PV067 / #449 Poster Topic:AS07 - Cutaneous Lupus Cutaneous lupus erythematosus (CLE) significantly impacts health-related quality of life (HRQoL). Although the EuroQol 5 Dimensions 5 Levels (EQ-5D-5L) is a widely used HRQoL measure, its psychometric properties in CLE have not been previously investigated. Given the important role of the EQ-5D-5L in health technology assessments (HTA), establishing the validity of this instrument is crucial for accurately reflecting health status and treatment effects in CLE, thereby guiding clinical and HTA decision making and improving patient care. Therefore, this study aimed to investigate the construct validity of the EQ-5D-5L in people with CLE. This was a secondary analysis of data from a prospective, cross-sectional, multicenter, multinational study assessing pruritis in participants with CLE.[1] Outcome measures included the EQ-5D-5L, Dermatology Life Quality Index (DLQI), Cutaneous Lupus Erythematosus Disease Area and Severity Index-Activity Scale (CLASI-A), Worst Itch Numeric Rating Scale (NRS), Worst Pain NRS, and the 12-item Pruritus Severity Scale (12-PSS). Descriptive analyses were performed on all demographic and disease characteristic variables. Convergent validity was assessed by examining correlations between the EQ-5D-5L domains and derived EQ-5D-5L index value with other disease-specific measures. Known-groups validity was evaluated by analyzing whether the EQ-5D-5L captured expected differences among participant groups with different CLASI-A severity levels: mild (score 0-9), moderate (10-20), and severe (21-70). A total of 149 people with CLE were included in the study; 75% were female and 79% were Caucasian/White, with a mean (SD) age of 49.6 (15.5) years. Mean (SD) CLASI-A score was 7.35 (7.77; n = 134) and EQ-5D-5L index value was 0.81 (0.21; n = 137). The EQ-5D-5L index value showed statistically significant low moderate (with CLASI-A and Worst Itch NRS) to high moderate (with DLQI, 12-PSS, and Worst Pain NRS) correlations with select reference variables. The known-groups hypothesis was confirmed with a medium effect size of η² = 0.11. Only the Pain/Discomfort and Anxiety/Depression domains demonstrated statistically significant low moderate to high moderate correlations with at least 2 reference variables. The known-groups hypothesis was confirmed for the Self-Care, Pain/Discomfort, and Anxiety/Depression domains with small to medium η² effect sizes (0.05 ≤ η² ≤ 0.08). Sensitivity analyses, excluding 5 outliers, resulted in the disappearance of all high moderate correlations of the EQ-5D-5L domains and derived EQ-5D-5L index value. Only the known-groups hypothesis of the EQ-5D-5L index value and the Self-Care domain remained confirmed, but with a diminished small (η² = 0.06) and medium (η² = 0.10) effect size. This study is the first to investigate the construct validity of the EQ-5D-5L in CLE, indicating moderate convergent and known-groups validity. However, sensitivity analysis results were notably weaker, compromising the robustness of the findings and suggesting potential limitations in the ability of the EQ-5D-5L measure to fully capture all health status dimensions relevant to CLE. Further psychometric studies are warranted to determine the overall appropriateness of the EQ-5D-5L in CLE.References:[1.] Samotij D. Lupus 2021;30:1385-93.Funding:The healthcare business of Merck KGaA, Darmstadt, Germany (CrossRef Funder ID: 10.13039/100009945) funded the study and editorial support by Bioscript Group.

Allergic diseases are thought to be caused by a combination of acquired immune activation, excessive activation of innate immunity, and disruption of epithelial barrier function due to scratching, proteases, and more. The removal and inactivation of allergy-related substances in indoor environments are considered effective for reducing allergic disease symptoms. The typical allergens in Japan are Japanese cedar pollen and house dust mites (HDM), and together with bacteria and fungi, HDM are the main sources of proteases in indoor environments. We investigated the inactivating effects of ozonated water on these substances in vitro in terms of allergenicity, inflammation induction in epithelial and immune cell lines (i.e., HaCaT, A549, and RAW 264 cells), and protease activity. We observed that ozonated water inactivated the Japanese cedar pollen allergen Cry j1 and the HDM allergen Der f1, the innate immune activator lipoteichoic acid from the bacterium Staphylococcus aureus, and proteases from S. aureus, HDM, and the fungus Alternaria in an ozone concentration-dependent manner. In all experiments, ozonated water at 7.5 mg/L significantly inactivated allergy-related substances compared to the untreated group (p < 0.01). The comparison of the effects of ozonated water treatment and thermal treatment at 80 °C revealed that ozonated water treatment is superior to thermal treatment in terms of both effectiveness and reaction time. Together, our findings demonstrate that ozonated water can inactivate allergy-related substances in the indoor environment. The management of indoor environments using ozonated water can thus be expected to contribute to the alleviation of symptoms and suppression of allergic diseases.

In recent years of research in immunology, the understanding of innate immunity has deepened, and the concept of innate immunity has been proposed even in the field of acquired immunity. The conventional immunosuppressive treatments mainly act via regulation of acquired immunity. However, the involvement of innate immunity was confirmed for hydroxychloroquine (HCQ), which has been confirmed to be effective in the treatment of cutaneous lupus erythematosus (CLE). Approximately 1.5 years prior to this trial, HCQ was officially approved for use in Japan by the Japanese Ministry of Health, Labor and Welfare for the treatment of CLE and SLE in September 2015. This review introduces the mechanism underlying the development of CLE from the viewpoint of autoantibodies, cytokines, and innate immunity. Furthermore, the mechanism of action of HCQ is introduced and discussed.

Heat shock proteins (HSPs) are molecular chaperones whose primary function is cytoprotection, supporting cell survival under (sub) lethal conditions. They have been implicated in various diseases such as inflammatory diseases and cancer due to their cytoprotective and immunomodulatory effects, and their biological mechanisms have been studied. Central family members include, HSP27, which is induced by various stimuli such as heat shock, hypoxia, hyperoxia, ultraviolet exposure, and nutritional deficiency, and HSP70, which is homeostatically expressed in many organs such as the gastrointestinal tract and has anti-cell death and anti-inflammatory effects. In this study, HSP27 and HSP70 were investigated during thrombus formation and dissolution in a deep vein thrombosis model by immunohistochemistry to determine their involvement in this process and whether their expression could be used as a forensic marker. In the process of thrombus formation and lysis, HSP27 and HSP70 were found to be expressed by immunohistochemical analysis. The role of inhibitors of HSP27 and HSP70 in the pathogenesis of thrombosis in mice was also investigated. When HSP27 or HSP70 inhibitors were administered, thrombi were significantly smaller than in the control group on day 5 after inferior vena cava ligation, indicating pro-thrombotic effects HSP27 and HSP70. If HSP27- or HSP70-positive cells were clearly visible and easily identifiable in the thrombus sections, the thrombus was presumed to be more than 10 days old. Thus, the detection of intrathrombotic HSP27 and HSP70 could forensically provide useful information for the estimation of thrombus ages. Collectively, our study implied that both HSP27 and HSP70 might be molecular targets for thrombus therapy and that the detection of HSP-related molecules such as HSP27 and HSP70 could be useful for the determination of thrombus ages.

Indoor air pollutants include biogenic pollutants such as house dust mites (HDM), fungi, and bacteria, and transboundary air pollutants such as Asian yellow dust (Kosa) and PM2.5. These pollutants act directly on the skin, inducing inflammation and causing or aggravating allergies. With the improved airtightness and insulation of dwellings, the economic development, and the expansion of deserts and arid regions, the potential for exposure to indoor pollutants seems to be greater than in the past. Given these circumstances, we focused on the inflammation of epidermal keratinocytes caused by these indoor pollutants, and examined the inflammatory effects of various indoor pollutants and tried to suppress them with the flavonoid naringenin. In this study, HDM extracts of body (Db) and fecal (Df), Alternaria extract (Alt), lipoteichoic acid (LTA) from Staphylococcus aureus, Kosa, and Urban aerosols including PM2.5 (UA) were used as pollutants. The human keratinocyte cell line HaCaT was stimulated with these pollutants, and the productions of cytokines and chemokines were evaluated by enzyme-linked immunosorbent assay (ELISA), as well as the pretreatment effect of naringenin on the cells. All of these pollutants showed inflammatory effects, with Df showing the strongest inflammatory effects and Alt showing strong cytotoxicity. In contrast, pretreatment with naringenin significantly suppressed the inflammatory effects of these pollutants in a dose-dependent manner. The inflammatory effects of these indoor air pollutants on epidermal keratinocytes were stronger than expected, suggesting that indoor air quality control is also necessary to maintain skin health. In addition, topical application or oral intake of naringenin, or active intake of foods containing naringenin or its glycosides (citrus fruits, tomatoes, etc.), may prevent skin inflammation caused by indoor air pollutants.

This chapter aims to discuss about Effective Treatments for Japanese Patients' Skin Lesions Associated with Discoid lupus erythematosus (DLE). DLE is an inflammatory skin disease that can occur in patients with or without systemic lupus erythematosus (SLE). Localized DLE, generalized/widespread DLE, hypertrophic/ verrucous DLE, and mucosal DLE are all clinical characteristics. There are topical and systemic therapies available. Corticosteroids and calcineurin inhibitors are examples of topical treatments. Antimalarials are widely accepted as first-line systemic therapy around the world. Dapsone and/or retinoids can also be beneficial if the positive and negative effects are carefully considered. It is critical to understand and explore the mechanics of skin eruption development utilizing various methodologies such as mouse models in order to find better treatments. This publication describes the characteristics of DLE, its pathogenesis, diagnostics, and current and novel targeted therapy. A PubMed and Cochrane Database of Systemic Reviews literature search was conducted, and the most relevant references were considered. DLE treatment encompasses both topical and systemic treatments. Professional application of topical corticosteroids and calcineurin inhibitors is considered first-line therapy. When topical treatments are ineffective, antimalarials are accepted as a first-line systemic therapy. An immunosuppressive or immunomodulator may be added if the antimalarial treatment program is insufficient. Some novel promising medicines have been evaluated or tested in patients who have become resistant to various combinations of antimalarials and immunosuppressives/immunomodulators.

Background:Parent-child saliva contact during infancy might stimulate the child's immune system for effective allergy prevention. However, few studies have investigated its relation to allergy development in school-age children. Objective:We sought to investigate the relationship between parent-child saliva contact during infancy and allergy development at school age. Methods:We performed a large multicenter cross-sectional study involving Japanese school children and their parents. The self-administered questionnaires including questions from the International Study of Asthma and Allergies in Childhood were distributed to 3570 elementary and junior high school children in 2 local cities. Data were analyzed for the relationship between saliva contact during infancy (age <12 months) and the risk of allergy development, specifically eczema, allergic rhinitis, and asthma. For detailed Methods, please see the Methods section in this article's Online Repository at www.jacionline.org. Results:The valid response rate was 94.7%. The mean and median age of children was 10.8 ± 2.7 and 11 (interquartile range, 9-13) years, respectively. Saliva contact via sharing eating utensils during infancy was significantly associated with a lower risk of eczema (odds ratio, 0.53; 95% CI, 0.34-0.83) at school age. Saliva contact via parental sucking of pacifiers was significantly associated with a lower risk of eczema (odds ratio, 0.24; 95% CI, 0.10-0.60) and allergic rhinitis (odds ratio, 0.33; 95% CI, 0.15-0.73), and had a borderline association with the risk of asthma in school-age children. Conclusions:Saliva contact during infancy may reduce the risk of developing eczema and allergic rhinitis in school-age children.

Estimating the age and vitality of human skin wounds is essential in forensic practice, and the use of immunohistochemical parameters in this regard remains a challenge. Heat shock proteins (HSPs) are evolutionarily conserved universal proteins that protect biological systems from various types of stress. However, its importance in forensic pathology for determining wound activation in neck compression skin remains unclear. The expression of HSP27 and HSP70 in neck skin samples was immunohistochemically examined to understand its forensic applicability in determining wound vitality. Skin samples were obtained from 45 cases of neck compression (hanging, 32 cases; strangulation, 10 cases; manual strangulation, 2 cases; other, 1 case) during forensic autopsies; intact skin from the same individual was used as a control. HSP27 expression was detected in 17.4% of keratinocytes in the intact skin samples. In the compressed region, the frequency of HSP27 expression in keratinocytes was 75.8%, which was significantly higher than that in intact skin. Similarly, HSP70 expression was 24.8% in intact skin samples and 81.9% in compressed skin samples, significantly higher in compressed skin than in intact skin samples. This increase in case compression cases may be due to the cell defence role of HSPs. From a forensic pathology perspective, the immunohistochemical examination of HSP27 and HSP70 expression in neck skin could be considered a valuable marker for diagnosing traces of antemortem compression.

We report a case of hemoperitoneum after percutaneous radiofrequency ablation in a patient with hepatocellular carcinoma. A 60-year-old female was hospitalized for the treatment of thrombasthenia and cirrhosis caused by chronic Hepatitis C, and computed tomography revealed hepatocellular carcinoma, which was treated by percutaneous radiofrequency ablation. After the ablation, hemoperitoneum was suspected because of the low hemoglobin level with abdominal pain. Approximately 6 h after the ablation treatment, the patient suddenly fell into a shock state and died. In this case, medical treatment-related death including malpractice was suspected, and forensic autopsy was performed. The abdominal cavity contained 910 mL of dark red fluid blood and 210 g of soft hemocoagula. Moreover, several puncture marks were observed on the liver surface and diaphragm, and there was no clear damage to the main arteries and veins. Considering the macroscopic and microscopic findings, the cause of death was assumed as hemorrhagic shock due to the hemoperitoneum caused by the damage to the liver by radiofrequency ablation. It is important to consider all the indications and adverse effects of radiofrequency ablation.

We investigated the dynamics of the gene expression of M1 and M2 macrophage markers during skin wound healing in mice. Expression of M1-macrophage markers, such as Il12a, Tnf, Il6, Il1b, and Nos2 was upregulated after wounding and peaked at 1 or 3 days after injury, and that of M2-macrophage markers such as Mrc1, Cd163, Ccl17, Arg, and Tgfb1, peaked at 6 days after injury. Consistent with these findings, using triple-color immunofluorescence analysis revealed that F4/80 + CD80 + M1 macrophages were more abundant than F4/80 + CD206 + M2 macrophages on day 3 in mouse wound specimens, and that M2 macrophages were prominently detected in day 6 wounds. For application in forensic practice, we examined macrophage polarization using human wound specimens. The average ratios of CD68 + iNOS + M1 macrophages to CD68 + CD163 + M2 macrophages (M1/M2 ratios) were greater than 2.5 for the wounds aged 2–5 days. Out of 11 wounds aged 1–5 days, five samples had the M1/M2 ratios of > 3.0. These observations propose that the M1/M2 ratios of 3.0 would indicate a wound age of 1–5 days as the forensic opinion. This study showed that M1 and M2 macrophages in human skin wound might be a promising marker for wound age determination.

Allergic diseases are currently considered diseases of excessive type 2 inflammation created by orchestration between the innate and acquired immune systems. Since pattern recognition receptors (PRRs) are present in epidermal keratinocytes, it is noteworthy that aggravating factors of allergic diseases act directly on keratinocytes via PRRs. To investigate the relationship between the activation of PRRs and inflammation, we stimulated a keratinocyte cell line (HaCaT cells) with agonists against proteinase-activated receptor-2 (PAR-2), Toll-like receptor (TLR)2, and TLR4, alone or in combination, and we evaluated the changes in inflammatory cytokines and chemokines. Activation of TLR2 or TLR4 alone induced interleukin 6 (IL-6), IL-8, and monocyte chemoattractant protein-1 (MCP-1) in an agonist concentration-dependent manner. Simultaneous activation of TLR2 and TLR4 induced IL-8 synergistically, MCP-1 in an additive trend, and IL-6 weakly but synergistically. PAR-2 activation of HaCaT cells induced IL-6 and IL-8 but suppressed MCP-1 in an agonist concentration-dependent manner. The enhancement of IL-8 and the suppression of MCP-1 by PAR-2 activation were both neutralized by the PAR-2 antagonist AZ3451, supporting the possibility that PAR-2 activation simultaneously induces the following opposing effects in inflammation: enhancement of IL-8 and suppression of MCP-1. The nuclear factor-κB (NF-κB) pathway inhibitor BAY 11-7082 neutralized the induction of IL-8 but not the suppression of MCP-1 by PAR-2 activation, indicating that PAR-2 activation induces activation of the NF-κB pathway, and that the suppression of MCP-1 by PAR-2 activation is not related to the NF-κB pathway.

ABSTR A C T Here, we report a case of necrotizing fasciitis following intra-articular injection of hyaluronic acid. A 73-year-old female received intra-articular injections of hyaluronic acid due to arthralgia at the left shoulder and knee, and was found dead in her living room at one day. At the forensic autopsy, injection marks with bullae and erythema were found at the left shoulder and knee and liquefactive necrosis of muscle tissues was observed in the left but not right extremities. Histopathological examinations of the left upper arm and thigh revealed severe rhabdo-myolysis with lots of bacterial clusters. Bacteriological examinations detected group A Streptococcus from intracardiac blood and affected muscle tissues. Postmortem biochemical analysis of blood showed escalated blood urea nitrogen (133.8 mg/dL), creatinine (4.57 mg/dL) and C-reactive protein (45.0 mg/dL). The cause of her death was diagnosed as streptococcal toxic shock syndrome (STSS). Moreover, it was suggested that the injection was inappropriately conducted and served as a portal of bacterial entry.

Ubiquitin is a member of the heat shock protein family and is rapidly induced by various types of stimuli, including ischemic and mechanical stress. However, its significance in determining wound vitality of neck compression skin in forensic pathology remains unclear. We immunohistochemically examined the expression of ubiquitin in the neck skin samples to understand its forensic applicability in determining wound vitality. Skin samples were obtained from 53 cases of neck compression (hanging, 42 cases; strangulation, 11 cases) during forensic autopsies. Intact skin from the same individual was used as the control. Ubiquitin expression was detected in 73.9% of keratinocytes in intact skin samples, but only in 21.2% of keratinocytes in the compression regions, with statistical differences between the control and compression groups. This depletion in the case of neck compression may be caused by the impaired conversion of conjugated to free ubiquitin and failure of de novo ubiquitin synthesis. From a forensic pathological perspective, immunohistochemical examination of ubiquitin expression in the skin of the neck can be regarded as a valuable marker for diagnosing traces of antemortem compression.

経口イソトレチノインは，皮脂の分泌と毛包漏斗部の角化異常を抑制することで痤瘡を改善することから，海外では集簇性痤瘡あるいは重症・最重症の尋常性痤瘡に対して推奨されているが，本邦では未承認である．そこで，本邦における集簇性痤瘡や重症・最重症の尋常性痤瘡の患者数や現状での治療状況，イソトレチノインに対する考え，使用実態などについて日本臨床皮膚医会（日臨皮）と日本美容皮膚科学会（美容皮膚）の会員を対象に調査を行った． 日臨皮会員4,539名中565名（12.4%），美容皮膚会員2,711名中の158名（5.8%）から回答を得た．その結果，「男性に好発し、顔面のみならず胸背部に、多数の面皰と嚢腫・結節の多発をみる難治性の痤瘡ないし膿皮症の一型」と定義した集簇性痤瘡を両学会会員の85.6%が経験し，うち48.6%が年間1～2例を経験していた．また，経験者の81.7%は「標準治療だけでは治療不可能」と回答し，81.5%は経口イソトレチノインが「必要」，あるいは「必要性がとても高い」と考えていた．従来の治療で十分な効果が得られない重症・最重症の尋常性痤瘡については，90.8%が何らかの形で経験しており，そのうちの75.0%が経口イソトレチノインが「必要」あるいは「必要性が高い」と回答していた．また，何らかの手段でイソトレチノインを現在処方している医師の割合は全体の5.1%（美容皮膚会員15.8%，日臨皮会員2.1%）であった． 本調査では，集簇性痤瘡および従来の治療で十分な効果が得られない重症・最重症の尋常性痤瘡は，稀ではあるが皮膚科医が経験する症状であり，それに対して海外のガイドラインで推奨されている経口イソトレチノインへの期待が高いことが示唆された．経口イソトレチノインの必要性は高く，一部の皮膚科医がすでに処方している実態がある．しかし，催奇形性等の重大な副作用を伴うことから，十分な管理の下で経口イソトレチノインは使用されるべきである．現状の使用状況をより好ましい形にするために，安全性と有効性を確認する臨床試験を経たうえで，早期に薬事承認を目指す必要があると考えた．

Heme oxygenase-1 (HO-1), an inducible stress-response protein, exerts anti-oxidant and anti-apoptotic effects. However, its significance in forensic diagnosis of acute ischemic heart diseases (AIHD) such as myocardial infarction (MI) is still unknown. We examined the immunohistochemical expression of HO-1 in the heart samples to discuss their forensic significance to determine acute cardiac ischemia. The heart samples were obtained from 23 AIHD cases and 33 non-AIHD cases as controls. HO-1 positive signals in cardiomyocyte nuclear were detected in 78.2% of AIHD cases, however, that were detected in only 24.2% control cases with statistical difference between AIHD and non-AIHD groups. In contrast to HO-1 protein expression, there was no significant difference in the appearance of myoglobin pallor regions and leukocyte infiltration in the hearts between AIHD and non-AIHD groups. From the viewpoints of forensic pathology, intracardiac HO-1 expression would be considered a valuable marker to diagnose AIHD as the cause of death.

OBJECTIVES:Blau syndrome is a distinct class of autoinflammatory syndrome presenting with early-onset systemic granulomatosis. Blau syndrome-causing NOD2 mutations located in the central nucleotide-oligomerization domain induce ligand-independent basal NF-κB activation in an in vitro reporter assay. However, the precise role of this signaling on granuloma formation has not yet been clarified.METHODS:Blau syndrome-causing NOD2 mutations were introduced into human monocytic THP-1 cells, and their morphological and molecular changes from parental cells were analyzed. Identified molecules with altered expression were examined in the patient's lesional skin by immunostaining.RESULTS:Although the production of proinflammatory cytokines was not altered without stimulation, mutant NOD2-expressing THP-1 cells attached persistently to the culture plate after stimulation with phorbol myristate acetate. Sustained surface ICAM-1 expression was observed in association with this phenomenon, but neither persistent ICAM-1 mRNA expression nor impaired ADAM17 mRNA expression was revealed. However, the transient induction of PDGF-B mRNA expression was specifically observed in stimulated THP-1 derivatives. In the granulomatous skin lesion of a Blau syndrome patient, ICAM-1 and PDGF-B were positively immunostained in NOD2-expressing giant cells.CONCLUSIONS:Sustained surface ICAM-1 expression and transient PDGF-B production by newly differentiating macrophages harboring mutant NOD2 might play a role in granuloma formation in Blau syndrome.

In recent years of immunology, the understanding of innate immunity has deepened, and the concept of innate immunity has been proposed even in the area of acquired immune subjects. The conventional immunosuppressive treatments have mainly controlled the step of acquired immunity. However, the involvement of innate immunity was clarified for hydroxychloroquine (HCQ), which has been confirmed to be very effective for cutaneous lupus erythematosus (CLE). This review introduces the mechanism of development of CLE from the viewpoint of autoantibodies, cytokines, and innate immunity. Furthermore, the mechanism of HCQ is introduced and discussed.

Objective Pruritus is an important symptom frequently accompanying various inflammatory skin conditions and some recent data indicated that it may be associated with autoimmune connective tissue diseases. The aim of this study was to assess the frequency and clinical presentation of itch in CLE. Methods A multinational, prospective, cross-sectional study was performed to assess the prevalence, intensity and clinical characteristic of pruritus in various subtypes of CLE. A total of 153 patients with active CLE lesions were included. Their age ranged between 17 and 82 years (mean 49.8 ± 15.4 years), and 115 patients (75.2%) were women. The disease activity and damage were assessed according to the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Pruritus severity was assessed with Numeric Rating Scale (NRS) and the 12-Item Pruritus Severity Scale. Dermatology Life Quality Index and EQ-5D questionnaire were used to measure quality of life. Results Pruritus was present in 116 (76.8%) of patients of whom half had NRS scoring equal or above 4 points indicating moderate or severe pruritus. Most commonly itch was localized on the scalp, face (excluding ears and nose) and arms (40.5%, 36.2%, 31.9%, respectively). Sensations connected with pruritus were most frequently described as burning, tingling and like ants crawling feeling, but 31.9% patients described it as “pure itch”. More than half of patients reported that pruritus was present every day, and it was most frequent during the evenings. The pruritus scoring and the CLASI activity score were significantly correlated (r = 0.42, p = 0.0001), while no correlation was found with the CLASI damage score (p = 0.16). Both the maximum and average itch intensity were correlated with systemic lupus erythematosus (SLE) activity measured with the Systemic Lupus Erythematosus Disease Activity Index. Conclusions Pruritus is a common, but frequently overlooked symptom of CLE. Its intensity correlates with the activity of CLE, but not with the skin damage. In more than a half of patients it occurs on a daily basis. The correlation between the intensity of pruritus and the activity of the skin lesions and the systemic involvement indicate that pruritus could be an individual indicator of both SLE and CLE activity.