Acute Cutaneous Lupus Research Articles

Tight correlation of 5-hydroxymethylcytosine expression with the scarring damage of discoid lupus erythematosus.

Cutaneous lupus erythematosus (CLE) is a heterogenous skin disease. The two most common subtypes are discoid LE (DLE) characterized by scarring skin damage and acute CLE (ACLE) presenting with transiently reversible skin lesions. It remains unknown what causes the difference of skin lesions. Studies have shown the existence of tissue-specific 5-Hydroxymethylcytosine (5 hmC)-modified regions in human tissues, which may affect the tissue-related diseases. Here, we aim to assess the expression of 5 hmc in DLE and ACLE lesions and explore the relationship of 5 hmc with scarring damage in DLE. 84 CLE samples were included in the study. We evaluated the skin damage score and reviewed the histopathologic sections. Immunohistochemical staining was performed to detect the expression of 5 hmc in the appendage and periappendageal inflammatory cells. The 5 hmc expression in periappendageal lymphocytic cells was investigated by multi-spectrum immunohistochemistry staining. Scarring/atrophy was the most significant damage in differentiating the DLE from ACLE. Perifollicular inflammatory infiltration was present in all patients with DLE scarring alopecia (DLESA). The 5 hmc expression in the appendage and periappendageal inflammatory cells was significantxly increased in DLESA than ACLE. Similar expression pattern was seen in the staining of IFN-alpha/beta Receptor (IFNAR). The expression of 5 hmc in the appendage was positively correlated with that in the periappendageal inflammatory cells. There was an increased 5 hmc expression in lymphocytes cluster around hair follicle consisting of CD4+ cells, CD8+ cells, and CD19+ cells in DLESA lesions. These data demonstrate a close association of the expression pattern of 5 hmc with the histopathological characteristic distribution, and with the type I interferons (IFNs) signals in DLESA, supporting the importance of 5 hmc in the amplification of appendage damage and periappendageal inflammation, thereby offering a novel insight into the scarring damage of DLE and the heterogeneity of CLE skin lesions.

Histology of Skin Alterations in Lupus Erythematosus

Abstract Lupus erythematosus is an autoimmune connective tissue disorder showing a broad spectrum of clinical manifestations. The aim of this study was to assess the correlation of skin histology and different types of lupus erythematosus. Materials and methods: Fifty-one skin specimens were assessed from 39 female and 12 male patients with acute, subcutaneous and chronic lupus erythematosus, diagnosed and treated in the Department of Dermatology and Venereology, Alexandrovska University Hospital for a 4-year period. Results: Follicular hyperkeratosis, epidermal atrophy, vacuolar degeneration and interface dermatitis were the most frequently observed lesions in chronic cutaneous lupus erythematosus while diffuse hyperkeratosis, epidermal atrophy and indistinct interface dermatitis in the dermis were predominant in subacute cutaneous lupus erythematosus. Lupus tumidus, a rare intermittent variant of cutaneous lupus erythematosus, showed almost no epidermal involvement and mucin deposition in the dermis. However, in one of our lupus tumidus patients the disease progressed to a systemic form with histological changes of acute cutaneous lupus erythematosus including atrophy, dermal-epidermal smoothing and lymphocytic infiltration in the dermis. Of note, a few patients showed histological changes of urticarial vasculitis-like and rheumatic-like patterns. Conclusion: The correlation of clinical course, histopathological findings and immunological tests are of vital importance for the correct diagnosis and follow up of patients with lupus erythematodes, thus preventing complications and improving their quality of life.

POS0773 AUTOANTIBODIES IN A MULTI-INSTITUTIONAL INDIAN COHORT (INSPIRE) OF SLE PATIENTS: PREVALENCE, CLUSTER ANALYSIS AND PHENOTYPE ASSOCIATION

BackgroundSystemic Lupus Erythematosus (SLE) is characterized by an array of autoantibodies. Different autoantibodies have been associated with different clinical features like anti-dsDNA antibodies with nephritis and anti-phospholipid antibodies with pregnancy loss. However, the prevalence of autoantibodies has been variable across different ethnic groups. Data on the Indian population is limited.ObjectivesTo assess the prevalence of different autoantibodies in a multi-institutional cohort (INSPIRE) of Indian SLE patients and to test their association with various clinical features using cluster analysisMethodsThe patients (n=1053) enrolled in a multi-institutional cohort of Indian patients (Indian SLE inception cohort for Research [INSPIRE]) were included.1 Antibodies were assayed using Immunoline (Euroimmune, Germany) 17 antigen kit. Anti-phospholipid antibodies (IgG and IgM anti-cardiolipin antibodies (ACLs), IgG anti-Beta 2 GpI antibodies) were measured using ELISA (Euroimmune). Lupus anticoagulant was available in a subset of patients.The prevalence data for autoantibodies were analyzed using an intensity of only ++ and above on Immunoline assay as significant. Univariate analysis by Chi-square test was done to identify associations between individual autoantibodies and their clusters with clinical manifestations.ResultsThe clinical features were fever in 702, alopecia in 813, oral ulcers in 628, acute cutaneous lupus (ACLE) in 520, proteinuria in 400, pleural effusion in 181, thrombocytopenia in 250 and autoimmune hemolytic anemia in 137 patients.The prevalence of various autoantibodies by ELISA was anti-dsDNA antibodies in 70.2% (551/784), IgG Anti- beta-2 GpI in 4.47% (42/938), IgG ACL in 6.14% (61/992) and IgM ACL in 7.1% (54/760). Lupus anticoagulant was present in 13.9% (112/ 805). By Immunoline assay, the prevalence for anti-Ro 52, anti-Ro 60, anti-La and anti-Ribosomal P was 28.49%, 33.14%, 10.07% and 24.03% respectively (Table 1).Table 1.Prevalence of different autoantibodies in the INSPIRE lupus cohortS. No.AutoantibodyPrevalence (%) (n=1053)1.dsDNA28.112.Nucleosomes27.833.Histones24.884.Ro_52_SSA28.495.Ro_60_SSA33.146.SSB-La10.077.Ribosomal P24.038.nRNP36.759.Sm33.1410.Scl-703.2311.PM-Scl0.3812.Jo-10.0913.CENP-B0.3814.PCNA1.3315.AMA-M22.28Cluster analysis (Figure 1) revealed association (Odds ratio with 95% confidence interval) of Cluster 1 (antibodies against dsDNA, histones and nucleosomes) with arthritis (1.51 [1.18-1.94]), proliferative nephritis (3.05[2.08-4.48]) and pleural effusion (1.49[1.08-2.05]), cluster 2 (antibodies against Sm, nRNP, Ro52, Ro60 and Ribosomal P) with ACLE (1.3[1.02-1.65]) and non-proliferative nephritis (1.64[1.09-2.46]) and cluster 3 (antiphospholipid antibodies) with thrombocytopenia (3.34[1.57-7.11]).Figure 1.Cluster analysis of autoantibodies (Cluster 1: dsDNA, histone and nucleosome; cluster 2: Sm, nRNP, Ro52, Ro60 and Ribosomal P; cluster 3: cardiolipin, β2GP1 and La and lupus anticoagulant; cluster 4: Scl-70, Jo-1, PCNA, AMA-M2, PM-SCL and CENP-B)ConclusionThe prevalence of anti-Sm antibody and Anti-Ribosomal P antibody is higher whereas that of anti-La antibody is lesser in the Indian SLE patients as compared to other cohorts of SLE patients with different ethnicities.2 Cluster analysis reveals co-occurrence of different autoantibodies in our patients and their significant association with various clinical manifestations which suggests a possible pathogenic role of autoantibodies or a common genetic basis for it.

Skin CD4+ Trm cells distinguish acute cutaneous lupus erythematosus from localized discoid lupus erythematosus/subacute cutaneous lupus erythematosus and other skin diseases

BackgroundAlthough the contribution of aberrant CD4+ T cell signaling to systemic lupus erythematosus (SLE) is well established, its role in cutaneous lupus erythematosus (CLE) skin is largely unknown. Because the rate of systemic manifestations varies in each subtype, resident memory CD4+ T cells in lesions that are responsible for only skin-associated tissue responses may vary in each subtype. However, the role of CD4+ tissue-resident memory T (CD4+ Trm) cells in each CLE subtype remains unclear. ObjectivesTo analyze and compare CD4+ Trm cells and absent in melanoma 2 (AIM2) identified by smart RNA sequencing (Smart-seq) in CD4+ Trm cells from patients with acute CLE (ACLE), subacute CLE (SCLE), and localized discoid lupus erythematosus (localized DLE) lesions. MethodsWe performed Smart-seq to investigate differences in dermal CD4+ Trm cells between patients with ACLE and normal controls (NCs). Multicolor immunohistochemistry was utilized to measure the levels of AIM2 in CD4+ Trm cells present in the skin of 134 clinical patients, which included patients with localized DLE (n = 19), ACLE (n = 19), SCLE (n = 16), psoriasis (n = 12), rosacea (n = 17), lichen planus (n = 18), and annular granuloma (n = 15), as well as NCs (n = 18). ResultsThe Smart-seq data showed higher AIM2 expression in skin CD4+ Trm cells from ACLE lesions than NCs (fold change >10, adjusted P < 0.05). AIM2 expression in CD4+ Trm cells did not vary according to age or sex. AIM2 expression in CD4+ Trm cells was significantly lower in patients with ACLE (6.38 ± 5.22) than localized DLE (179.41 ± 160.98, P < 0.0001) and SCLE (63.43 ± 62.27, P < 0.05). In an overall comparison of ACLE with localized DLE and SCLE, the receiver operating characteristic curve for AIM2 expression in CD4+ Trm cells had a sensitivity of 100.00% and a specificity of 82.86% at a cutoff value of 18.26. In a comparison of ACLE with localized DLE, the sensitivity was 89.47%, and the specificity was 100.00% at a cutoff value of 12.26. In a comparison of ACLE with SCLE, the sensitivity was 100.00%, and the specificity was 75.00% at a cutoff value of 18.26. ConclusionsThe number of CD4+ Trm cells is increased in lesions of SCLE and localized DLE compared to ACLE, suggesting that CD4+ Trm cells may have a more crucial role in persistent lesions of SCLE and localized DLE. In addition, AIM2 expression in CD4+ Trm cells discriminates patients with ACLE from those with localized DLE and SCLE.

Toxic epidermal necrolysis as a manifestation of acute cutaneous lupus erythematosus.

Toxic epidermal necrolysis as a manifestation of acute cutaneous lupus erythematosus.

Correlation of whole blood hydroxychloroquine concentration with cutaneous lupus erythematosus and factors associated with it: First multicenter, cross-sectional analysis in Malaysia.

Hydroxychloroquine (HCQ) is the first-line systemic treatment for cutaneous lupus erythematosus (CLE). Whole blood HCQ concentration (WBHCQ) was found to correlate with CLE severity among Caucasians. However, studies on Asians are scarce. We aim to explore the relationship of WBHCQ with CLE disease activity among multi-racial Malaysians and the factors associated with WBHCQ. A cross-sectional study targeting patients with CLE was conducted from 1 June till 30 November 2019. Disease activity was assessed using Cutaneous Lupus Erythematosus Disease Area and Severity Index - Activity Score (CLASI-AS). Blood was analyzed for WBHCQ concentration using a high-performance liquid chromatography technique. Statistical analysis was done using R studio version 1.2.1335. A total of 88 subjects (male:female, 4.5:1) with a median age of 41years old were recruited. The median duration CLE was 5years. The majority had acute cutaneous lupus (n=45, 51.1%). The median WBHCQ was 946.8ng/mL. Indians were found to have the highest WBHCQ (median±interquartile range [IQR], 1515.4±1494.8ng/mL). Males had a lower WBHCQ (median±IQR, 733.5±573.8ng/mL) than females (995.5±925.1ng/mL). However, no statistically significant association between race and sex with WBHCQ was demonstrable (p=0.247, p=0.066). No correlation was demonstrated between WBHCQ and CLASI-AS (r=-0.02, p=0.851). A positive correlation was found between HCQ dosage (ideal bodyweight) and WBHCQ (r=0.24, p=0.027). No other factors were found associated with WBHCQ. Indians and females were observed to have higher WBCHQ; however, no significant correlation was identified. Further study is required to confirm the finding.

B Cell Signatures Distinguish Cutaneous Lupus Erythematosus Subtypes and the Presence of Systemic Disease Activity.

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.

Clinico-Pathological Profile of Cutaneous Lupus Erythematosus Patients: A Report from A Tertiary Care Center of Chattogram

Background : Nearly all epidemiologic studies have involved patients with Systemic Lupus Erythematosus (SLE). Few authors have investigated the characteristics of patients with Cutaneous Lupus Erythematosus (CLE). We aim to describe the epidemiologic, clinical, and immunologic characteristics of a series of patients diagnosed with CLE.&#x0D; Materials and methods : This is a descriptive retrospective cross-sectional study carried out using the register records of total 218 patients attending the ‘Lupus Clinic’ in Chittagong Medical College Hospital during the period 2010 and 2020. The disease activity and damage of CLE were assessed according to the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI).&#x0D; Results : There were 187 females (86%) and 31 males (14%) with the female: male ratio being 6.1:1. The mean age was 28 ± 10.06 (mean ± SD) ranging between 11 and 65 years. The Chronic Cutaneous Lupus Erythematosus (CCLE) patients accounted 154 (71%) followed by Acute Cutaneous Lupus Erythematosus (ACLE) 46 (21%) and Subacute Cutaneous Lupus Erythematosus (SCLE) 18 (8%). On the basis of CLASI score, 91 (42%) patients were in mild form, 85 (39%) in moderate form and 42 (19%) in severe state. In LE specific skin lesions, common manifestation was photosensitivity 198 (91%) followed by discoid rash 154 (71%) and maculo-papular lupus rash 55 (25%). Oral ulcer was seen in 49 (22%) patients and malar rash in 46 (21%) patients. Other observed LE specific skin manifestations were papulo squamous rash 11 (5%), Toxic epidermal necrolysis like lesions 7 (3%) and lichenoid lesions 6 (3%). Among LE nonspecific skin lesions, common manifestation was nonscarring alopecia 123 (56%) followed by Raynaud’s phenomenon 17 (8%) livedo reticularis 17 (8%) Vasculitis 15 (7%) Periungual telangiectasia 7 (3%) erythema multiforme 6 (3%) and leg Ulcer 5 (2%). Antinuclear Antibody (ANA) 132 (61%) was the most common autoantibody followed by anti-ds DNA 91 (42%) anti-Sm antibody 2 (1%) anti-phospholipid antibodies 9 (4%) and anti-RNP 3 (1%). Hematological manifestations were seen in 161 (73.85%) where erythrocyte sedimentation rate was the most common hematological abnormality 161(73.85%) followed by lymphopenia 126 (57.80%) leucopenia 113 (51.80%) thrombocytopenia 107 (49.10%) anemia 92 (42%) monocytopenia 37 (16.97%). Conclusions: CCLE was the most common subtypes of CLE. Patients with different subtypes of CLE have distinct clinical and pathological characteristics. The onset or concurrence of SLE mandates the involvement of other disciplines depending on organ involvement. In the absence of consensus on a definition that makes it possible to differentiate cutaneous forms of LE from SLE, the dermatologist’s role in the correct diagnosis and classification of such patients is fundamental.&#x0D; Chatt Maa Shi Hosp Med Coll J; Vol.20 (2); July 2021; Page 79-84

Generalized acute cutaneous lupus erythematosus in a young female.

A history of systemic lupus and discontinuation of medications for 3 months led to increased disease activity.

Catatonia in systemic lupus erythematosus: case based review.

Catatonia is a rare psychomotor syndrome characterized by stupor, posturing and echophenomena. It can be associated with schizophrenia, infections, drugs and autoimmune causes like anti N-methyl D-aspartate (NMDA) receptor encephalitis and systemic lupus erythematosus (SLE). Here we report two cases of systemic lupus erythematosus with catatonia, who improved with immunosuppressive treatment and review the cases described in the literature. The first case presented with fever, pancytopenia, toxic epidermal necrolysis (TEN)-like rash and later developed catatonia and macrophage activation syndrome (MAS). The second case presented with acute cutaneous lupus erythematosus (ACLE), fever, alopecia, polyarthralgias, nephritis, cytopenias along with catatonia. Successful management of this syndrome requires prompt recognition and treatment with immunosuppression as well as benzodiazepines with or without electroconvulsive therapy (ECT).

A Cross-Sectional Study of Clinical and Immunological Profile of SLE Patients from a Tertiary Care Center in the Sub Himalayan Region of Northern India

Introduction: Systemic Lupus Erythematosus (SLE) is a disease of thousand faces whose manifestations vary with the interplay of genetic, ethnic, and host factors along with environmental exposures and geographical conditions. Since the first case of SLE reported in India in 1955, many case series have been published from various centers depicting Clinical and Immunological features in the Indian population. This study was undertaken to study the clinical and immunological profile in a tertiary care center in the sub-Himalayan region of northern India. Materials and Methods: This study is a cross-sectional retrospective study conducted at a tertiary care center in the hilly northern state of Himachal Pradesh over a period of 1 year; from 1st June 2017 to 31st May 2018. The records of all patients admitted in the study period with the department of medicine and diagnosed with SLICC 2012 classification criteria were studied to abstract the Clinical and Immunological profile of patients. Results: A total of sixty-nine patients were included in the study. 96% of the subjects were female patients, and 84 % of the patients were in the reproductive age group of 20 – 40 years. Mucocutaneous manifestations were most commonly seen in nearly 89 % of patients followed by musculoskeletal involvement in about 71 %. Hematological involvement was more frequent than reported in the literature. Lupus Nephritis was diagnosed in about 30 % of the patients. Fatigue and synovitis were the most common features, followed by oral ulcers and Acute cutaneous lupus. ANA and ds DNA positivity was 98.5 and 72.4 % respectively. Six cases of MCTD were d=found in the study group. Conclusion: In hospitalized SLE patients admitted in a tertiary care center of the northern hilly state, females of reproductive age group constituted the majority of patients. Mucocutaneous, Musculoskeletal, hematological, and renal involvement was most prevalent. Keywords: SLE, cross-sectional study, Mucocutaneous involvement, Renal Biopsy, Hilly area.

Guideline for the diagnosis, treatment and long-term management of cutaneous lupus erythematosus

Cutaneous lupus erythematosus (CLE) is an inflammatory, autoimmune disease encompassing a broad spectrum of subtypes including acute, subacute, chronic and intermittent CLE. Among these, chronic CLE can be further classified into several subclasses of lupus erythematosus (LE) such as discoid LE, verrucous LE, LE profundus, chilblain LE and Blaschko linear LE. To provide all dermatologists and rheumatologists with a practical guideline for the diagnosis, treatment and long-term management of CLE, this evidence- and consensus-based guideline was developed following the checklist established by the international Reporting Items for Practice Guidelines in Healthcare (RIGHT) Working Group and was registered at the International Practice Guideline Registry Platform. With the joint efforts of the Asian Dermatological Association (ADA), the Asian Academy of Dermatology and Venereology (AADV) and the Lupus Erythematosus Research Center of Chinese Society of Dermatology (CSD), a total of 25 dermatologists, 7 rheumatologists, one research scientist on lupus and 2 methodologists, from 16 countries/regions in Asia, America and Europe, participated in the development of this guideline. All recommendations were agreed on by at least 80% of the 32 voting physicians. As a consensus, diagnosis of CLE is mainly based on the evaluation of clinical and histopathological manifestations, with an exclusion of SLE by assessment of systemic involvement. For localized CLE lesions, topical corticosteroids and topical calcineurin inhibitors are first-line treatment. For widespread or severe CLE lesions and (or) cases resistant to topical treatment, systemic treatment including antimalarials and (or) short-term corticosteroids can be added. Notably, antimalarials are the first-line systemic treatment for all types of CLE, and can also be used in pregnant patients and pediatric patients. Second-line choices include thalidomide, retinoids, dapsone and MTX, whereas MMF is third-line treatment. Finally, pulsed-dye laser or surgery can be added as fourth-line treatment for localized, refractory lesions of CCLE in cosmetically unacceptable areas, whereas belimumab may be used as fourth-line treatment for widespread CLE lesions in patients with active SLE, or recurrence of ACLE during tapering of corticosteroids. As for management of the disease, patient education and a long-term follow-up are necessary. Disease activity, damage of skin and other organs, quality of life, comorbidities and possible adverse events are suggested to be assessed in every follow-up visit, when appropriate.

S2k guideline: Diagnosis and management of cutaneous lupus erythematosus - Part1: Classification, diagnosis, prevention, activity scores.

S2k guideline: Diagnosis and management of cutaneous lupus erythematosus - Part1: Classification, diagnosis, prevention, activity scores.

Dermoscopic features of acute cutaneous lupus erythematosus: A retrospective analysis from a tertiary care centre of East India.

The cutaneous features of acute cutaneous lupus erythematosus (ACLE) can be confused with other dermatoses. The dermoscopic features of ACLE have rarely been reported. We aimed to study the dermoscopic features of ACLE, malar rash, and generalized ACLE. The dermoscopic features of all biopsy and direct immunofluorescence confirmed ACLE cases were analysed retrospectively between May 2019 and May 2020. A total of 21 patients with skin phototypes III, IV, or V were included in the analysis. A pinkish-white to reddish-white homogenous area (94.1%), brown dots, globules and peppering (76.4%), and keratotic follicular plugging (64.7%) were the common dermoscopic features observed in the case of generalized ACLE. Similarly, in the case of malar rash, a pinkish-white to reddish-white homogenous area (77.8%) was the most common dermoscopic feature, followed by keratotic follicular plugging (66.7%), scales (44.5%), and brown dots, globules, and peppering (44.5%). In both variants, vascular structures were dominated by linear vessels with or without branching. A multicomponent pattern comprising white scales, homogenous pinkish-white to the reddish-white area, brown dots/globules/peppering, keratotic follicular plugging, and linear vessels with or without branching were the common dermoscopic features observed in our study and may indicate the diagnosis of both localized and generalized ACLE. In addition, a paucity of dotted vessels may serve as an additional clue.

Cutaneous Manifestations of "Lupus": Systemic Lupus Erythematosus and Beyond.

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. Cutaneous lupus can be subdivided into three main categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. Physical exam, laboratory studies, and histopathology enable differentiation of cutaneous lupus subtypes. This differentiation is paramount as the subtype of cutaneous lupus informs upon treatment, disease monitoring, and prognostication. This review outlines the different cutaneous manifestations of lupus erythematosus and provides an update on both topical and systemic treatment options for these patients. Other conditions that utilize the term “lupus” but are not cutaneous lupus erythematosus are also discussed.

POS-221 Clinico-Pathological Profile of Children with Lupus Nephritis in A Tertiary Care Hospital

This study was done to observe the clinicopathological presentation of children presented with lupus nephritis (LN). This cross-sectional study was done in children aged 1-18 years with LN, admitted to the Pediatric Nephrology Department of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from January 2017 to April 2019. LN was diagnosed according to Systemic Lupus International Collaborating Clinics (SLICC) criteria. All patients underwent renal biopsy and tissues were examined under light microscopy and immunofluorescence by a pathologist in the Department of Pathology. Thirty-five children with LN with a mean age of 11.6 ± 3.9 years were evaluated and 26 (74.28%) were female. Twenty-seven (77.14%) patients presented with acute cutaneous lupus followed by oral ulcer (51.4%), arthritis (48.6%), chronic cutaneous lupus (28.57%), and psychosis (11.4%). Significant proteinuria was present in 20(57.1%) and 9 (25.7%) patients had hemolytic anemia. Class IV nephritis was the most common histopathology (51.43%) followed by class II (25.7%). Acute cutaneous lupus was the most common clinical presentation in children with lupus nephritis though psychosis was not uncommon as the initial presentation. Class IV was the most common stage on histology.

AB004. Evaluating differences in meaningful change in disease activity between different races and clinical subtypes affected by cutaneous lupus erythematosus

Cutaneous lupus erythematosus (CLE) has a profound impact on a patient’s quality of life (QoL), despite epidemiologic differences disease presentation. Disease activity, rather than damage, has a greater impact on QoL, and improvement in disease activity, defined by the Cutaneous Lupus Disease Area and Severity Index score for activity (CLASI-A), is correlated to improvement in QoL. We aimed to evaluate the differences between meaningful improvement in disease activity, experienced by Caucasian and African-American patients and disease subtypes. This study included 144 patients seen at the Hospital of the University of Pennsylvania who participated in a longitudinal research database of lupus patients. Patients with an initial CLASI-A ≥4 were included, and classified by their self-identified racial background and clinical disease subtype. Linear regression models were used to calculate change in disease activity needed to predict meaningful improvement in QoL, defined as a respective 9.38-point in the Emotions subscale of Skindex-29. Due to the limited number of patients with acute CLE, we compared meaningful improvement between patients with discoid lupus erythematosus (DLE) and patients with subacute CLE (SCLE). An improvement of disease activity by 58.6% and 52.5% is associated with a meaningful impact on the Emotions subscale of Caucasian and African-American patients, respectively. Decreasing disease activity by 52.1% and 55.2% is meaningful for patients with DLE and SCLE, respectively. Regardless of individual differences in disease activity and presentation of CLE, patients experience improvement in QoL, particularly in the emotional component, with similar meaningful changes in their disease activity.

AB023. An atypical clinical presentation of alopecia in two patients with systemic lupus erythematosus

Alopecia in varying patterns is a common feature of lupus erythematosus (LE). Several forms of alopecia that are not specific to LE can occur in the setting of lupus, such as alopecia areata, telogen effluvium, and anagen effluvium. LE-specific alopecias are defined as those with histology consistent with LE, and include forms of acute, subacute, and chronic cutaneous lupus erythematosus (CCLE). Common patterns of LE-specific alopecia include the non-scarring diffuse hair thinning and fragility of acute LE and the scarring, erythematous scaly plaques with follicular keratotic plugs, peripheral hyperpigmentation, and central hypopigmentation of discoid lupus erythematosus (DLE). Lupus erythematosus tumidus (LET) may also present on the scalp as well-defined, non-scarring alopecia without overlying scale, atrophy, and dyspigmentation. We report two male patients with large circular non-scarring alopecic plaques on the scalp without overlying scale or erythema, but with central hyperpigmentation and scarring – findings reminiscent of but also distinct from both DLE and LET. Punch biopsy from the hyperpigmented area of alopecia revealed peri-follicular lymphocytic infiltrate, follicular dropout, and increased dermal mucin consistent with LE; further workup and serologic testing revealed systemic lupus in both patients. These two cases demonstrate a unique clinical presentation of central scarring alopecia within a larger non-scarring alopecic plaque in the setting of SLE that deviates from typical lupus-related alopecia. Teaching point: these two cases underscore the morphologic heterogeneity of CCLE and need for thorough evaluation of systemic disease in patients presenting with both scarring and non-scarring alopecia.

A deep learning-based smartphone platform for cutaneous lupus erythematosus classification assistance: Simplifying the diagnosis of complicated diseases

To the Editor: Cutaneous lupus erythematosus (CLE) can be divided into different subtypes including acute cutaneous LE, subcutaneous LE, and discoid LE. These subtypes of CLE are easily confused with other skin diseases,1 which leads to inappropriate or delayed treatment. Convolutional neural networks (CNNs) have shown great power for automatic analysis of clinical images.2 However, data concerning CLE are limited.3 Here, we report our development of a smartphone-based platform to assist CLE diagnosis and classification.

Demyelination with autoimmune features: a distinct clinical entity? Results from a longitudinal cohort.

CNS demyelinating syndromes occurring in the context of SLE may represent a manifestation of neuropsychiatric lupus, or an overlap of SLE and multiple sclerosis (MS). We evaluated prospectively patients presenting with demyelinating syndrome for clinical and serological evidence of SLE and characterized the evolution of their clinical syndrome to a defined disease. Patients with CNS demyelinating syndromes not fulfilling the criteria for MS were evaluated in a rheumatology unit for features of SLE and followed longitudinally (enrolment period 2016-20). Clinical, laboratory and neuroimaging data were recorded at every visit, following multidisciplinary evaluation. At end of follow-up, patients were assessed for their final neurological and rheumatological diagnosis, and classified accordingly. A total of 79 patients were included in the study [91.1% female, mean (s.d.) age at first demyelinating episode 38.4 (10.3) years, median (interquartile range) observation period 39 (57) months]. At last follow-up, 38 patients (48.1%) had evolved into MS. Of the remaining patients, 7 (17.1%) had SLE, while 34 (82.9%) had features of systemic autoimmunity without fulfilling classification criteria for SLE. The most common rheumatological features of these patients were inflammatory arthritis (73.5%), acute cutaneous lupus (47.1%) and positive ANA (72.1%). Importantly, these patients were less likely to have elevated IgG index (odds ratio 0.11, 95% CI 0.04, 0.32) and positive oligoclonal bands (odds ratio 0.21, 95% CI 0.08, 0.55). A significant number of patients with demyelination do not fulfill criteria for either MS or SLE at follow-up. These patients exhibit lupus-like autoimmune features and may represent a distinct entity, 'demyelination with autoimmune features'.