Cutaneous Area Research Articles

Subtype and Racial Erythema Variation for Cutaneous Lupus Trials

Regulatory guidance and standardization of disease outcome measures are essential to improve cutaneous lupus erythematosus (CLE) trial design and enhance the diversity of trial participants. To assess variability in erythema presentation across CLE subtypes and among race and ethnicity groups to determine whether these potential differences affect patient eligibility for erythema trials. This cross-sectional study included 377 patients with CLE enrolled in the University of Pennsylvania Cutaneous Lupus Erythematosus Database from January 2007 to December 2023. Data analyses were performed from December 2023 to February 2024. Race and CLE subtype. Mean erythema calculated per the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI)-Activity total score divided by areas affected; then, the result was categorized as pink (1.00-1.49) or red (≥1.50) as surrogate estimates of scores per the Cutaneous Lupus Activity Investigator Global Assessment (CLA-IGA). The total study cohort included 377 adult patients with CLE (mean [SD; range] age, 45.2 [14.8; 18.4-88.8] years; 305 females [80.9%] and 72 males [19.1%]; 115 Black [30.5%], 228 White [60.5%], 34 patients of other races [9.0%; Asian, multiple races, Native American/Pacific Islander, or unknown], and 11 of Hispanic/Latino ethnicity [2.9%]). The most common CLE subtype was chronic CLE (CCLE), affecting 243 patients (64.5%), followed by subacute CLE (SCLE) in 103 patients (27.3%) and acute CLE (ACLE) in 31 patients (8.2%). Significant differences were observed in average erythema across subtypes, with mean (SD) SCLE of 1.62 (0.39) and hypertrophic CCLE of 1.78 (0.25) as the only subtypes routinely classified as red. Significant differences were also observed by race and ethnicity: mean (SD) erythema score in White patients was red (1.58 [0.45]) more frequently than in Black patients (1.36 [0.40]) and patients of other races (1.30 [0.39]), in whom, on average, it was scored as pink. Importantly, among patients who would meet typical CLASI entry criteria (score ≥8) for clinical trials, erythema in more Black patients than in White patients was classified as pink (42% [96 patients] vs 24% [28 patients]), which suggests exclusion from trial participation when a baseline of red lesions is required. The findings of this cross-sectional study suggest that using average erythema scores per the CLA-IGA scale imposes substantial limitations on trial eligibility, specifically by race and subtype. Given the critical need to standardize CLE trial outcome measures and increase diverse representation in clinical trials, our findings support the use of the CLASI as the primary scoring tool to determine erythema trial eligibility.

A double-blind pilot study of oral baricitinib in adult patients with lupus erythematosus panniculitis.

Lupus erythematosus panniculitis (LEP) is a chronic inflammatory skin disease with a significant impact on the overall well-being of patients. The safety and efficacy of oral baricitinib for the treatment of LEP have not been studied. This study aimed to explore the efficacy of oral baricitinib in patients with LEP who are recalcitrant or intolerant to conventional therapies. Patients (aged ≥18 years) with active LEP (with a revised cutaneous lupus erythematosus disease area and severity index [RCLASI]-active score ≥4] were randomly assigned 2:1 to baricitinib (4 mg) or placebo (once daily for 20 weeks). The placebo group was switched to baricitinib (4 mg) at week 13, and the final evaluation was conducted at week 24. The primary endpoint was the proportion of patients with an RCLASI-A score decreased by 20% at week 12. The secondary endpoints included the changes in the Cutaneous Lupus Erythematosus Disease Area and Severity Index active-(CLASI-A) score, the Dermatology Life Quality Index (DLQI), the Physician's Global Assessment (PGA) score, and safety. Five patients were enrolled. Three patients received baricitinib (4 mg), and two patients were treated with placebo. Two patients in the baricitinib treatment group showed a significant RCLASI-A decrease at week 12 and week 24. Two patients in the placebo group had no change in RCLASI-A at week 12 and a significant decrease at week 24. No new safety events were observed. Treatment with baricitinib was effective and well tolerated in patients with LEP.

Extent of Pain Distribution in Entrapment of the Greater Occipital Nerve Identified Through Skin Lesions of Herpes Zoster Involving the Greater Occipital Nerve

Herpes zoster (HZ) involving the upper cervical nerves (C2-C4), which innervate the posterior head, is rare. There have also been few reports of HZ lesions involving the greater occipital nerve (GON), which refers to the medial branch of the dorsal ramus of the second cervical nerve. Pain due to GON entrapment radiates not only to the temporo-occipital region, but also to the ear, retro-auricular and sub-auricular areas of the neck, the angle of the jaw, and the posterolateral neck. Herein, we present the case of a patient who underwent decompression surgery for referred trigeminal pain due to GON entrapment and who developed HZ one week later. Skin lesions of HZ involving the GON occurred not only in the occipital area but also in the ipsilateral ear, auricle, posterior and anterolateral neck. This case of HZ involved all cutaneous areas innervated by the C2, C3, and C4 spinal nerves. The extent of the skin lesions indicated where the pain from the GON radiated.

Sensory assessment of intramuscular quadratus lumborum block at the L2 level in open inguinal hernia repair patients

BackgroundThe effectiveness of the quadratus lumborum block (QLB) for postoperative pain management depends on the injection pathway used. There is limited research on the block area produced by intramuscular injection of local anesthesia in the quadratus lumborum muscle. This study aimed to determine the cutaneous sensory blockade area produced by an intramuscular quadratus lumborum block (QLBi) at the L2 level.MethodsTwenty patients aged 18–60 years with ASA grade I-II and a BMI of 18–30 kg/m2 who were scheduled for open inguinal hernia repair with mesh underwent ultrasound-guided QLBi injection of 20 ml of 0.5% ropivacaine. The cutaneous sensory blockade area was measured by applying a cold stimulus 1 h after the block and then measured every hour after surgery until the sensation returned to normal. The duration of a blockade is defined as the time it takes for all affected areas to fully regain normal sensation following a blockade. Pain scores (numeric rating scale, NRS) were recorded at 2, 4, 8, 12, and 24 h after surgery. Adverse reactions to QLBi were recorded 24 h after surgery.ResultsAll 20 patients had reduced or lost cold sensation areas. The greatest extent of cold sensation reduction occurred at T7 (10%), and the least amount of cold sensation reduction occurred at L3 (10%). The block level covered T8 (20%), T9 (30%), T10 (45%), T11 (90%), T12 (95%), L1 (100%), and L2 (15%). Eighteen patients experienced areas of sensory loss, with the highest range at T11 and the lowest at L2. The duration of the blockade was 8.9 ± 3.8 h, with a maximum of 24 h and a minimum of 5 h. One patient experienced quadriceps weakness after surgery.ConclusionQuadratus lumborum block of intramuscular pathway can produce effective cutaneous sensory blockade, which can be used for postoperative analgesia of indirect inguinal hernia operation, and may also be beneficial to analgesia of other lower abdominal operations. However, the best method needs further confirmation to determine specific anesthesia methods for various operations.Chinese clinical trial registryJune 2, 2018; ChiCTR1800016457.

Preparation of vascularized forearm dermal fat flap and repairing of donor site

Objective:This study prepared vascularized dermal fat flaps and introduced a local split-thickness skin graft from an in situ cutaneous area cutaneous area to manage forearm flap donor sites and evaluated the esthetic and functional outcomes post-operatively. From July 2020 to June 2023, 13 patients with soft tissue defects in Oral and Maxillary Area were repaired with tvascular forearm dermal fat flap. There were 8 males and 5 females, aged from 42-71 years. The flaps ranged from 8 cm×7 cm to 7 cm×5 cm in size. The donor site defects were covered by local split-thickness skin graft from the in situ skins. The color matching degree, surgical scars, ranges of wrist movement and hand sensations in donor forearms were assessed at 6 months after surgery. Results:The tvascular forearm dermal fat flaps for 15 cases all survived. All the local split-thickness skin grafts transplanted with this technique showed primary healing. The follow-up period for 6 months, Donor site exhibited suitabler color matching and there was not severe complications. Conclusion:The vascularized dermal fat flap provides an alternative to conventional forearm flap harvest, which enables primary donor site closure with reduced rates of delayed donor site healing. The vascularized dermal fat flap is a relatively reliable repair method for soft defects in Oral and Maxillary Area.

Characterization and Management of Epithelioid Hemangioma on the Lip Mucosa: A Case Report

Epithelioid Hemangioma (EH) is a rare benign vascular tumor characterized by well-formed blood vessels and epithelioid endothelial cells. Known as Angiolymphoid Hyperplasia with Eosinophilia (ALHE), the World Health Organization does not recommend the use of this term since 2020. Despite its common occurrence in subcutaneous soft tissues of head and neck, cases involving oral mucosa and other extra cutaneous areas are infrequent. This article presents a case of EH in an unusual location and discusses the differential diagnosis with other entities, including Kimura´s Disease (KD). We report the clinical case of a 26 year old man with no significant medical history who presented with a painless increase in volume on the right upper lip, subsequently diagnosed as EH through biopsy and histopathology. The lesion exhibited typical clinical features and a complex histological pattern including epithelioid endothelial cells and an inflammatory infiltrate rich in eosinophils. Diagnosis of EH is challenging due to morphological variability and the need to differentiate it from similar conditions such as KD. Differential diagnosis is crucial, especially in atypical locations. This case shows the importance of histopathology and complementary examinations in diagnosis and the need to consider KD in the differential diagnosis due to its therapeutic and prognostic implications. Therefore, it is concluded that EH, although benign, requires a rigorous diagnostic approach to differentiate it from similar diseases and manage it appropriately. Continuous control is essential due to the potential for recurrence. This case shows the importance of careful differential diagnosis, especially in atypical locations, to optimize management and improve patient outcomes.

Severity Scores for Cutaneous Lupus Erythematosus

Despite the significant disease burden of cutaneous lupus erythematosus (CLE), there have been no United States Food and Drug Administration-approved therapies for 65 years. To facilitate advancement of therapies, severity scores are needed to evaluate QOL, how patients feel, activity of disease, and organ-specific damage to assess response to therapies and disease progression. In this paper, we delineate the development process of provider- and patient-reported severity scores for CLE. Cutaneous Lupus Disease Area and Severity Index (CLASI), a provider-reported measure that distinguishes between activity and damage, has undergone rigorous validation and reliability testing for over 20 years. Its performance has been tested in clinical trials as a primary or secondary endpoint and tool to stratify patients. As an objective disease measure that captures a provider's perspective of disease activity and damage, the CLASI inherently does not assess disease impact on patients' QOL. Cutaneous Lupus Erythematosus Quality of Life (CLEQoL), a patient-reported measure, captures features elucidated through focus groups, including symptoms, emotions, functioning, body image, and photosensitivity. It has undergone psychometric property testing to ensure reliability and validity. Together, CLASI and CLEQoL are simple and reliable CLE-specific severity scores capturing disease activity, damage, and QOL from provider and patient perspectives.

Cutaneous sensory block area of the laparoscopic-assisted transversus abdominis plane block.

Different approaches and techniques are used to apply a transversus abdominis plane block (TAP), but their characteristics are poorly described. Precise injection of local anaesthetic is considered crucial to achieving the desired block effect. Laparoscopic-assisted TAP (L-TAP) is a blind technique and potentially less reliable than ultrasound (US)-guided techniques. This study assessed the cutaneous sensory block area (CSBA) after an L-TAP adopting a subcostal dual block approach. Thirty elective laparoscopic cholecystectomy patients received bilateral L-TAPs. The CSBA was mapped 150 min. after block application using cold sensation and a sterile marker, photo-documented and transferred to a transparency sheet from which the area was calculated. The median CSBA of the subcostal bilateral dual L-TAP was 161 cm2 (interquartile range: 131-217 cm2; range: 67-408 cm2). In all patients, the CSBA mainly covered the skin over the epigastrium, whereas 23% also had an infraumbilical component. In none of the patients did the CSBA cover the abdominal wall laterally to a vertical line through the anterior superior iliac spine. The subcostal bilateral dual L-TAP produces a heterogeneous non-dermatomal CSBA of varying size and distribution across the medial epigastric abdominal wall, similar to the CSBA described in the existing literature on US-guided subcostal TAP. The authors have no sources of funding to declare for this manuscript. Not relevant.

Clinical endpoints in myositis: challenges and ways forward.

This review addresses the challenges and advances in clinical endpoints for myositis, with a particular focus on ensuring comprehensive assessment of both muscle and skin disease activity. The relevance of this review stems from recent developments in outcome measures and their implications for clinical trial design and patient inclusivity. While quality of life (QoL) and lung involvement are also important aspects of myositis, they are beyond the scope of this review and need to be addressed in future studies. Traditional outcome measures like the Total Improvement Score (TIS) have limitations, especially for patients with skin-predominant dermatomyositis (DM). Recent studies highlight the importance of incorporating skin-specific measures such as the Cutaneous Disease Area and Severity Index (CDASI) and the novel composite measure, Dermatomyositis Outcomes for Muscle and Skin (DMOMS). These measures provide a more balanced assessment of disease activity. Clinical trial data analyzed using these measures have demonstrated significant benefits for patients with both classic and amyopathic DM, emphasizing the need for their broader adoption. Advancements in outcome measures are crucial for inclusive and effective myositis clinical trials. Incorporating comprehensive tools like the DMOMS can enhance the assessment of both muscle and skin disease activities, potentially leading to better therapeutic strategies and improved patient outcomes. This shift is essential for addressing the needs of all Idiopathic inflammatory myopathy patients, including those with skin-predominant DM.

Efficient topical treatments of cutaneous lupus erythematosus: A systematic review and network meta-analysis.

Although cutaneous lupus erythematosus (CLE) has been treated with topical agents, there was no high-quality evidence of which agents were more effective, and which clinical scores were more suitable. On December 22nd, 2023, a search was conducted across five databases to identify randomized controlled trials (RCTs). Two authors independently screened the titles and abstracts of articles based on predetermined criteria. Selected articles were then assessed for inclusion in a blinded manner, with any disagreements resolved through consensus. Data were abstracted in duplicate, and a random-effects model was utilized for network meta-analysis. The certainty of the evidence was evaluated according to PRISMA guidelines, using the Grading of Recommendations Assessment, Development and Evaluation approach. The analysis was finalized in January 2024, with the primary outcome focused on the change in cutaneous lupus erythematosus disease area and severity index (CLASI) from baseline. Seven RCTs involving 231 participants were analyzed. The network meta-analysis (NMA) revealed that 4% nicotinamide demonstrated the highest probability of achieving the intended outcomes, with a mean difference (MD) of 3.10 and a 95% confidence interval (CI) of 1.99-4.21. Additionally, 0.05% clobetasol, 2% nicotinamide, and 0.1% tacrolimus also exhibited statistically significant differences, with MDs of 2.30 and 95% CIs of 0.73-3.88; 2.30 and 0.97-3.63; and 1.30 and 0.03-2.57, respectively. As data with a high level of evidence, NMAs demonstrated that 4% nicotinamide, 0.05% clobetasol, 2% nicotinamide, and 0.1% tacrolimus are statistically significant topical agents. CLASI may be an appropriate outcome to evaluate drug efficacy in CLE.

Anifrolumab for systemic lupus erythematosus with multi-refractory skin disease: A case series of 18 patients.

Skin involvement is common in systemic lupus erythematosus (SLE), but may be resistant to conventional treatment. We sought to evaluate the efficacy of anifrolumab (ANI) in refractory cutaneous manifestations of SLE. Case series of patients with refractory cutaneous SLE from three Rheumatology Departments in Greece. Outcome measures were improvement in Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K), physician global assessment (PGA) and Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Clinically relevant improvement in skin was defined as decrease ≥50% (CLASI50) from baseline values. Eighteen patients received ANI; all had active skin involvement at baseline. Mean (SD) SLEDAI and PGA at ANI initiation were 7.4 (2.7) and 1.4 (0.5), respectively, with a mean prednisone dose 4.9 (4.5) mg/day. Mean CLASI (Activity/Damage) at baseline was 13.9 (9.7)/2.9 (4.6). Patients were refractory to a mean 6.3 (1.5) immunomodulatory agents (including hydroxychloroquine and glucocorticoids) before the initiation of ANI. After a mean 8.5 (4.6) months, 89% (n = 16/18) of patients demonstrated significant improvement in general lupus and cutaneous disease activity, and glucocorticoid tapering. Mean SLEDAI and mean CLASI at last visit were 3.4 (1.9) and 2.1 (2.4)/1.4 (2.2), respectively, and mean daily prednisone dose decreased to 2.4 (2.2). Of note, in this group of highly refractory patients CLASI50 was achieved in 16/18 (89%) patients. One patient discontinued ANI after 4 infusions due to a varicella-zoster virus infection and one patient, who initially responded to treatment with ANI, experienced a skin flare due to temporary discontinuation due to Covid 19 infection. DORIS remission and LLDAS were attained in two (11.1%) and eleven (61.1%) patients, respectively. Anifrolumab is highly effective in various skin manifestations of SLE, even after prior failure to multiple treatments.

Comparative Analysis of Mathematical Models and App-Based Measurement for Estimating the Cutaneous Wound Areas of Captive Asian Elephants.

Objective To evaluate the variation in the area estimation under different mathematical calculations against measurement by a smartphone application in estimating the cutaneous wound areas in captive Asian elephants. Methods The study was conducted on captive Asian elephants (Elephas maximus) with cutaneous wounds reported to Veterinary Hospitals of Kerala Veterinary and Animal Sciences University and elephant camps within and outside Kerala state (mostly southern states of India, namely, Kerala and Tamil Nadu) over the period September 2019 to October 2022. Thirty-five clinical cases diagnosed with skin wounds of different aetiologies at various parts of the body were subjected to measurement, and 111 measurements were taken using a smartphone application, Imito Measure (Imito AG, Zurich, Switzerland). Based on the outer wound perimeters hand-marked on the mobile screen over the image taken, Imito Measure calculated the length, width, perimeter, and area. The length and width measurements from this were applied to four mathematical models of wound measurements. Wound surface area calculations were further done by these models and were compared. Results The observed results indicated no significant difference between the five methods of area measurement in all the studied cases since the P > 0.05. Conclusion The findings revealed no significant difference between the five techniques of wound area measurement. From the practical clinical utility point, the smartphone application has an edge over the mathematical methods in animals, especially captive Asian elephants, as it has the major advantage of being non-contact and thus addresses some major welfare concerns.

Anifrolumab in Refractory Systemic Lupus Erythematosus: A Real-World, Multicenter Study.

To report real-world experience on the use of anifrolumab (ANI) in refractory systemic lupus erythematosus (SLE). The present study is a multicenter, retrospective study involving 9 Italian SLE referral centers participating in a compassionate use program for the use of ANI in adult patients with active SLE in whom all the available treatment choices failed, were not tolerated, or were contraindicated. At baseline and 1, 3, 6, 9, and 12 months of treatment, overall and organ-specific disease activity, flares, daily glucocorticoid (GC) dose, and adverse events were recorded. A total of 26 patients were enrolled. At 4 weeks after starting ANI, a significant decrease in the Systemic Lupus Erythematosus Disease Activity Index 2000 (P = 0.01), Systemic Lupus Erythematosus-Disease Activity Score (P = 0.01), and physician global assessment (P = 0.001) was recorded, and the same trend was maintained over time. A significant reduction in Cutaneous Lupus Erythematosus Disease Area and Severity Index-activity (P < 0.001) and in tender (P = 0.03) and swollen (P = 0.02) joint counts was also recorded. At 3 months of follow-up, 33% of patients already achieved a remission state, whereas 46% were in Lupus Low Disease Activity State (LLDAS); at 6 months, 50% were in remission and 80% were in LLDAS. A significant reduction in the mean GC daily dose was observed, starting from week 4 (P = 0.04). A total of 4 disease flares according to the Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus Erythematosus Disease Activity Index were recorded (3 mild-moderate and 1 severe). Overall, 4/20 patients with at least 24 weeks of follow-up (20%) were considered nonresponders. This study provides real-world experience on the use of ANI in patients with refractory SLE, confirming its rapid effectiveness and an overall acceptable safety profile.

Epidemiology and Genetic Characterization of Leishmania RNA Virus in Leishmania (Viannia) spp. Isolates from Cutaneous Leishmaniasis Endemic Areas in Panama.

Leishmania (Viannia) spp. can harbor a double-stranded RNA virus known as Leishmania RNA virus 1 (LRV-1), whose presence has been reported in nine countries across the Americas and seven Leishmania species. Here, we studied 100 Leishmania (Viannia) isolates from patients with cutaneous leishmaniasis collected from different endemic areas in Panama from 2016 to 2022. We identified L. (V.) panamensis, L. (V.) guyanensis, L. (V.) braziliensis/guyanensis hybrid, and L. (V.) panamensis sp.1. (genetic variant). LRV-1 was detected by RT-PCR in 9% of L. (Viannia) isolates (eight cases in L. (V.) panamensis, and one in L. (V.) guyanensis). Phylogenetic analysis based on sequencing data classified all LRV-1 isolates within genotype A, suggesting that LRV phylogenetic proximity is closely aligned with geographical distribution or to the phylogenetic proximity of the Leishmania host in the case of the L. (V.) panamensis and L. (V.) guyanensis in Panama.

JAK inhibitors in refractory dermatomyositis: A case series.

This retrospective cohort study assessed the efficacy and safety of Janus kinase (JAK) inhibitors, tofacitinib and baricitinib, in 14 patients with refractory dermatomyositis (DM), a multisystemic autoimmune disorder with limited therapeutic options. Results demonstrated a significant median decrease of 21 points and a 76% reduction in the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) scores, along with a complete resolution of muscular symptoms in 64% of the patients. JAK inhibitors were effective in managing refractory DM across various subtypes with mild and manageable adverse events.

Comparing the Contributions of Cutaneous and Respiratory Evaporative Water Loss to Cooling in Anna’s Hummingbirds (Calypte anna) Under Fasted and Fed Conditions

During high-temperature periods, endothermic animals evaporate water from the body surface to cool down. The two primary routes of evaporative water loss are: (1) evaporating water from the skin; and (2) evaporation from the respiratory surfaces during breathing and panting. In smaller avian species like Anna's hummingbirds ( Calypte anna), the higher cutaneous surface area to volume ratio presents an opportunity for the skin to become an important avenue for evaporative cooling, possibly becoming a major site. Additionally, hummingbirds feed on water-rich nectar. As a result, they eliminate surplus water through urination, but significant evaporation can also occur through their skin and respiratory system. Thus, total evaporative water loss could rise significantly for these animals when in a hydrated state. Here, this study investigates the relative importance of the two evaporative water loss routes (cutaneous and respiratory) of Anna's hummingbirds in fed and fasted states. We hypothesize that cutaneous evaporation has a high contribution to total evaporation. Moreover, we predict that evaporative water loss rates are higher under the fed (hydrated) condition relative to fasted states. Metabolic and evaporation rates from the head and the body were measured separately in a partitioned chamber at 30°C. Birds were fed immediately prior to entry into respirometry, and the transition from fed to fasted states was recorded over a two-hour period. Total evaporative water loss nearly doubled in the fed versus fasted condition (1.75 ± 0.85 mg H2O/min and 0.97 ± 0.28 mg H2O/min, respectively), with respiratory water loss being significantly higher in the fed compared to the fasted condition (0.87 ± 0.38 mg H2O/min versus 0.42 ± 0.12 mg H2O/min, respectively). On the other hand, the body had a large, but non-significant, difference in evaporative water loss rates between the fed and fasted conditions (0.88 ± 0.55 mg H2O/min and 0.57 ± 0.17 mg H2O/min, respectively). Importantly, cutaneous evaporative water loss made up half of the total evaporation in both the fed and fasted conditions. These results demonstrate that evaporative water loss is modulated by hydration status at both the respiratory and cutaneous surfaces. Furthermore, cutaneous evaporation makes up approximately the same proportion of total evaporation to other birds at thermoneutral temperatures. Future work manipulating ambient temperature in conjunction with hydration status will further our understanding of the relative importance of cutaneous evaporation for these small-bodied birds. Funded by the Genentech Foundations Scholarship, CSUPERB New Investigator Grant, and San Francisco State University. This is the full abstract presented at the American Physiology Summit 2024 meeting and is only available in HTML format. There are no additional versions or additional content available for this abstract. Physiology was not involved in the peer review process.

Indexes of skin activity and damage in patients with systemic lupus erythematosus – CLASI and R-CLASI

Introduction. Cutaneous Lupus Disease Area and Severity Index (CLASI) and its modified version, the Revised Cutaneous Lupus Erythematosus Disease Areas and Severity Index (R-CLASI) are tools for quantifying skin and mucosal lesions in patients with both cutaneous lupus erythematosus and its systemic variant. Evaluation of the scales of activity and skin damage in systemic lupus erythematosus (SLE) is associated with the need to stratify their quantitative characteristics. The Cutaneous Lupus Disease Area and Severity Index (CLASI) and its modified version the Revised Cutaneous Lupus Erythematosus Disease Area and Severity Index (R-CLASI) are a tool for quantifying skin and mucosal lesions in patients with both cutaneous lupus erythematosus (CLE) and its system version.Objective. To validate the indexes of objective assessment of skin activity and damage CLASI and R-CLASI in the Russian cohort of patients with systemic lupus erythematosus and compare it with dermatological assessments of the quality of life.Material and methods. The study included 55 patients with SLE with various types of skin and mucosal lesions, the median age was 30.0 [26.0; 40.0] years, the duration of the disease was 7.0 [3.0; 14.0] years. To assess the active (reversible) lesion and irreversible skin damage, the CLASI and R-CLASI indexes were used, for the general assessment of activity and damage in SLE, the SLEDAI-2K and SLICC/ACR DI were used.Results. The most common variant of skin lesions in patients with SLE is acute cutaneous lupus erythematosus (ACLE) – 45%, as well as alopecia, which occurs in 62% of cases. The median activity index for CLASI was 5.0 [2.0; 11.0], and R-CLASI was 7.0 [3.0; 18.0]; the median damage index for CLASI was 5.0 [2.0; 11.0], and R-CLASI was 2.0 [0.0; 7.0]. A significant relationship was revealed between the medians of CLASI and R-CLASI scores depending on the degree of activity according to SLEDAI-2K (Systemic Lupus Erythematosus Disease Activity Index) and the damage Index (DI) in SLE (SLICC/ACR DI, Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index) when recalculating these indexes only for skin and mucous lesions. According to the ROC analysis, the CLASI and R-CLASI skin activity and damage indices showed high sensitivity (CLASI activity index – 98%, R-CLASI – 93%, CLASI and R-CLASI damage index – 91%) and specificity (CLASI activity index – 64%, R-CLASI – 71%, CLASI and R-CLASI damage index – 86%).Conclusion. To assess the severity of skin and mucosal lesions in patients with SLE in the Russian Federation, it is reasonable to use the CLASI and R-CLASI indices. The CLASI and R-CLASI indices reflect the level of activity and severity of skin lesions, with higher values of these indices indicating more severe skin lesions and a significant impact on the overall well-being of SLE patients. Patients with high values of these indices often experience feelings of embarrassment, discomfort, difficulty in performing daily tasks, and limitations in social life. To assess the severity of skin and mucous lesions in patients with SLE in the Russian Federation, it is advisable to use the CLASI and R-CLASI indexes.

Longitudinal study of patients with anti-SAE antibody positive dermatomyositis: a multicenter cohort study in China.

To describe the longitudinal study and long-term prognosis of multicentre large inception cohort of patients with anti-SAE positive DM. We retrospectively recruited patients with anti-SAE+DM in four tertiary referral centers from China between March 2005 and December 2022. Long-term survival analysis was performed in the enrolled patients. The Myositis Damage Index (MDI) and Cutaneous Disease Area and Severity Index (CDASI) were used to evaluate the degree of different organ damage and the extent of skin rashes. Longitudinal computed tomographic (CT) patterns were analyzed. Phenotypes were characterized using unsupervised cluster analysis. All-cause death occurred in 10.5% (4/38) of all patients, in which three patients succumbed to malignancies at 13, 18, and 36 months. Most patients had favorable long-term outcomes, 35.3% of them were in drug-free remission. Skin rashes showed significant improvement evaluated by CDASI with time. However, damage to different systems was observed in 70.6% of the surviving patients using the MDI, which mainly consisted in skin damage, accounting for 47.1%. Nine patients with anti-SAE+DM associated interstitial lung disease (ILD) underwent repeat CT showed marked radiological improvement at 6 months or being stable after 12 months. In further, different characteristics and outcomes were also showed in three clusters identified by unsupervised analysis. Anti-SAE+DM is characterized with lower mortality rate and the development of malignancies being the primary cause of death. Patients who survived showed notable cutaneous damage, while the ILD tends to stabilize. Clusters identified with unsupervised analysis could assist physicians in identifying higher risk of mortality.

Efficacy and safety of the type I interferon receptor inhibitor anifrolumab in patients with systemic lupus erythematosus (results of a 6-month study)

Objective: to evaluate the efficacy and safety of the type I interferon (IFN) receptor inhibitor anifrolumab (AFM, Safnelo®) in patients with systemic lupus erythematosus (SLE) in real-life clinical practice over an observation period of 6 months.Material and methods. The prospective 6-month study included 21 patients with SLE fulfilling the 2012 SLICC criteria, predominantly women (n=17,81%), median age – 31 [27; 46] years, disease duration – 9 [6.0; 11.0] years. Standard laboratory values and immunological markers of SLE were examined in all patients. The SLEDAI-2K index was used to determine the activity of SLE, and the severity of the mucocutaneous syndrome was assessed using the Cutaneous Lupus Disease Area and Severity Index (CLASI) index. Organ damage was assessed using the SLICC/ACR Damage Index (DI). After 6 months, the achievement of low activity was assessed according to the Lupus Low Disease Activity State (LLDAS) indexResults and discussion. At the time of inclusion in the study, the mean SLEDAI-2K activity index for the group was 8 [6.0; 10.0] points, the median CLASI index – 8.6±8.2 points, 81% of patients had skin and mucosal lesions, 66% had non-erosive polyarthritis, and high immunological activity was observed in all cases. Various irreversible organ damage was observed in 86 of patients. The average DI was 2.2±1.5 points. At the start of AFM therapy, all patients received glucocorticoids (GCs) at a mean dose of 10.7±5.6 mg/day, 52% of patients received a dose above 10 mg/day, 76% of patients continued to take hydroxychloroquine, and 33% of patients took immunosuppressants. Significant positive dynamics were observed with AFM therapy. The average CLASI index for the group after 3 months of treatment was 1.2±4.1 points, after 6 months – 0.3±1.2 points (p&lt;0.0001). The SLEDAI-2K index fell significantly in the group on average from 8 [6.0; 10.0] to 2 [2.0; 4.0] points from the 3rd month of treatment (p&lt;0.0001) and remained at the same level after 6 months. At month 6 of treatment, 13 (62%) of 21 patients met the LLDAS criteria for low disease activity. After the 3rd month of treatment, a significant decrease in antibodies against dsDNA was observed, which persisted for 6 months after the start of treatment. In the group as a whole, there were no significant changes in complement fractions values during the observation period. There was no increase in irreversible organ damage (DI – mean 2.2±1.5 points). The mean daily dose of GCs was significantly reduced from 10.7±5.6 mg/day to 7.5±4.0 mg/day (p&lt;0.01) by the 3rd month and to 5.2±2.1 (p&lt;0.001) by the 6th month of treatment. No infusion reactions were observed in any case. Adverse events occurred in 9 (42%) out of 21 patients, mainly herpes infections of varying severity, mainly after the 1st to 3rd infusions. In one case, severe herpes zoster was observed, so the drug was discontinued.Conclusion. At a dose of 300 mg intravenously monthly AFM is a highly effective drug with a relatively good safety profile in patients with active SLE in whom autoantibodies are present and who do not respond adequately to standard therapy.

Cuban Policosanol (Raydel®) Exerts Higher Antioxidant and Anti-Glycation Activities than Chinese Policosanol (BOC Sciences) in Reconstituted High-Density Lipoproteins: In Vivo Anti-Inflammatory Activities in Zebrafish and Its Embryos.

The present study compares sugarcane-wax purified policosanols sourced from Cuba (Raydel®) and China (BOC Sciences) and utilized following the synthesis of reconstituted high-density lipoproteins (rHDL). The two policosanols exhibited distinctly different ingredient ratios of long-chain aliphatic alcohols, particularly 1-octacosanol (C28) and 1-tetratriacotanol (C34). After synthesizing rHDL with apolipoprotein A-I (apoA-I), the two policosanols bound well with phospholipid and apoA-I to form the discoidal rHDL. Notably, rHDL-1, containing Cuban policosanol, displayed the largest particle diameter at approximately 78 ± 3 nm. In contrast, both control rHDL (rHDL-0) and rHDL containing Chinese policosanol (rHDL-2) exhibited smaller particles, with diameters of approximately 58 ± 3 nm and 61 ± 2 nm, respectively. Furthermore, rHDL-1 demonstrated enhanced anti-glycation activity, safeguarding apoA-I from degradation within HDL, and displayed the antioxidant ability to inhibit LDL oxidation. A microinjection of each rHDL into zebrafish embryos in the presence of carboxymethyllysine (CML) revealed rHDL-1 to have the strongest antioxidant activity with the highest embryo survivability and normal developmental morphology. Dermal application to recover the wound revealed rHDL-1 to have the highest wound-healing activity (75%) and survivability (92%) in the cutaneous wound area in the presence of CML. In adult zebrafish, injecting CML (250 μg) caused acute death and hyperinflammation, marked by heightened neutrophil infiltration and interleukin (IL)-6 production in liver. However, co-administering rHDL-1 notably increased survival (85%) and exhibited strong anti-inflammatory properties, reducing IL-6 production while improving the blood lipid profile. However, a co-injection of rHDL-2 resulted in the lowest survivability (47%) with more hepatic inflammation. In conclusion, Cuban policosanol (Raydel®) has more desirable properties for the in vitro synthesis of rHDL with stronger anti-glycation and antioxidant activities than those of Chinese policosanol (BOC Sciences). Moreover, Raydel-policosanol-integrated rHDL demonstrates a noteworthy effect on accelerated wound healing and robust anti-inflammatory properties, leading to increased survivability in zebrafish embryos and adults by effectively suppressing CML-induced hyperinflammation.