Lesions Of Lupus Erythematosus Research Articles

Lupus Erythematosus Panniculitis in Pregnancy.

A case of lupus erythematosus (LE) panniculitis in pregnancy without any lesions of discoid LE or systemic LE is being reported. There were no systemic symptoms. Her ANA, anti-dsDNA, anti-Ro/SSA, and anti-La/SSB antibodies were within normal limits. Diagnosis of lupus panniculitis was considered on clinical and histopathological grounds. The condition responded favorably to systemic steroid therapy.

Association of Anti–Acidic Ribosomal Protein P0 and Anti–Galectin 3 Antibodies With the Development of Skin Lesions in Systemic Lupus Erythematosus

The specific autoantibodies and antigens that mediate systemic lupus erythematosus (SLE)-related organ injuries remain largely unknown. This study was undertaken to investigate the antibody-mediated immune response that leads to SLE skin lesions. The study included 85 SLE patients with lupus-specific skin lesions and 31 without skin lesions. The reactivity of serum antibody with skin antigens was determined by immunoblotting using human foreskin as the substrate. Skin antigens were identified using mass spectrometry. Serum antibody was isolated by affinity purification and was injected intracutaneously into mouse skin to determine pathogenicity. Serum antibody levels were monitored by enzyme-linked immunosorbent assay. We determined that 78% of the patients with skin lesions had serum antibodies reactive with 35-kd and/or 25-kd skin antigens, which was significantly higher than the percentage of patients without skin lesions (P < 0.0001), suggesting a correlation between immune response and skin lesions. Acidic ribosomal protein P0 (RPLP0) and galectin 3 were 2 target antigens identified from 35-kd and 25-kd proteins, respectively. Purified serum anti-RPLP0 and anti-galectin 3 antibodies induced lupus-like histologic changes after intracutaneous injection. Anti-RPLP0 and anti-galectin 3 antibody levels were significantly higher in SLE patients than in healthy controls and decreased with skin recovery. Anti-galectin 3 antibody levels were not significantly higher in SLE patients than in patients with dermatomyositis or scleroderma, but strongly related to lupus cutaneous vasculitis. Additionally, levels of the 2 antibodies were positively correlated with leukopenia and C3 deficiency, and the anti-RPLP0 antibody level was also positively correlated with arthritis and SLE disease activity. Our findings indicate that the immune response mediated by serum anti-RPLP0 and anti-galectin 3 antibodies plays a key role in the pathogenesis of SLE skin lesions. These findings provide new insights into the mechanism of SLE-related organ disorders.

Anti-high mobility group box 1 (anti-HMGB1) antibodies are not related to the occurrence of cutaneous lesions in systemic lupus erythematosus

Objectives: To investigate whether serum anti-high mobility group box 1 (anti-HMGB1) antibodies are related to the development of skin lesions in systemic lupus erythematosus (SLE).Method: This study involved 21 SLE patients with skin lesions, 18 without skin lesions, and 22 healthy controls. The presence and serum levels of anti-HMGB1-IgG and -IgM were measured by western blot and enzyme-linked immunosorbent assay (ELISA), respectively. HMGB1 expression and serum antibodies deposited in the skin were visualized by immunofluorescence staining.Results: Using western blot analysis, we detected anti-HMGB1-IgG antibodies in 13 out of 21 SLE patients with skin lesions and 11 out of 18 SLE patients without skin lesions (p > 0.05). Serum levels of anti-HMGB1-IgG measured by ELISA were also comparable between the two groups of SLE patients (p > 0.05) but were higher in patients than in healthy controls (p < 0.05). Similar results were found with serum anti-HMGB1-IgM antibodies. HMGB1 accumulated under the stratum corneum in lupus cutaneous lesions without forming immune complexes with IgG or IgM, which were mainly observed along the epidermal–dermal junction. Furthermore, serum anti-HMGB1-IgM was higher in the group of patients with arthritis than in those without arthritis.Conclusions: Based on the findings of the current study, it is unlikely that anti-HMGB1 antibodies play a role in the development of SLE cutaneous lesions.

The lip in lupus erythematosus.

Lupus erythematosus (LE) frequently compromises the skin, lips and oral mucosa. There is a large body of medical and dental literature about the cutaneous and mucosal lesions of LE, but very little has been written specifically about labial lupus. The lip has a peculiar anatomical and histological architecture, and LE lesions at this site may have some distinct features. This article reviews the existing data and adds some new concepts to the issue of labial lupus in all of its presentations, comparing such lesions to the well-established characterization of cutaneous LE (in acute, subacute and chronic lesions), and highlighting some special clinical features that may enable a better diagnosis and differential diagnosis of lip diseases.

FRI0421 Immune Effects of Human Papilloma Virus (HPV) Immunization in Patients with Systemic Lupus Erythematosus (SLE)

BackgroundWomen with SLE show an increased risk for HPV infection and development of precancerous lesions. Although HPV vaccine is safe and effective in healthy females, there are limited data on...

AB0533 Lupusqol and Associations with Clinical Measurements of 104 Russian Patients with Lupus (Renaissance)

MethodsA group of 104 SLE inpatients completed the LupusQoL. Demographic (age, sex, marital status,) and clinical variables (disease duration, disease activity, damage) were recorded.Associations between the 8 LupusQoL domains and...

Diverse vascular lesions in systemic lupus erythematosus and clinical implications

Vascular injury is one of the typical symptoms of systemic lupus erythematosus (SLE), and may play a key role in the choice of treatment strategy and prediction of prognosis. In this review, diverse vascular lesions in SLE and their clinical significance are discussed. The clinical features of vascular disease in SLE differ from organ to organ, and may be extreme with regard to renal vascular lesions. Vascular lesions in SLE may be of inflammatory or thrombotic origin, and immune system dysfunction is considered to be a predominant feature. Numerous lines of evidence suggest that the activation and injury of endothelial cells might play a key role in the pathogenesis. Vascular lesions in SLE are mediated by a complex interaction between the immune system and other contributing factors. Different therapies developed for vascular lesions, both immunosuppressive and nonimmunosuppressive, should be selected based on the different clinical and pathological characteristics, and our future understanding of the different mechanisms involved.

Systemic Lupus Erythematosus Presenting as Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN)-like lesions in acute cutaneous lupus erythematosus [LE]) are an unusual manifestation of systemic LE. We describe a patient with widespread vesiculobullous lesions diagnosed as SJS/TEN-like acute cutaneous LE as the initial presentation of systemic LE. Stevens-Johnson syndrome/TEN-like LE may be differentiated from other vesiculobullous lesions by factors including a history of recent LE exacerbation, photodistribution of lesions, lack of a precipitating infection or medication exposure, minimal mucosal involvement, a prolonged course, response steroid treatment, and histologic and immunofluorescence findings. It is paramount to identify SJS/TEN-like LE as this condition requires early and aggressive intervention. The optimal treatment approach for SJS/TEN-like LE is unclear, and although some case reports have shown glucocorticoids to be useful, there are also reports of cases in which additional measures, such as intravenous immunoglobulin and plasmapheresis, were required to achieve a response. Our patient's condition was refractory to high-dose corticosteroids and intravenous immunoglobulin but was successfully treated using plasma exchange. As such, this treatment may hold potential for improving the care of other patients with refractory SJS/TEN-like LE.

Impact assessment of energy-efficient lighting in patients with lupus erythematosus: a pilot study

Patients with lupus erythematosus (LE) are often abnormally photosensitive. Ultraviolet (UV) exposure can not only induce cutaneous lesions but may also contribute to systemic flares and disease progression. Various forms of energy-efficient lighting have been shown to emit UV radiation. To determine the effects of these emissions on individuals with LE. This assessment investigated cutaneous responses to repeated exposures from three types of lighting: compact fluorescent lamp (CFL), light-emitting diode (LED) and energy-efficient halogen (EEH). The subjects were 15 patients with LE and a control group of five healthy volunteers. No cutaneous LE lesions were induced by any of the light sources. Delayed skin erythema was induced at the site of CFL irradiation in six of the 15 patients with LE and two of the five healthy subjects. Erythema was increased in severity and more persistent in patients with LE. One patient with LE produced a positive delayed erythema to the EEH. A single patient with LE produced immediate abnormal erythemal responses to the CFL, LED and EEH. Further investigation revealed that this patient also had solar urticaria. All other subjects had negative responses to LED exposure. Compact fluorescent lamps emit UV that can induce skin erythema in both individuals with LE and healthy individuals when situated in close proximity. However, this occurs to a greater extent and is more persistent in patients with LE. EEHs emit UVA that can induce erythema in patients with LE. LEDs provide a safer alternative light source without risk of UV exposure.

Photosensitivity, Apoptosis, and Cytokines in the Pathogenesis of Lupus Erythematosus: a Critical Review

The underlying pathomechanisms of lupus erythematosus (LE), a multifactorial autoimmune disease, remain elusive. Due to the clinical evidence demonstrating a clear relationship between ultraviolet (UV) light exposure and skin lesions of LE, photosensitivity has been proven to be an important factor in the pathogenesis of the disease. Standardised photoprovocation with UVA and UVB irradiation has been shown to be a reliable model for evaluating photosensitivity in patients with cutaneous LE (CLE) and analysing the underlying medical conditions of the disease. In this respect, UV irradiation can cause aberrant induction of apoptosis in keratinocytes and contribute to the appearance of excessive apoptotic cells in the skin of CLE patients. Moreover, apoptotic cells that cannot be cleared by phagocytes may undergo secondary necrosis and release proinflammatory compounds and potential autoantigens, which may contribute to the inflammatory micromilieu that leads to formation of skin lesions in the disease. In addition to UV-mediated induction of apoptosis, the molecular and cellular factors that may cause the abnormal long-lasting photoreactivity in CLE include mediators of inflammation, such as cytokines and chemokines. In particular, interferons (IFNs) are important players in the early activation of the immune system and have a specific role in the immunological interface between the innate and the adaptive immune system. The fact that treatment with recombinant type I IFNs (α and β) can induce not only systemic organ manifestations but also LE-like skin lesions provides additional evidence for a pathogenetic role of these IFNs in the disease.

The association of anti-annexin1 antibodies with the occurrence of skin lesions in systemic lupus erythematosus

Anti-annexin1 antibodies are associated with the subtypes of cutaneous lupus and are elevated in systemic lupus erythematosus (SLE) patients. In this study, we investigated the correlation of this antibody with the incidence of SLE skin lesions. The presence of anti-annexin1-IgG and-IgM determined by Western blot was no different among healthy controls and SLE patients with and without skin lesions. Serum levels of anti-annexin1-IgG and -IgM measured by enzyme-linked immunosorbent assay were comparable between patients with and without skin lesions, whereas anti-annexin1-IgM was lower in SLE patients than in healthy controls. Annexin1 was abundantly detected in each epidermal layer in lupus lesional skin. Additionally, anti-annexin1-IgG was higher in SLE patients with arthritis and negatively correlated with white blood cells (WBC). Anti-annexin1-IgM was higher in patients with antinuclear antibody (ANA)-positive sera, and was positively related to hemoglobin and total serum IgM. Collectively, anti-annexin1 antibodies are not related to the incidence of skin lesions in SLE, and annexin1 abundantly distributes in epidermis in lesional skin.

Prognosis of patients with systemic lupus erythematosus and discoid lesions

BACKGROUND It has been observed that patients with systemic lupus erythematosus and discoid lesions have a milder systemic disease. OBJECTIVE To compare the clinical, demographic and autoantibody profile of systemic lupus erythematosus patients with and without discoid lesions. METHODSWe carried out a retrospective study involving 288 systemic lupus erythematosus patients who met at least four classification criteria of the American College of Rheumatology for systemic lupus erythematosus, comparing the clinical, serological and demographic factors between patients with and without discoid manifestations. RESULTS Of the 288 patients, 13.8% had discoid lesions. Univariate analysis found no differences in the prevalence of malar rash, photosensitivity, arthritis, serositis, leukopenia, lymphopenia and hemolytic anemia or anemia of the central nervous system (p = ns). Renal lesions were more common in those without discoid lesions (p =0.016), and hemolysis (p<0.0001) was more common in those with discoid lesions. Regarding the profile of autoantibodies, only the anti-RNP antibody was more common in those with discoid events (p =0.04). In a logistic regression study, only the renal lesions and anti-RNP maintained their associations with discoid manifestations. CONCLUSIONPatients with lesions of systemic lupus erythematosus and discoid lesions have lower prevalence of renal involvement and a greater presence of anti RNP.

Lupus Profundus (Panniculitis): A Potential Mimic of Subcutaneous Panniculitis-like T-Cell Lymphoma

Lupus profundus is a rare manifestation of cutaneous lupus erythematosus, seen in 1% to 3% of patients. It most commonly presents in association with classic chronic cutaneous lesions of discoid lupus erythematosus; however, such lesions, as well as a clinical history of lupus erythematosus, may be lacking. The differential diagnosis for lymphocytic lobular panniculitides is broad; however, the consideration of subcutaneous panniculitis-like T-cell lymphoma is most critical. Recently, there have been several reports emphasizing the overlapping histomorphologic features between lupus profundus and subcutaneous panniculitis-like T-cell lymphoma. Although this is controversial, some authors suggest that patients with lupus profundus are at risk for the development of abnormal, clonal T-cell proliferations and/or overt subcutaneous panniculitis-like T-cell lymphoma. In cases of atypical lymphocytic lobular panniculitis that fail to meet diagnostic criteria for subcutaneous panniculitis-like T-cell lymphoma, patients should be clinically followed indefinitely, as future subcutaneous lymphoma cannot be excluded.

Effects of rituximab-based B-cell depletion therapy on skin manifestations of lupus erythematosus – report of 17 cases and review of the literature

Cutaneous manifestations occur frequently in systemic lupus erythematosus (SLE) and are pathognomonic in subacute-cutaneous lupus erythematosus (SCLE) and chronic cutaneous lupus erythematosus (CCLE). Although B-cell depletion therapy (BCDT) has demonstrated efficacy in SLE with visceral involvement, its usefulness for patients with predominant skin manifestations has not been fully established. In this single-centre, retrospective study 14 consecutive SLE, one CCLE and two SCLE patients with recalcitrant skin involvement were treated with 2 × rituximab 1 g, and 1 × cyclophosphamide 750 mg.Six months after BCDT, nine of 17 (53%) patients were in complete (CR) or partial remission (PR). Relapses occurred in 12 patients (71%) at a mean time of 10 ± 1.8 months after BCDT. A second cycle of BCDT achieved a more sustained remission in seven of nine patients (78%) lasting for a mean time of 18.4 ± 2.7 months. Minor adverse events were experienced by three patients. Mean follow-up was 30 months.Our own results and the literature review demonstrate that BCDT based on rituximab is well tolerated and may be effective for cutaneous lesions of lupus erythematosus. Randomized controlled trials are necessary to further evaluate the value of BCDT for this group of patients.

THU0335 Support vector machines classification of texture parameters of white matter lesions in childhood-onset systemic lupus erythematosus. Possible mechanism to distinguish between demyelination and ischemia

BackgroundTexture analysis (TA) is a branch of image processing which seeks to reduce image information by extracting texture descriptors from the image. White matter hyperintensities (WMH) are frequently observed in...

The presence of clusters of plasmacytoid dendritic cells is a helpful feature for differentiating lupus panniculitis from subcutaneous panniculitis‐like T‐cell lymphoma

Both lupus panniculitis (LP) and subcutaneous panniculitis-like T-cell lymphoma (SPTCL) are characterized by subcutaneous lobular lymphocytic infiltrates, and they are sometimes difficult to differentiate. Recently, plasmacytoid dendritic cells (PDCs) were found to be present in various types of cutaneous lupus erythematosus lesions, including LP, and are supposed to play important pathogenetic roles. The aim of this study was to investigate whether PDCs are differentially present in these two diseases and can be utilized to differentiate them. Conventional histopathological features were also compared. Initial biopsies from 21 LP and 11 SPTCL patients were analysed. Our results showed that the presence of lymphoid follicles, dermal mucin deposition and lack of moderate to marked nuclear atypia or adipocyte rimming were more suggestive of LP. Several distinct patterns of fat necrosis, i.e. hyaline/lipomembranous and fibrinoid/coagulative in LP and SPTCL, respectively, were also diagnostically useful. Also, clusters of PDCs were characteristically seen in LP lesions (17/21, 81%) but not in SPTCL lesions (2/11, 18.2%). In LP lesions, but not in SPTCL lesions, the presence of epidermal interface change correlated perfectly with the presence of PDCs in the papillary dermis. We conclude that the presence of clusters of PDCs and certain histological features are helpful for the differential diagnosis.

Oral manifestations of lupus erythematosus – report of two cases

Lupus erythematosus is a serious autoimmune disease, which can cause oral mucosal lesions, manifesting as white striae and erythematous, atrophic and hyperkeratotic areas, as well as erosions and ulcerations. Sometimes, these signs can be misdiagnosed as other oral diseases such as lichen planus. Also, on histopathological examination with haematoxylin-eosin staining (HE), the features of these diseases can overlap. The authors report here two cases of oral lesions of lupus erythematosus, with a discussion of their clinical features and the process of differential diagnosis. It is crucial to consider in the diagnostic process for lupus erythematosus the whole clinical information as well as haematological tests, histopathological features in HE and direct immunofluorescence, not only at first evaluation but also during patient follow-up.

Annular subacute cutaneous lupus erythematosus lesions and polymyositis onset in a patient with primary Sjögren's syndrome: how should this unusual association be classified?

Classification of the wide variety of autoimmune diseases that can occur before or after the onset of Sjögren's syndrome (SS) is currently debated within the conventional SS criteria or as primary SS (pSS) developing autoimmune disease or as 'associated-overlap' with other systemic autoimmune diseases. There is also debate on whether or not to consider annular polycyclic subacute cutaneous lupus erythematosus (SCLE) and annular erythema associated with Sjögren's syndrome (AESS) as a spectrum linked to Ro-SSA and/or La-SSB auto-antibodies (SSA/SSB auto-ab). We present the case of a 55-year-old female patient, with pSS positive for SSA and SSB auto-ab, who developed chronic relapsing polymyositis and atypical annular non-polycyclic SCLE lesions resembling AESS, which seemed to suggest a common spectrum. While a chronic-progressive polymyositis may be generally accepted as a relatively rare myositis complicating pSS, interpretation of annular lesions of non-systemic SCLE in SS patients might actually be underestimated as pSS skin manifestation likely related to SSA/SSB auto-ab.

Hypertrophic (Verrucous) Cutaneous Lupus Erythematosus of the Lip and Oral Cavity: A Series of 4 Cases

So-called “hypertrophic cutaneous lupus erythematosus (LE)” (also termed “verrucous cutaneous LE”) is a dis-tinct and rare clinical variant of chronic cutaneous LE that is characterized by intense tissue hyperplasia and hyperkeratosis. Hypertrophic LE causes marked disfigu-rement and is usually resistant to therapy (1, 2). Lesions occur more commonly on the face and upper limbs, with no specific mention in the literature of lesions occurring on the lips or oral mucosa. We describe here a series of 4 cases with a diagnosis of LE who presented with hypertrophic lesions on the skin and oral mucosa. Clinical and laboratory data are presented, together with detailed characterization of the mucosal involvement.CaSE REpoRtSClinical data for the 4 patients are shown in table SI (available from http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1433). all patients presented hypertrophic cutaneous LE of longstanding duration. only patient 1 had multiple active cutaneous LE lesions at the time of examination (Fig. 1A, B). Skin lesions in patients 2 and 4 were mainly residual, with intense scarring. The lesion in patent 3 was confined to the lip area (Fig. 1C–F). Histopathology of biopsied oral lesions revealed marked hyperkeratosis, presence of a granular layer, focal hydropic degeneration of the basal layer, necro-tic basal keratinocytes, and melanophages of variable intensity (Fig. 1G, H). There were no signs of kerati-nocytic atypia. Dermal components included dilated papillary vessels, mucin, and scarce inflammatory in-filtrate, except for case 2, in which inflammatory cells were abundant in a superficial and deep pattern. Direct immunofluorescence findings were non-diagnostic.In patients 1, 2 and 3, LE activity was more pronoun -ced on the tegument. In addition to skin lesions, patient 4 had a several year history of lupus arthritis.DISCuSSIonthere are few published case series of oral compromise in LE. of these, some uniformity can be observed in the clinical picture, despite the great variety of descriptive terms employed (“oral discoid lesion”, “chronic pla-que”, “lupus cheilitis”, “acute ulcer”, “erythematous ulcer”, “ulcerated plaque”, “pebbly red areas”, “ho-neycomb lesions”, “keratotic white lesion”, “purpuric lesion”, and “diffuse palatal petechial erythema”, among others); a clinical characterization based on established dermatological criteria would be useful. Our group has proposed a method that simply compares mucosal LE lesions with their cutaneous counterparts (acute, subacute and chronic mucosal LE in analogy to acute, subacute and chronic cutaneous LE) in order to better understand those lesions (3).We describe here 4 patients with what is known as hypertrophic/verrucous LE (a subtype of chronic cuta -

Microinvasive squamous cell carcinoma arising in discoid lupus erythematosus lesions successfully treated with imiquimod 5% cream

Microinvasive squamous cell carcinoma arising in discoid lupus erythematosus lesions successfully treated with imiquimod 5% cream