Different Subtypes Of Cutaneous Lupus Erythematosus Research Articles

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.
 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).
 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.
 Chatt Maa Shi Hosp Med Coll J; Vol.20 (2); July 2021; Page 79-84

Clinicopathological characteristics of cutaneous lupus erythematosus patients in Bangladesh

IntroductionNearly all epidemiologic studies have involved patients with systemic lupus erythematosus (SLE). Few authors have investigated the characteristics of patients with cutaneous lupus erythematosus (CLE).AimTo describe the clinical and pathologic characteristics of a series of patients diagnosed with CLE.Material and methodsThis is a descriptive retrospective cross-sectional study carried out using the consecutive registered records of 218 patients attending the ‘Lupus Clinic’ in Chittagong Medical College Hospital during the period between 2010 and 2020. The activity and damage of CLE were assessed according to the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI).ResultsThere were 187 (85.8%) females and 31 (14.2%) males, with the female:male ratio being 6 : 1. The mean age was 30.0 ±11.7 years. The chronic cutaneous lupus erythematosus (CCLE) patients numbered 154 (70.6%), followed by acute cutaneous lupus erythematosus (ACLE) n = 46 (21.1%), and subacute cutaneous lupus erythematosus (SCLE) n = 18 (8.3%). In LE-specific skin lesions, the most common manifestation was photosensitivity, 198 (90.8%), followed by discoid rash, 155 (71.1%) and maculo-papular lupus rash, 55 (25.2%). Among LE-nonspecific skin lesions, the most common manifestation was non-scarring alopecia, 123 (56.4%), followed by livedo reticularis, 18 (8.3%), Raynaud’s phenomenon, 17 (7.8%), vasculitis, 15 (6.9%), periungual telangiectasia, 7 (3.2%), erythema multiforme, 6 (2.7%) and leg ulcers, 5 (2.3%). Antinuclear antibodies (ANA) were the most common type of autoantibody (n = 132, 60.5%) followed by anti-ds DNA (n = 91, 41.7%) and anti-phospholipid antibodies (n = 9, 4.1%).ConclusionsCCLE was the most common subtypes of CLE. Photosensitivity was the most common clinical manifestation, whereas ANA were the most frequent autoantibodies of the LE patients of this region. Patients with different subtypes of CLE have distinct clinical and pathological characteristics.

Relevant new insights into the effects of photoprotection in cutaneous lupus erythematosus.

Cutaneous lupus erythematosus (CLE) denotes a heterogeneous spectrum of autoimmune diseases that primarily affect the skin. Ultraviolet irradiation (UV) is one of the most important environmental factors that can trigger skin lesions in CLE or even induce systemic organ manifestation. It has been shown that broad-spectrum sunscreen with high sun protection factors can effectively prevent UV-induced skin lesions in patients with different subtypes of CLE. In a recent issue of Experimental Dermatology, Zahn and colleagues demonstrate that potent photoprotection blocks disease-specific skin lesions in CLE patients by reducing lesional tissue damage and interferon-driven inflammation.

Epidemiological Characteristics of Patients With Cutaneous Lupus Erythematosus

Introduction and objectivesLupus erythematosus is a chronic autoimmune inflammatory disease with a wide clinical spectrum and variable clinical course. Few studies have analyzed the characteristics of patients with only cutaneous lupus erythematosus (CLE). The aim of this study was to describe the epidemiological, clinical, and immunological characteristics of a series of patients diagnosed with CLE. Patients and methodsAn analysis was performed of the data from all patients over 18 years of age with a diagnosis of CLE and seen between January 1, 2007 and December 31, 2011 in a tertiary hospital. Results and conclusionsOne hundred one patients were included in the study. The mean age at diagnosis was 40 years and 84% were women. Subacute forms of presentation were observed in 94% of patients. The chronic discoid forms were localized to the head and neck in 79% of cases, whereas the subacute forms were on the trunk in 97% of cases. Patients with acute forms were positive for antinuclear, anti-DNA, anti-smooth muscle, and anti-RNP (ribonucleoprotein) antibodies, whereas anti-Ro and anti-La antibodies predominated in patients with subacute forms. Seventeen patients presented more than 1 subtype of CLE. Fifteen (88%) of these patients received immunosuppressor treatment versus 44 (52%) of the other 84 patients with only 1 subtype of CLE. Patients with distinct subtypes of CLE present different clinical and immunological characteristics. Oral immunosuppressants are often needed to control the disease in a large proportion of patients with different subtypes of CLE.

Características epidemiológicas de los pacientes con lupus eritematoso cutáneo

Introduction and objectivesLupus erythematosus is a chronic autoimmune inflammatory disease with a wide clinical spectrum and variable clinical course. Few studies have analyzed the characteristics of patients with only cutaneous lupus erythematosus (CLE). The aim of this study was to describe the epidemiological, clinical, and immunological characteristics of a series of patients diagnosed with CLE. Patients and methodsAn analysis was performed of the data from all patients over 18 years of age with a diagnosis of CLE and seen between January 1, 2007 and December 31, 2011 in a tertiary hospital. Results and conclusionsOne hundred- one patients were included in the study. The mean age at diagnosis was 40 years and 84% were women. Subacute forms of presentation were observed in 94% of patients. The chronic discoid forms were localized to the head and neck in 79% of cases, whereas the subacute forms were on the trunk in 97% of cases. Patients with acute forms were positive for antinuclear, anti-DNA, anti-smooth muscle, and anti-RNP (ribonucleoprotein) antibodies, whereas anti-Ro and anti-La antibodies predominated in patients with subacute forms. Seventeen patients presented more than 1 subtype of CLE. Fifteen (88%) of these patients received immunosuppressor treatment versus 44 (52%) of the other 84 patients with only 1 subtype of CLE. Patients with distinct subtypes of CLE present different clinical and immunological characteristics. Oral immunosuppressants are often needed to control the disease in a large proportion of patients with different subtypes of CLE.

Efficacy of tacrolimus 0.1% ointment in cutaneous lupus erythematosus: A multicenter, randomized, double-blind, vehicle-controlled trial

Topical calcineurin inhibitors are licensed for the treatment of atopic dermatitis; however, the efficacy of tacrolimus in cutaneous lupus erythematosus (CLE) has only been shown in single case reports. In a multicenter, randomized, double-blind, vehicle-controlled trial, we sought to evaluate the efficacy of tacrolimus 0.1% ointment for skin lesions in CLE. Thirty patients (18 female, 12 male) with different subtypes of CLE were included, and two selected skin lesions in each patient were treated either with tacrolimus 0.1% ointment or vehicle twice daily for 12 weeks. The evaluation included scoring of clinical features, such as erythema, hypertrophy/desquamation, edema, and dysesthesia. Significant improvement (P < .05) was seen in skin lesions of CLE patients treated with tacrolimus 0.1% ointment after 28 and 56 days, but not after 84 days, compared with skin lesions treated with vehicle. Edema responded most rapidly to tacrolimus 0.1% ointment and the effect was significant (P < .001) in comparison to treatment with vehicle after 28 days. Clinical score changes in erythema also showed remarkable improvement (P < .05) after 28 days, but not after 56 and 84 days. Moreover, patients with lupus erythematosus tumidus revealed the highest degree of improvement. None of the patients with CLE demonstrated any major side effects. The study was limited by the small sample size. Explorative subgroup analyses revealed that topical application of tacrolimus 0.1% ointment may provide at least temporary benefit, especially in acute, edematous, non-hyperkeratotic lesions of CLE patients, suggesting that calcineurin inhibitors may represent an alternative treatment for the various disease subtypes.

Expression of antimicrobial peptides in different subtypes of cutaneous lupus erythematosus

Antimicrobial peptides (AMPs) are small effector molecules of the innate immune system with well-known antimicrobial activity. Skin infections rarely occur in patients with cutaneous lupus erythematosus (CLE), and AMP expression in CLE has not been previously evaluated. We aimed to determine the expression of several important AMPs in 3 different subtypes ofCLE. Skin lesions were analyzed for the gene and protein expression of human β-defensin (hBD)-1, -2, and -3; RNase-7; the cathelicidin LL-37; and psoriasin (S100A7) using real-time reverse transcriptase polymerase chain reaction and immunohistochemistry. Skin biopsy specimens of 96 study participants including 47 patients with CLE (15 patients with discoid lupus erythematosus [LE], 11 patients with subacute CLE, and 21 patients with LE tumidus), 34 patients with psoriasis, and 15 healthy control subjects were evaluated in this study. HBD-2, hBD-3, LL-37, and psoriasin were significantly more highly expressed in CLE as compared with healthy controls, and most AMPs were significantly more highly induced in subacute CLE as compared with discoid LE and LE tumidus. AMP gene expression paralleled well with AMP protein expression in CLE and controls. Subacute CLE and discoid LE showed a similar correlation of AMP gene expression (significant correlations between hBD-1 and RNase-7, hBD-2 and hBD-3, hBD-2 and psoriasin, and hBD-3 and psoriasin). The relatively small number of samples and the lack of analysis of the lesional bacterial colonization are a limitation. Several AMPs are increased in CLE at both gene and protein levels. This could explain the low prevalence of skin infections in CLE. It remains to be elucidated whether AMPs play a pathogenic role in CLE.

Photoprotective effects of a broad-spectrum sunscreen in ultraviolet-induced cutaneous lupus erythematosus: A randomized, vehicle-controlled, double-blind study

We sought to assess if the exclusive use of a broad-spectrum sunscreen can prevent skin lesions in patients with different subtypes of cutaneous lupus erythematosus (CLE) induced by ultraviolet (UV) irradiation under standardized conditions. A total of 25 patients with a medical history of photosensitive CLE were included in this monocentric, randomized, vehicle-controlled, double-blind, intraindividual study. The test product and its vehicle were applied 15 minutes before UVA and UVB irradiation of uninvolved skin areas on the upper aspect of the back in a random order, and standardized phototesting was performed daily for 3 consecutive days. Characteristic skin lesions were induced by UVA and UVB irradiation in 16 patients with CLE in the untreated area, and 14 patients showed a positive test result in the vehicle-treated area. In contrast, no eruptions compatible with CLE were observed in the sunscreen-treated area in any of the 25 patients. This resulted in significant differences (P < .001) between UV-irradiated sunscreen-treated versus vehicle-treated areas, and between UV-irradiated sunscreen-treated versus untreated areas. Furthermore, a significant difference (P < .05) was observed concerning the age of disease onset and the patient history of photosensitivity. Patients who were younger than 40 years at onset of CLE reported photosensitivity significantly more often than patients with a higher age of disease onset. None of the patients showed any adverse events from application of the test product or the vehicle. Data resulting from standardized experimental phototesting might not be transferable to a clinical setting. These results indicate clearly that the use of a highly protective broad-spectrum sunscreen can prevent skin lesions in photosensitive patients with different subtypes of CLE.

Evaluation of disease activity and damage in different subtypes of cutaneous lupus erythematosus using the CLASI

The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) is a scoring system for patients with cutaneous lupus erythematosus (CLE) to assess disease activity and damage. Objective The aim of this study was to evaluate whether the CLASI is a useful instrument which reflects the different subtypes of CLE comparably well in each parameter. A total of 50 patients (42 female, 8 male) with different subtypes of CLE, including acute CLE (ACLE), subacute CLE (SCLE), chronic CLE (CCLE) and LE tumidus (LET), from the Departments of Dermatology, University of Düsseldorf, Germany, and Danderyd Hospital, Stockholm, Sweden, were evaluated using the CLASI at one time point. The total CLASI activity score was significantly lower in patients with LET compared with ACLE (P<0.05) and CCLE (P<0.001), and the total CLASI damage score was significantly lower in patients with LET than with ACLE (P<0.05), SCLE (P<0.001) and CCLE (P<0.001). The erythema score and the scale/hypertrophy score were significantly lower in LET than in ACLE (P<0.05, both) and CCLE (P<0.05 and P < 0.001, respectively). The dyspigmentation score was lowest in patients with LET, differing significantly from ACLE (P<0.05), SCLE (P<0.05) and CCLE (P<0.001). The scarring/atrophy/panniculitis score was significantly higher in patients with CCLE in contrast to SCLE and LET (P<0.05 and P<0.001, respectively). These data characterize the CLASI as an overall useful instrument to analyse disease activity and damage in CLE. However, the CLASI does not give an accurate assessment of all disease subtypes; therefore, a revision of the CLASI with critical analysis of all parameters is recommended.

The impact of the EUSCLE core set questionnaire for the assessment of cutaneous lupus erythematosus

Epidemiological data and standard European guidelines for the diagnosis and treatment of cutaneous lupus erythematosus (CLE) are lacking in the current literature. In order to provide a standardized tool for an extensive consistent data collection, a study group of the European Society of Cutaneous Lupus Erythematosus (EUSCLE) recently developed a Core Set Questionnaire for the assessment of patients with different subtypes of CLE. The EUSCLE Core Set Questionnaire includes six sections on patient data, diagnosis, skin involvement, activity and damage of disease, laboratory analysis, and treatment. An instrument like the EUSCLE Core Set Questionnaire is essential to gain a broad and comparable data collection of patients with CLE from different European centres and to achieve consensus concerning clinical standards for the disease. The data will also be important for further characterization of the different CLE subtypes and the evaluation of therapeutic strategies; moreover, the EUSCLE Core Set Questionnaire might also be useful for the comparison of data in clinical trials. In this review, the impact of the EUSCLE Core Set Questionnaire is discussed in detail with regard to clinical and serological features as well as therapeutic modalities in CLE.

Revised Cutaneous Lupus Erythematosus Disease Area and Severity Index (RCLASI): a modified outcome instrument for cutaneous lupus erythematosus

In 2005, a scoring system (CLASI, Cutaneous Lupus Erythematosus Disease Area and Severity Index) was developed for patients with cutaneous lupus erythematosus (CLE) to assess disease 'activity' and 'damage'. However, the CLASI does not give an accurate assessment of the severity in all disease subtypes. The main objective of this study was to analyse critically the included parameters of the CLASI and to revise the activity and damage score taking into account various clinical features of the different subtypes of CLE. The revised CLASI (RCLASI) was also validated for use in clinical trials. Patients and methods A RCLASI was designed with regard to the anatomical region (i.e. face, chest, arms) and morphological aspects (i.e. erythema, scaling/hyperkeratosis, oedema/infiltration, scarring/atrophy) of skin lesions and evaluated by nine dermatologists who scored 12 patients with different subtypes of CLE to estimate inter- and intrarater reliability. Reliability studies demonstrated an intraclass correlation coefficient (ICC) for an inter-rater reliability of 0.89 for the activity score [95% confidence interval (CI) 0.79-0.96] and of 0.79 for the damage score (95% CI 0.62-0.92). The ICC for intrarater reliability for the activity score was 0.92 (95% CI 0.89-0.95) and the ICC for the damage score was 0.95 (95% CI 0.92-0.98). In the present study, a RCLASI was developed by experts, and reliability studies supported the validity and applicability of the revised scoring instrument for CLE. Thus, the RCLASI is a valuable instrument in multicentre studies and for the clinical evaluation of activity and damage in different disease subtypes.

Lupus erythematosus tumidus as a separate subtype of cutaneous lupus erythematosus

Conflicts of interest: none declared. Madam, I read with interest the article by Schmitt et al.1 in a recent issue of the BJD. The article continues the previous legacy of the authors who should be highly commended for their efforts in studying cutaneous lupus erythematosus (CLE), especially lupus erythematosus tumidus (LET). My problem with the paper is that it did not add anything new to what is already known about tumid lupus, neither did it accomplish a resolution to the objectives stated by the authors, namely ‘whether LET can be distinguished as a separate entity in the classification system of the disease’. This objective seems to be ill conceived in view of having at least 25 papers in the reference list (from a total of 46) that have the words ‘lupus tumidus’ in their title. It is difficult to accept such an objective in the first place. Another objective of the authors (as written in the summary) is ‘to compare characteristic features of different subtypes of CLE’. If the authors start with the widely accepted premise that there are different subtypes of CLE that are clearly based on characteristic features of each, what does it mean when the authors say they wanted to compare characteristic features of different subtypes of CLE?

Lupus erythematosus tumidus is a separate subtype of cutaneous lupus erythematosus

Background Lupus erythematosus tumidus (LET) is a rare disease which was first described in 1909 but has not always been considered as a separate entity of cutaneous lupus erythematosus (CLE) in the international literature. Objectives To compare characteristic features of different subtypes of CLE and to analyse whether LET can be distinguished as a separate entity in the classification system of the disease. Methods The study involved 44 patients with CLE, including 24 patients with LET, 12 with discoid lupus erythematosus (DLE) and eight with subacute CLE (SCLE), from two centres in Germany. A core set questionnaire and an SPSS database were designed to enable a consistent statistical analysis. Results Location of skin lesions did not differ significantly between the CLE subtypes; however, the activity score was significantly lower in LET than in DLE (P < 0.01), and the damage score was significantly lower in LET than in SCLE (P < 0.01) and DLE (P < 0.01). Photosensitivity and antinuclear antibodies were confirmed to be different in LET compared with SCLE and DLE but without statistical significance. Moreover, histological analysis of skin biopsy specimens showed that abundant mucin deposition is significantly more present in LET compared with SCLE (P < 0.01) and DLE (P < 0.01) while prominent interface dermatitis and alteration of hair follicles were absent in LET. Conclusions Several significant differences were found between LET and other subtypes of CLE with regard to clinical, histological and laboratory parameters. These data strongly indicate that LET should be defined as a separate entity in the classification of CLE.

Kutaner Lupus erythematodes

Cutaneous lupus erythematosus (CLE) is a heterogenous disorder with a wide range of skin manifestations. Therefore, it has been difficult to develop a unifying concept for classifying CLE from the dermatologic perspective in the past. In 2004, the classification system was updated and includes now acute CLE (ACLE), subacute CLE (SCLE), chronic CLE (CCLE), and intermittent CLE (ICLE). Additional rarely described variants are not listed as separate entities but are included in the classical forms. Diagnosis of the different subtypes of CLE is made by considering genetic, clinical, histopathologic, and immunoserologic findings, with a systematic analysis of individual criteria. In the past years, the etiology and pathogenesis of CLE has been subject of intensive research and it has been shown by several groups that exogenous factors, such as ultraviolet light and drugs, can induce CLE. The first part of this review will enable the reader to identify the various clinical manifestations of CLE and to employ characteristic criteria to assess differential diagnostic considerations.

Upregulation of epidermal surface molecule expression in primary and ultraviolet-induced lesions of lupus erythematosus tumidus.

Lupus erythematosus tumidus (LET), a photosensitive skin disorder with characteristic clinical and histological features, has not been generally accepted as a subset of cutaneous lupus erythematosus (CLE). To analyse the expression of epidermal surface molecules in skin biopsy specimens from patients with LET and to relate the results to other variants of CLE, such as discoid lupus erythematosus (DLE) and subacute CLE (SCLE). In total, 45 patients with different subtypes of CLE were included in the study, and cryostat sections from primary and ultraviolet (UV) A- and UVB-induced skin lesions were investigated using immunohistochemical methods. In contrast to healthy controls, skin lesions of LET showed upregulation of intercellular adhesion molecule-1 (ICAM-1) and histocompatibility class II molecules (HLA-DR), with an expression pattern resembling that seen in DLE and SCLE. Furthermore, staining with a monoclonal antibody against 27E10, a distinct marker for cell activation and differentiation, revealed intense focal or band-like labelling of all epidermal layers independent of the type of lesion. Expression of epidermal surface molecules such as ICAM-1, HLA-DR and 27E10 is equally upregulated in primary and UV-induced lesions of patients with LET, DLE and SCLE. These results support our recent clinical findings that LET represents a distinct subset of CLE with a similar immunopathomechanism rather than a different disease.