Pathogenesis of cutaneous lupus erythematosus: common and different features in distinct subsets

Lupus ◽  
2010 ◽  
Vol 19 (9) ◽  
pp. 1020-1028 ◽  
Author(s):  
J. Wenzel ◽  
S. Zahn ◽  
T. Tüting
Keyword(s):  
Adhesion Molecules ◽  
Complex Network ◽  
Lupus Erythematosus ◽  
Clinical Presentation ◽  
Tissue Injury ◽  
Cutaneous Lupus Erythematosus ◽  
Skin Lesions ◽  
Pathological Features ◽  
Complete Understanding ◽  
Cutaneous Lupus

The term ‘cutaneous lupus erythematosus’ (CLE) comprises several related autoimmune skin disorders, defined as ‘specific’ skin manifestations of lupus erythematosus (LE). The spectrum of clinical presentation of CLE is wide, reaching from mild erythema to disseminated scarring skin lesions. There is increasing knowledge concerning the pathogenesis of LE skin lesions and it has been shown that a complex network of cutaneous cytokines, chemokines and adhesion molecules orchestrate and promote tissue injury observed in LE skin lesions. However, a complete understanding of the diverse pathophysiological mechanisms in the different CLE subsets does not exist. Here we review the main pathological features described in CLE patients against the background of the clinical diversity of different CLE subtypes. Lupus (2010) 19, 1020—1028.

scholarly journals THU0010 GENES ASSOCIATED WITH NUCLEOTIDE OLIGOMERIZATION DOMAIN-LIKE RECEPTOR SIGNALING PATHWAY ARE UPREGULATED IN CUTANEOUS LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 217.3-217
Author(s):  
I. Calderon ◽  
R. Mina
Keyword(s):  
Immune System ◽  
Signaling Pathway ◽  
Lupus Erythematosus ◽  
Innate Immune System ◽  
Cutaneous Lupus Erythematosus ◽  
Skin Lesions ◽  
Innate Immune ◽  
Normal Controls ◽  
Cutaneous Lupus ◽  
Skin Samples

Background:Cutaneous Lupus Erythematosus (CLE) is a disfiguring autoimmune skin disorder with several subtypes: discoid lupus, subacute cutaneous lupus, and acute cutaneous lupus. CLE is associated with defects in the adaptive immune system, and, at times, systemic involvement. The innate immune system is likely involved as seen in the presence of interface dermatitis, which is observed in viral exanthems, and improvement of CLE using inhibitors to membrane-bound Pattern Recognition Receptors.Objectives:Compare the expression of genes associated with the innate immune system in active CLE skin lesions of different subtypes compared to normal skin controls.Methods:Five datasets selected from the Gene Expression Omnibus (GEO) were analyzed using GEO2R to compare the gene expressions between different subtypes of CLE. Search Tool for the Retrieval of Interacting Genes/Proteins (STRING) database, Gene Card, and Kyoto Encyclopedia of Genes and Genomes (KEGG) Pathway analysis were used to identify the interaction and function of specific genes.Results:There were a total of 147 CLE skin samples and 52 normal controls. Genes associated with the Nucleotide-Binding Oligomerization Domain-Like Receptor (NLR) signaling pathway were upregulated in CLE skin samples (adjusted p-value < 0.001). Five genes associated with the NLR signaling pathway, STAT1, OAS1, OAS2, OAS3, and AIM2, were found to be upregulated in skin samples of CLE patients in all datasets, regardless of type, compared to normal controls in all datasets. These five genes are associated with transcription activation, regulation of viral infection, and interferon response.Conclusion:Genes associated with the NLR signaling pathway are upregulated in the skin lesions of CLE patients compared to normal controls, supporting the role of the innate immune system in CLE. Further validation studies using experimental methods are needed.References:[1]Enhanced inflammasome activity in systemic lupus erythematosus is mediated via type I interferon upregulation of interferon regulatory factor 1. Liu J, et al. Arth Rheum. 2017; 69(9): 1840-1849.Disclosure of Interests:None declared

The study of Cutaneous Lupus Erythematosus Disease Area and Severity Index in Indian patients with systemic lupus erythematosus

Lupus ◽  
2011 ◽  
Vol 20 (14) ◽  
pp. 1510-1517 ◽  
Author(s):  
P Salphale ◽  
D Danda ◽  
L Chandrashekar ◽  
D Peter ◽  
N Jayaseeli ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Cutaneous Lupus Erythematosus ◽  
Severity Index ◽  
Skin Lesions ◽  
Systemic Lupus ◽  
Disease Area ◽  
Indian Patients ◽  
The Mean ◽  
Cutaneous Lupus

The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) is a newly described tool used to assess the activity of and damage caused by cutaneous lupus erythematosus (CLE). There is a paucity of data on CLASI from the Indian subcontinent. We sought to determine the applicability of CLASI in specific lesions of CLE in patients with systemic lupus erythematosus (SLE) attending a tertiary care hospital in India. In this prospective, cross-sectional study, 93 patients of SLE with cutaneous lesions were recruited. CLASI activity and damage scores of lupus erythematosus (LE)-specific skin lesions were done in 75 patients with SLE. The mean CLASI activity score was 15.4 ± 9.4 (range 0–39) and the mean damage score was 6.87 ± 7.75 (range 0–30). Higher mean CLASI activity scores were seen in patients with a combination of acute, subacute and chronic CLE and in those with widespread lesions. Patients with longstanding disease and long duration of skin lesions had higher damage scores. This study shows that CLASI is an effective tool to assess cutaneous activity of LE-specific lesions, and the damage caused by them, in Indian patients.

scholarly journals Low number of regulatory T cells in skin lesions of patients with cutaneous lupus erythematosus

2007 ◽  
Vol 56 (6) ◽  
pp. 1910-1920 ◽  
Author(s):  
B. Franz ◽  
B. Fritzsching ◽  
A. Riehl ◽  
N. Oberle ◽  
C.-D. Klemke ◽  
...  
Keyword(s):  
T Cells ◽  
Regulatory T Cells ◽  
Lupus Erythematosus ◽  
Cutaneous Lupus Erythematosus ◽  
Skin Lesions ◽  
Cutaneous Lupus

Photosensitivity, phototesting, and photoprotection in cutaneous lupus erythematosus

Lupus ◽  
2010 ◽  
Vol 19 (9) ◽  
pp. 1036-1046 ◽  
Author(s):  
A. Kuhn ◽  
V. Ruland ◽  
G. Bonsmann
Keyword(s):  
Lupus Erythematosus ◽  
Current Knowledge ◽  
Cutaneous Lupus Erythematosus ◽  
Sun Exposure ◽  
Skin Lesions ◽  
Cellular Adhesion ◽  
Cellular Adhesion Molecules ◽  
Cytokines And Chemokines ◽  
Mediators Of Inflammation ◽  
Cutaneous Lupus

Cutaneous lupus erythematosus (CLE) is a heterogeneous autoimmune disease involving well-defined skin lesions that can be categorized as acute CLE (ACLE), subacute CLE (SCLE), chronic CLE (CCLE), or intermittent CLE (ICLE). It is commonly accepted that ultraviolet (UV) exposure can induce and exacerbate skin lesions in patients with certain subtypes of CLE. Phototesting with UVA and UVB irradiation using a standardized protocol has proven to be a reliable model to study photosensitivity in CLE and to analyse the underlying pathomechanisms of the disease. In addition to UV-mediated induction of apoptosis, the molecular and cellular factors that may underlie the abnormal long-lasting photoreactivity in CLE include mediators of inflammation such as cytokines and chemokines, inducible nitric oxide (NO) synthase (iNOS), and cellular adhesion molecules. The photosensitivity associated with CLE requires education of the patient about avoidance of excessive sun exposure, continuous photoprotection through physical measures such as protective clothing, and daily application of broad-spectrum sunscreens. Novel approaches to UV-protection, such as alpha-MSH or thymidine dinucleotides, might also have an impact on photosensitivity in patients with CLE. In this review, we summarize the current knowledge about photosensitivity in patients with CLE, including an overview of standardized phototesting procedures, possible molecular pathomechanisms, and photoprotection. Lupus (2010) 19, 1036—1046.

scholarly journals Proteome Study of Cutaneous Lupus Erythematosus (CLE) and Dermatomyositis Skin Lesions Reveals IL-16 Is Differentially Upregulated in CLE

2021 ◽  
Author(s):  
Timothy B. Niewold ◽  
Alexander Meves ◽  
Julia S. Lehman ◽  
Karin Popovic-Silwerfeldt ◽  
Aliisa Häyry ◽  
...  
Keyword(s):  
Mass Spectrometry ◽  
Lupus Erythematosus ◽  
Caspase 3 ◽  
Active Form ◽  
Cutaneous Lupus Erythematosus ◽  
Skin Lesions ◽  
Differential Diagnostics ◽  
Skin Biopsies ◽  
Inflammatory Infiltrates ◽  
Cutaneous Lupus

Abstract Background: The objective of the study was to explore disease pathways activated in the inflammatory foci of skin lesions in cutaneous lupus erythematosus (CLE) and dermatomyositis (DM). Methods: Skin biopsies acquired from active CLE and DM lesions, patient (PC) and also healthy controls (HC) were investigated. Biopsy sections were examined by a pathologist and inflammatory foci were laser micro-dissected, captured and proteins within captured tissue were detected in a hypothesis free manner by mass-spectrometry. Protein pathway analysis was performed by string-db.org platform. Findings of interest were confirmed by immunohistochemistry (IHC).Results: Proteome investigation identified interleukin (IL)-16 to be the only detectable and abundant cytokine differentially expressed in CLE compared to DM. Caspase-3, enzyme that cleaves IL-16 into its active form, was detected in low levels. Significantly higher proportion of IL-16 and Caspase-3 positive cells were identified in CLE lesions in comparison to DM, PC and HC. Interferon-regulated proteins (IRP) were abundant in both CLE and DM. Proteomic results indicate more abundant complement deposition in CLE skin lesions. Conclusions: Using hypothesis free mass-spectrometry investigation of CLE inflammatory infiltrates, we identified that IL-16 is the only detectable and highly abundant cytokine, while IRP was a common feature of both CLE and DM. IHC confirmed high expression of IL-16 and caspase-3 in CLE. Our novel molecular findings indicate that IL-16 detection could be useful in differential diagnostics between the two conditions that can display similar histopathological appearance. Potentially, IL-16 could be of interest as a future therapeutic target for CLE.

The devil's in the dosing: severe drug-induced liver injury in a hydroxychloroquine-naive patient with subacute cutaneous lupus erythematosus and porphyria cutanea tarda

Lupus ◽  
2018 ◽  
Vol 27 (8) ◽  
pp. 1383-1386 ◽  
Author(s):  
B Sunkara ◽  
D Roofeh ◽  
S Silver ◽  
T LeBleu Pearson ◽  
M Ettel ◽  
...  
Keyword(s):  
Liver Injury ◽  
Lupus Erythematosus ◽  
Porphyria Cutanea Tarda ◽  
Cutaneous Lupus Erythematosus ◽  
Antibody Test ◽  
Skin Lesions ◽  
Drug Induced ◽  
Naive Patient ◽  
Drug Induced Liver Injury ◽  
Cutaneous Lupus

A 29-year-old woman with a 1.5 year history of photosensitive skin lesions on her hands presented with a malar rash, bullous lesions on her hands, and was diagnosed with subacute lupus erythematosus after serologies revealed a positive antinuclear antibody test (1:2560), and antibodies to Ro/SSA and dsDNA. Hydroxychloroquine (400 mg/day) was prescribed and the patient developed severe drug-induced liver injury. Biopsy of her bullous skin lesions was consistent with porphyria cutanea tarda, as were her serological and urinary exams. She was successfully treated with therapeutic phlebotomy. This case identifies porphyria cutanea tarda as an important differential diagnosis for the rheumatologist to consider when evaluating patients with bullous skin lesions. Hydroxychloroquine in lower doses is an effective treatment for porphyria cutanea tarda; at doses used to treat systemic lupus erythematosus and subacute cutaneous lupus, there is a potentially life-threatening complication of hepatotoxicity.

Selective Expansions of T cells Expressing Vβ38 and Vβ13 in Skin Lesions of Patients with Chronic Cutaneous Lupus Erythematosus

1996 ◽  
Vol 23 (10) ◽  
pp. 670-676 ◽  
Author(s):  
Fukumi Furukawa ◽  
Yoshiki Tokura ◽  
Kayo Matsushita ◽  
Kayoko Iwasaki-Inuzuka ◽  
Kazue Onagi-Suzuki ◽  
...  
Keyword(s):  
T Cells ◽  
Lupus Erythematosus ◽  
Cutaneous Lupus Erythematosus ◽  
Skin Lesions ◽  
Cutaneous Lupus
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