe wrist joint synovitis. Another 6 months later, he presented with clinical symptoms and laboratory findings of anaemia with increased, corpuscular volume (102.9 fl; normal range 81–98 fl), leukopoenia, lymphopenia, thrombocytopenia, increased levels of CRP, serum amyloid A and IL-6 as well as anti-mutated citrullinated vimentin (MCV) antibodies (Fig. 1G). Treatment with MTX was started at 15 mg/week and was well tolerated, leading to improvement of skin and joint symptoms. The patient experienced one episode of epilepsy with generalized convulsions. Cerebral MRI scan showed unspecific cerebral microangiopathy but no specific lesion. To rule out further foci of inflammation, an 18-fluorodeoxyglucose (18FDG)-PET/CT was performed, showing an enhanced FDG uptake typical for bone marrow activation (Fig. 1E and F), in accordance with a previous description [1]. Over the last 6 months, his skin and joints were well controlled by MTX. However, haematological and inflammatory parameters did not improve. As the patient fulfilled the major diagnostic traits of VEXAS [2], including male sex, increased corpuscular volume >100 fl and low platelets G/l, genetic testing was done. This patient was identified to carry the pathogenic variant c.121A>C, p. (Met41Leu) in exon 2 of UBA1 (NM_003334) (ACMG class 5) in mosaic state (68% of 93 reads), confirming the diagnosis of VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome.

Сlinical cases of newly diagnosed systemic lupus erythematosus at the appointment with a dermatologist

Lupus erythematosus is a multisystemic connective tissue disease that has a wide range of manifestations affecting the skin and mucous membranes, joints, kidneys, heart, lungs, and central nervous system. In dermatology, cutaneous forms of lupus erythematosus can be classified into discoid, disseminated, centrifugal erythema, lupus panniculitis, subacute and chronic lupus, and systemic lupus erythematosus with damage to internal organs. It can develop as an independent disease or can be a result of progression of cutaneous forms.&#x0D; The skin is the second most commonly affected organ in systemic lupus erythematosus folowing the joints, with skin manifestations observed in 80% of patients throughout the course of the disease. And in 2030% of cases, the disease debuts with skin rash. Thus, these patients primarily present to dermatologists. Knowledge of the criteria for making a diagnosis, the clinical characteristics, in combination with performance of advanced laboratory diagnostic tests allow a dermatologist to suspect a systemic process in a timely manner and prescribe appropriate therapy.&#x0D; The article presents two clinical observations of male patients with systemic lupus erythematosus, the diagnosis of which could not have been established for several years. The diagnosis in these cases met the 2019 European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) classification criteria for systemic lupus erythematosus, confirmed by laboratory tests. Patients had a positive trend to response to therapy.

LB932 Dermatomyositis following treatment with cyclin-dependent kinases inhibitors: A single-center retrospective cohort study

The cyclin-dependent kinases 4/6 inhibitors (CDK4/6i) palbociclib, ribociclib, and abemaciclib have been reported to show an association with new-onset subacute lupus erythematosus (SCLE) but CDK4/6i’s role in new-onset dermatomyositis (DM) is unexplored. We evaluated the clinical characteristics of patients presenting with new-onset DM following CDK4/6i. In this retrospective cohort study we queried data registries of Mass General Brigham and Dana-Farber Cancer Institute from October 1, 2014 - November 30, 2021, excluding patients with rash pre-initiation or post-termination of CDK4/6i.

Uncommon Non-Infectious Annular Dermatoses

Several cutaneous diseases can present with annular lesions, making a distinction by physical appearance alone challenging. They can be distinguished into infectious and non-infectious, and common and uncommon annular dermatoses. Common non-infectious diseases include granuloma annulare, urticaria, and subacute lupus erythematosus. In addition, there are rare non-infectious non-neoplastic annular dermatoses whose nosographic attribution is established, including annually recurring erythema annulare centrifugum (EAC) and annular erythema in Sjögren syndrome and others whose nosographic positioning is still debated. They are neutrophilic figurate erythema, palpable migratory arciform erythema, eosinophilic annular erythema, and annular lichenoid dermatitis of youth. Their etiopathogenesis is largely unknown, although immune-mediated mechanisms are likely involved. It is difficult to establish if they are variants of reaction patterns or separate clinic-pathological entities. In fact, EAC and annually recurring EAC may represent different aspects of the same disease. Palpable migratory arciform erythema is hardly distinguishable from EAC deep type, Jessner-Kanof disease, and lupus tumidus. Neutrophilic figurate erythema and eosinophilic figurate erythema are clinically very similar and differing only in the relative proportion of eosinophils and neutrophils.

Clinical profile of cutaneous manifestations of connective tissue diseases in North-East India

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Connective tissue diseases (CTDs) are a heterogeneous group of autoimmune disorders having overlapping clinical features. Skin is often involved and it may be the earliest sign of the disease. This study highlighted the various cutaneous manifestations of common CTDs.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; A hospital-based cross-sectional study was carried out for a period of two years in 83 patients with CTDs in dermatology OPD, RIMS, Imphal. Detailed history taking, examination and relevant serological tests were performed.&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; The mean age was 39.78±17.29 years with female to male ratio of 4.5:1. Majority of the patients had lupus erythematosus (LE) (N=45) followed by systemic sclerosis (SSc) (N=25), rheumatoid arthritis (RA) (N=6), mixed connective tissue disease (MCTD) (N=4) and morphea (N=3). The most common presentation was raised skin lesions (45.8%) followed by Raynaud’s phenomenon (36.1%), photosensitivity (27.7%), skin tightness (26.5%) and joint pain (19.3%). Among LE patients, chronic cutaneous lupus erythematosus (CCLE) was the commonest variant and localised discoid lupus erythematosus (DLE) (22.9%) was the commonest presentation followed by malar rash and annular subacute lupus erythematosus (SCLE). Skin induration, microstomia and sclerodactyly were seen in most patients of SSc. Antinuclear antibodies were positive in 89.1% of patients. Anti-dsDNA and anti-Sm antibodies were positive in 62.2% and 33.3% of LE patients, anti-Scl 70 antibody was positive in 68% of SSc patients.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; CTDs are rare but potentially life-threatening. Proper understanding of the spectrum of cutaneous manifestations of CTDs is therefore necessary for early diagnosis and efficient management.&lt;/p&gt;

AB006. A modular analysis approach to studying gene expression in cutaneous lupus erythematosus

Previous studies on blood transcription profiles of patients with cutaneous lupus (CLE) have primarily used a gene expression approach. The large amount of data involved in these studies can make data analysis and interpretation cumbersome. Consequently, a novel approach in gene expression analyses was devised that groups genes into transcriptional modules (i.e., apoptosis, protein synthesis, inflammation) to identify important themes and relevant genes. This approach was used to study gene expression in systemic lupus patients and subsequently identified a unique interferon signature. Thus, we aim to use the modular analysis approach to identify unique modules and genes of CLE patients in order to better understand their disease pathophysiology. We conducted RNA sequencing of whole transcriptomes of blood from 66 CLE patients and 10 human controls and subsequently performed modular analyses. Based on module scores, we performed an unsupervised cluster analysis to identify subgroups of CLE patients with distinct gene expression patterns. Statistical analyses comparing module scores of these clusters was performed using the Kruskal-Wallis tests with Bonferroni’s correction. Modules were associated with patient subgroups distinguished by demographic, clinical, and laboratory features such as CLE subtypes, autoantibody profiles (BioPlex® 2200 ANA Screen with MDSS), and cytokine profiles (Thermo Scientific ProCarta xMAP cytokine assay kits). The unsupervised cluster analysis identified eight distinct clusters of CLE patients and controls. A neutrophilic predominant signature governed by azurophilic granule genes (i.e., BPI, LTF, MPO) and neutrophil chemotaxis genes (CEACAM6, CEACAM8) was seen in two groups of patients mostly with subacute lupus erythematosus (SCLE) [n=7 (77.8% of total SCLE)] or lupus erythematosus tumidus (LET) [n=6 (60% of total LET)] and European-American descent [n=28 (53.6% of total European-Americans)]. Four groups consisting of a majority of discoid lupus erythematosus (DLE) patients [n=29 (60% of total DLE)] had a predominant T cell signature driven by genes important for antigen presentation and T cell proliferation and differentiation (i.e., SKAP1, ITK). Our data suggests that unique cell populations may help differentiate CLE subgroups, which can potentially affect their disease course and treatment selection. Further studies examining individual gene and protein levels are necessary to confirm our hypotheses.

Exclusive palmoplantar maculopatchy lesions in a patient of secondary syphilis

Syphilis, sexually transmitted disease which is caused by spirochete bacterium, Treponema pallidum. It presents with a spectrum of lesions and the signs and symptoms of syphilis vary depending on which of the four stages it presents. One needs a high level of clinical suspicion and keen history taking are required to identify the underlying syphilis, which is very commonly masked as some other cutaneous condition. Many different skin diseases may have lesions with a annular maculo-papular erythematous morphology and scaling, e.g., pityriasis rosea of Gibert, actinic porokeratosis, erythema annulare centrifugum, granuloma annulare, subacute or discoid lupus erythematosus and certain annular variants of psoriasis. Some of these also have palmo-plantar involvement where these need to be differentiated from from the rash of secondary syphilis. The present article addresses a case of solely palmoplantar hyperpigmented lesion without any clinical evidence of secondary syphilis.

Dermatologic adverse reactions to proton‐pump inhibitors: A synthetized review

Proton-pump inhibitors (PPIs) are one of the most prescribed drugs. Their dermatological adverse reactions are multiple and vary in severity. This review discusses all reported cutaneous side effects of PPIs in order to help physicians understand them and provide appropriate management. A thorough search of PubMed, Embase, and FDAAQ8 drugs websites was conducted. 56 articles including case reports, case series, and review articles of PPI-induced cutaneous adverse reactions were selected. Data were recorded regarding patient age, gender, history, clinical manifestations, diagnostic tests, management, and clinical outcomes. PPI-induced adverse skin reactions are mostly immunological and include both immediate and delayed-type hypersensitivity reactions. These reactions are sometimes life-threatening. All PPIs can induce immediate IgE-mediated reactions. Most of previously published cases of delayed-type hypersensitivity reactions have involved esomeprazole, omeprazole, and lansoprazole. Skin tests are helpful in confirming PPI-induced hypersensitivity reactions and diagnosing potential cross-reactivities. PPIs should also be added to our list of usual suspects when considering possible culprits for a new presentation of drug-induced subacute lupus erythematosus. PPI-related occupational contact dermatitis has also been numerously reported. PPIs should be considered in our list of culprits when considering a patient with a cutaneous drug reaction, taking into account that these drugs can cause severe immunological manifestations.

Innate Immunity in Cutaneous Lupus Erythematosus

BackgroundCutaneous Lupus Erythematosus (CLE) is an autoimmune skin disorder. The adaptive immune system is involved in the etiology of CLE. CLE lesions respond to inhibition of endosomal toll like receptors, hence supporting other disease mechanisms in its pathogenesis.HypothesisGenes associated with defects in the innate immune system would be differentially expressed in CLE skin compared to normal controls.MethodUsing the Gene Expression Omnibus, gene expression data from skin biopsies of patients with cutaneous and normal controls were compared. Data sets selected included: GSE109248, GSE81071, GSE52417, and GSE95474, including acute lupus, subacute lupus, discoid lupus, and normal skin samples. GEO2R was used to analyze the gene expressions between the two sample groups for each data set. STRING (Search Tool for the Retrieval of Interacting Genes/Proteins), GeneCard, and KEGG (Kyoto Encyclopedia of Genes and Genomes Pathway) analysis were used to identify the interaction and function of specific genes.ResultsGenes associated with the NOD like receptor (NLR) signaling pathway were consistently differentially expressed in discoid lupus skin samples in all data sets (p‐value &lt; 8.74e‐05). Five genes associated with the NLR signaling pathway were found to be upregulated in skin samples of discoid lupus patients in all datasets compared to normal controls. These included: STAT1 (signal transducer and activator of transcription 1), Oligoadenylate Synthetases (OAS1, OAS2, OAS3), and AIM2 (absent in melanoma 2). These five genes are known to be involved with transcription activation, regulation of viral infection, and interferon response.ConclusionGenes associated with the NLR signaling pathway are differentially expressed in the skin lesions of lupus patients, specifically discoid lupus, compared to normal controls, supporting the role of the innate immune system in its pathogenesis. Further validation studies using experimental methods such as polymerase chain reaction are needed.

A Case of Systemic Lupus Erythematosus Associated with Drug-Induced Liver and Erythema Multiforme-Like Lesions Which Was Mostly Consistent with the Rowell Syndrome

Rowell syndrome is a disease diagnosed by three major and three minor criteria. The major criteria include the presence of lupus erythematosus (systemic, discoid, or subacute lupus), EM-like lesions (with or without involvement of mucous membranes), and a speckled pattern of antinuclear antibodies. The minor criteria include chilblains, anti-Ro and/or anti-La antibodies, and positive RF. In this article, we present a patient whose manifestation indicated RS, because his diagnosis of Systemic lupus erythematosus was definite, and also onset with EM-like lesions and positive antinuclear antibodies. All these above are consistent with the three major criteria. In addition, his RF was positive, which was the complement for the minor criteria.

Inflammatory chemokine profiles and their correlations with effector CD4 T cell and regulatory cell subpopulations in cutaneous lupus erythematosus

We compared the chemokine/receptor expression in skin biopsies of discoid (SLE/DLE) and subacute lupus (SLE/SCLE) and correlated it with tissue and circulating effector CD4 T cells/regulatory cells. Skin biopsies and peripheral blood from 9 active SLE/DLE patients, 9 SLE/SCLE patients, 5 control SLE patients without cutaneous lesions and 10 control healthy donors were included. Clinical skin activity was measured by Cutaneous Lupus Erythematosus Disease Area and Severity Index scoring, and systemic activity was measured by a modified SLEDAI-2K excluding the cutaneous items. Pain and pruritus were evaluated by a 10-point visual analogue scale. To determine the frequencies of CXCL-10/CXCR3-, CCL2/CCR2-, CCL17/CCR4-, CCL20/CCR6-, CCL27/CCR10-, CXCL8/CXCR1-, CXCL13/CXCR5-, IL-22-, CD4/IL-17A-, CD4/IL-4-, CD4/IFN-γ-, CD123/IDO-, CD25/Foxp3-, and CD20/IL-10-expressing cells, double immunostaining procedures were performed. Circulating CD4+/CD161-/IL-22+, CD4+/CD161+/IL-17+, CD4+/CD25-/IL-4+, CD4+/CD25-/IFN-γ+, CD4+/CD25hi/Foxp3+, CD3+/CD19+/CD38hi/IL-10+, and CD123+/CD196+/IDO + cells were analyzed by flow cytometry. ResultsIn the tissue, CXCL10, CXCR5, and CCL20 expression and IL-22+, CD4+/IL-17+, CD4+/IFN-γ + and CD123+/IDO + cell percentages were increased in SLE/DLE versus SLE/SCLE. Circulating CD4+/CD161-/IL-22+, CD4+/CD161+/IL-17+, CD4+/CD25-/IFN-γ+, CD19+/CD38hi/IL-10 + and CD123+/CD196+/IDO + cell percentages were higher in SLE/DLE versus SLE/SCLE. In the tissue, we found positive correlations between CXCR3 and CD4+/IL-17 + cells; CCR2 and CD4+/IFN-γ + cells; and CCR10 and CD123+/IDO + cells in the SLE/DLE patients and between CXCL13 and CD20+/IL-10 + cells in the SLE/SCLE patients. In the peripheral blood, we determined positive correlations between CXCR5 and CD4+/CD25-/IFN-γ + cells; CCL17 and CD4+/CD161-/IL-22 + cells; and CCL17 and CD4+/CD161+/IL-17 + cells in the SLE/DLE patients and between CXCR5 and CD3+/CD19+/CD38hi/IL-10 + cells; CCR2 and CD4+/CD25hi/Foxp3 + cells; and CXCR1 and CD4+/CD25hi/Foxp3 + cells in the SLE/SCLE patients. Positive correlations between the pain score and the expression of CCL2 and CCR6 expression were found in the SLE/SCLE patients.In conclusion, the correlations between the expression of chemokines/receptors and subpopulations of effector/regulatory cells showed differential responses among the cutaneous pathologies.