Features Of Cutaneous Lupus Erythematosus Research Articles

Dermoscopic Characteristics of Cutaneous Lupus Erythematosus According to Subtype, Lesion Location, Lesion Duration, and CLASI Score.

Dermoscopic findings are used to diagnose and evaluate disease activity in patients with cutaneous lupus erythematosus (CLE). This study aimed to characterize the dermoscopic features of discoid LE (DLE) and LE tumidus (LET) by lesion duration and CLE Disease Area and Severity Index (CLASI) scores and to examine the dermoscopic findings of lesions in different locations in DLE patients. Dermoscopic findings (follicular features, perifollicular surface, interfollicular features, and vessel pattern) were assessed and lesion duration (≤12 and >12 months) and CLASI scores (grouped as mild or moderate) were calculated. DLE lesion locations were categorized as, non-scalp, scalp and lip. Forty-eight dermoscopic images from 35 DLE and 4 LET patients were analyzed. The most common dermoscopic findings in non-scalp DLE were follicular keratotic plugs (82.8%) and white scales (69%). In scalp DLE (n=9), the most common findings were absent follicular openings (77.8%), white structureless areas (77.8%), and perifollicular scaling (66.7%). All LET patients had pink-white background and linear vessels. Follicular plugs, peripheral pigmentation, and polymorphous vessels were lower in patients with mild CLASI activity than moderate activity (P = 0.036, 0.039, and 0.019, respectively). Fibrotic white dots, honeycomb pigment pattern, and blue-gray dots/globules were lower in those with mild CLASI damage scores than moderate damage (P = 0.010, 0.010, and 0.020, respectively). Peripheral pigmentation was more common in patients with lesion duration ≤12 months, while blue-gray dots/globules were more common with lesion durations >12 months. Certain dermoscopic features may facilitate the differential diagnosis of DLE and LET.

Noncytotoxic Inhibition of the Immunoproteasome Regulates Human Immune Cells In Vitro and Suppresses Cutaneous Inflammation in the Mouse.

Inhibitors of the immunoproteasome (i-20S) have shown promise in mouse models of autoimmune diseases and allograft rejection. In this study, we used a novel inhibitor of the immunoproteasome, PKS3053, that is reversible, noncovalent, tight-binding, and highly selective for the β5i subunit of the i-20S to evaluate the role that i-20S plays in regulating immune responses in vitro and in vivo. In contrast to irreversible, less-selective inhibitors, PKS3053 did not kill any of the primary human cell types tested, including plasmacytoid dendritic cells, conventional dendritic cells, macrophages, and T cells, all of which expressed genes encoding both the constitutive proteasome (c-20S) and i-20S. PKS3053 reduced TLR-dependent activation of plasmacytoid dendritic cells, decreasing their maturation and IFN-α response and reducing their ability to activate allogenic T cells. In addition, PKS3053 reduced T cell proliferation directly and inhibited TLR-mediated activation of conventional dendritic cells and macrophages. In a mouse model of skin injury that shares some features of cutaneous lupus erythematosus, blocking i-20S decreased inflammation, cellular infiltration, and tissue damage. We conclude that the immunoproteasome is involved in the activation of innate and adaptive immune cells, that their activation can be suppressed with an i-20S inhibitor without killing them, and that selective inhibition of β5i holds promise as a potential therapy for inflammatory skin diseases such as psoriasis, cutaneous lupus erythematosus, and systemic sclerosis.

Current Insights in Cutaneous Lupus Erythematosus Immunopathogenesis.

Cutaneous Lupus Erythematosus (CLE) is a clinically diverse group of autoimmune skin diseases with shared histological features of interface dermatitis and autoantibodies deposited at the dermal–epidermal junction. Various genetic and environmental triggers of CLE promote infiltration of T cells, B cells, neutrophils, antigen presenting cells, and NK cells into lesional skin. In this mini-review, we will discuss the clinical features of CLE, insights into CLE immunopathogenesis, and novel treatment approaches.

Clinical characteristics of pediatric cutaneous lupus erythematosus: experience from a tertiary referral center in Korea

The clinical characteristics of cutaneous lupus erythematosus (CLE) are well delineated in adults, but pediatric data, particularly in Asian populations, are limited. Therefore, we evaluated the characteristics of pediatric cases by retrospectively reviewing the medical records of children with CLE during a 15-year period in a tertiary care dermatology clinic in South Korea. The study included 21 children (8 males and 13 females), 4 of whom had neonatal lupus erythematosus (NLE). Among 17 patients with CLE, discoid lupus erythematosus (DLE) was most common (47.1%), followed by acute CLE (ACLE, 35.3%). All ACLE cases had systemic lupus erythematosus (SLE). Female predominance was conspicuous in ACLE/SLE (6/11 females versus 0/6 males), as was older age, whereas DLE and NLE showed near-equal sex distributions. The median age at the diagnosis of CLE was significantly higher in females than in males (15 years versus 4.5 years, p = 0.02). All patients with ACLE/SLE simultaneously showed skin and systemic symptoms from onset. The kidney was the most commonly involved organ. This study revealed unique characteristics of pediatric CLE, further warranting a comprehensive review among various ethnicities to understand the wide spectrum of CLE in the pediatric population.

Ten‐year retrospective clinicohistological study of cutaneous lupus erythematosus in Korea

An understanding of the differences in clinical manifestations and laboratory abnormalities between subtypes of cutaneous lupus erythematosus (CLE) is still lacking. The purpose of this study was to analyze demographic, clinical and histological features of CLE according to three main presentation subsets: acute (ACLE), subacute (SCLE) and chronic (CCLE). A 10-year retrospective analysis was performed on data from patients who were diagnosed with CLE between March 2005 and September 2015 in a Korean tertiary referral dermatology clinic. We compared demographic data and clinical and histological findings between three different CLE groups. An overall sample of 220 patients with CLE consisted of 67 patients with ACLE, 25 patients with SCLE and 135 patients with CCLE. Patients with CCLE regardless of systemic lupus erythematosus (SLE) presence had lower prevalence of anemia, urinary abnormalities and elevated erythrocyte sedimentation rate. Furthermore, CCLE patients who only had skin lesions showed lower female predominance, lower extracutaneous manifestation, fewer laboratory and immunological abnormalities including low antinuclear antibody titers and the lowest positivity for C3, C4 and anti-dsDNA, anti-Ro, anti-Sm and anti-RNP antibodies, and more prominent perieccrine inflammation and dermal fibrosis in histological findings. Considering distinct cutaneous manifestations of LE, a comprehensive awareness of each CLE subtype is important for achieving a favorable prognosis through appropriate diagnosis and management. This study provides comparative clinical and histological profiles of patients with different CLE subtypes in Korea.

Clinical characteristics of cutaneous lupus erythematosus.

IntroductionLupus erythematosus (LE) shows a wide variety of clinical manifestations, skin involvement being one of the most important.AimTo analyze the clinical presentation of cutaneous variants of lupus erythematosus in terms of skin lesion spectrum and extracutaneous involvement.Material and methodsA total of 64 patients with cutaneous LE (CLE) were included. The study was based on the “Core Set Questionnaire” developed by the European Society of Cutaneous Lupus Erythematosus (EUSCLE). Clinical severity of skin lesions was evaluated with the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). All results were subjected to statistical analysis.ResultsFifteen (23.4%) patients had an acute CLE (ACLE), 26 (40.6%) subacute CLE (SCLE) and 21 (32.8%) chronic CLE (CCLE). Two (3.2%) individuals only demonstrated urticarial vasculitis as a cutaneous manifestation of LE and these patients were excluded. Patients with ACLE were characterized by the earliest onset of the disease (mean age of 31.9 ±15.0 years; p < 0.001). On average, 4.8 ±1.8 criteria of systemic LE were found in the ACLE group compared to 2.7 ±1.3 criteria in SCLE and 2.5 ±1.5 criteria in CCLE (p < 0.001). The highest activity of skin lesions according to CLASI was found in the SCLE group (p = 0.002). On the other hand, the most severe skin damage was observed in CCLE (p < 0.01).ConclusionsEach variant of CLE differs significantly from the others in respect of various aspects of clinical manifestations. Due to a number of different variants of LE skin lesions, a unified classification of CLE still remains a challenge.

Effects of mycophenolate mofetil on cutaneous lupus erythematosus in (NZB × NZW) F1 mice

BackgroundFew studies have evaluated the effects and precise molecular mechanism of mycophenolate mofetil (MMF) in the treatment of human cutaneous lupus erythematosus (CLE). Our findings shed light on the therapeutic effects of MMF in a UVB-induced NZB × NZW (NZBW) F1 CLE mouse model. MethodsContinuous MMF treatment (60 mg/kg/day) was administered up to Day 50 from the beginning of UVB induction (Day 0; 20 weeks old), as the pathologic features of CLE are present after 50 days. The therapeutic effects of MMF treatment in NZBW lupus mice were examined by comparing histopathological changes, lupus band test (deposition of immune complexes at the dermal–epidermal junction) and colocalization of autoantibodies with a dermal autoantigen Dsg3, and by evaluating the associations of local matrix metalloprotease activities. ResultsMMF improved survival in the NZBW lupus mice from 35.7% to 81.8%. The proteinuria, blood urea nitrogen, and interleukin 6 levels were significantly reduced after MMF treatment. The dermal lymphocytic infiltration, deposition of immune complexes at the dermal–epidermal junction, colocalized autoantibodies with Dsg3, and epidermal matrix metalloprotease activity were also attenuated in MMF-treated NZBW F1 mice. ConclusionThe results confirmed that MMF could substantially attenuate skin damage due to CLE in the NZBW F1 mouse model.

Demographic and clinical characteristics of cutaneous lupus erythematosus at a paediatric dermatology referral centre

Paediatric cutaneous lupus erythematosus (CLE) is uncommon and inadequately described in the literature. Similar to adults, children with CLE develop LE-specific and/or LE-nonspecific skin findings. Similarities and differences in demographics and clinical course between paediatric and adult CLE have not been sufficiently described. To detail the demographic and clinical features of paediatric CLE and compare these findings with those reported in the adult literature. A retrospective chart review was performed of 53 children seen in a paediatric dermatology clinic with cutaneous manifestations of LE. Patients presented with all five major subtypes of CLE, with some notable differences from adult CLE and previously published reports of paediatric CLE. Progression from discoid LE to systemic LE (SLE) did not occur in our cohort. Patients with subacute CLE were more likely than adults to have lesions below the waist as well as concomitant SLE. Sex distribution for CLE in our study was equal prior to puberty and female predominant in post-pubertal patients. Children with CLE have variable clinical presentations and progression to SLE that may be different from adult disease. Specifically, children with acute and subacute CLE may be more likely than adults to have systemic disease; therefore, patients with these subtypes should be monitored closely for evidence of SLE. Study limitations included small patient numbers that may limit the ability to generalize these data and relatively short follow-up intervals.

Pure cutaneous lupus erythematosus in a population of African descent in French Guiana: a retrospective population-based description

The objective of this study was to examine the characteristics of cutaneous lupus erythematosus, excluding systemic lupus erythematosus (SLE), in patients of African descent. Indeed, since the description of subacute cutaneous lupus erythematosus (SCLE), which had been included in chronic cutaneous lupus erythematosus (CCLE), there has been no description of the disease in black patients. In 2000, we performed a retrospective epidemiological study by querying multiple sources to identify all patients with lupus in French Guiana--a part of France in South America having western living conditions, free healthcare and 157,000 inhabitants, most of whom are of African origin. We found 45 patients with pure cutaneous lupus, which included CCLE (mostly discoid), SCLE and bullous lupus. The disease characteristics of these patients exhibited few differences compared with those of the Caucasian patients cited in the literature. However, the age of onset for our patients of African descent was younger than that of Caucasian patients. In contrast to the race-related differences reported for SLE, we found no major differences in terms of demographic, clinical and biological presentation between this cohort of pure cutaneous lupus erythematosus patients of African origin and Caucasian patients with similar forms of lupus.

Cutaneous lupus erythematosus on the elbows: a peculiar localization

The presence of lesions limited to the elbows as expression of a cutaneous lupus erythematosus (CLE) is very unusual. To describe the clinical and microscopic characteristics of these lesions, as well as their relationship with the different cutaneous lupus erythematosus subsets. Seven cases of CLE with lesions on the elbows, collected from 1998 to 2009, were retrospectively analysed. All patients had a previous or concomitant diagnosis of lupus erythematosus based on other typical skin lesions for each subtype of lupus erythematosus, and they all met clinical and microscopic criteria. Six patients were women with a mean age of 33.8 years. Five cases had been previously diagnosed with CLE (three lupus erythematosus [LE] tumidus, one subacute CLE and one acute CLE). In the other two cases, these lesions were the first manifestation of the disease. The lesions consisted of pruriginous, erythematous papules and plaques with a slightly scaly surface, located on both elbows. Microscopically, in addition to the typical features of CLE, other changes were observed, including alterations in the texture and the staining properties of collagen fibres (five cases), an interstitial histiocytic infiltrate (four cases), eosinophils (one case) and a Churg-Strauss granuloma (one case). These peculiar lesions located on the elbows should be included in the spectrum of cutaneous manifestations of LE. Their histopathology combines changes of both LE and interstitial granulomatous dermatitis.

Cutaneous lupus erythematosus: recent lessons from animal models

Cutaneous lupus erythematosus (CLE) may present as a clinically heterogeneous group of lupus-specific skin lesions that have common histopathological findings. Determination of the immunopathological sequence of events in this group of disorders has been challenging for dermatologists and immunologists but is vital for therapeutic targeting. We review animal models in which different aspects of immune alteration in CLE have been addressed. The MRL/lpr mouse develops spontaneous skin disease with some features of CLE. Study of this strain and related gene-manipulated strains has revealed roles for multiple cytokines, including interleukin (IL)-6, IL-18, and IL-21, in disease pathogenesis. A role for the growth factor colony stimulating factor 1 and the inflammatory protein high-mobility group box 1 has also been suggested. We discuss potential novel treatment options suggested by these models.

Involvement of scalp and nails in lupus erythematosus

Lupus erythematosus (LE) is a systemic autoimmune disorder associated with polyclonal B-cell activation resulting in diverse patterns of autoantibody production and a heterogeneous clinical expression constituting a spectrum extending from limited cutaneous disease to life-threatening systemic manifestations. For daily clinical practice, the characteristics of cutaneous lupus erythematosus (CLE) have been well defined in terms of morphology, and clinical and laboratory criteria are available for the classification as systemic lupus erythematosus (SLE). The many different types of skin lesions encountered in patients with LE have been classified into those that are histologically specific for LE and those that are not. While LE non-specific skin lesions on their own do not enable a diagnosis of LE, they can be important reflections of underlying SLE disease activity. This also applies to the involvement of the scalp and nails. Finally, it must be kept in mind that LE patients may also develop drug-related, or other unrelated common disorders of the hair and nails that do not reflect LE disease activity.

Concurrent features of cutaneous lupus erythematosus and pemphigus erythematosus following myasthenia gravis and thymoma

A case that exhibits the pathologic features of cutaneous lupus erythematosus and the immunopathologic characteristics of pemphigus erythematosus following myasthenia gravis and thymoma is presented, and the literature pertaining to these disease associations is reviewed. The findings in this case were analyzed with respect to the accepted diagnostic criteria for lupus erythematosus, pemphigus, and pemphigus erythematosus. The pathogenic mechanisms that lead to acantholysis in pemphigus are examined in an attempt to explain why acantholysis did not occur in this case.