Lupus Erythematosus Tumidus Research Articles

Tumid Lupus Erythematosus (TLE): A Review of a Rare Variant of Chronic Cutaneous Lupus Erythematosus (cCLE) with Emphasis on Differential Diagnosis.

Tumid lupus erythematosus (TLE) has been the subject of heated debate regarding its correct nosographic classification. The definition of TLE has changed over time, varying according to the different studies performed. In this review, we address the initial definition of TLE, the changes that have taken place in the understanding of TLE, and its placement within the classification of cutaneous lupus erythematosus (CLE), with a focus on clinical, histopathological, immunophenotypical, and differential diagnosis aspects.

Tildrakizumab in Psoriasis and Beyond

Background: Tildrakizumab is an interleukin-23p19 inhibitor, approved by the Food and Drug Administration for the management of moderate-to-severe plaque psoriasis. Purpose: This review aims to describe the dermatological implications and applications of tildrakizumab. Methods: PubMed and Google Scholar were searched for scholarly articles related to tildrakizumab and its utility in dermatology using the search terms “Tildrakizumab” AND “Psoriasis” AND “other dermatological disorders.” Results: Tildrakizumab is a valuable biologic agent for the management of psoriasis. It has also been successfully used for other dermatologic disorders such as hidradenitis suppurativa, lichen planus, pityriasis rubra pilaris, lupus erythematosus tumidus, and pyoderma gangrenosum. Conclusion: Tildrakizumab’s usage is not limited to psoriasis. Its benefit extends to many more dermatologic conditions. Besides, it has an acceptable safety profile.

Hydroxychloroquine-induced generalized myopathy in a patient with lupus tumidus: a case report.

A subtype of cutaneous lupus erythematosus known as lupus erythematosus tumidus (LET) is characterized by sun-exposed areas that typically display urticaria-like papules and plaques. For LET, systemic therapy with antimalarials - particularly hydroxychloroquine (HCQ) - is the first line of treatment. Even though the safety profile of these medications appears to be high, there have been very few reports of side effects in the literature, including hemolytic anemia, retinal toxicity, maculopapular rash, gastrointestinal disturbance, and blue-gray discoloration of the skin or mucous membranes. Here, we report a unique instance of a 46-year-old LET smoker who, following HCQ treatment, developed a generalized myopathy.

CPC07 Lupus comedonicus: a not so common entity

Abstract Lupus comedonicus (LC) is an exceedingly rare variant of chronic cutaneous lupus erythematosus (CCLE), with only 13 reported cases in the literature to date. While its aetiology remains unknown, hallmarks include the presence of comedones on erythematous plaques, particularly in seborrhoeic areas. We report a case of LC involving the scalp, concha bowl and upper back. A 53-year-old female smoker with a 20-pack-year history and previous unprovoked pulmonary embolism presented to dermatology with a 2-year history of alopecia and rash affecting her face and upper back. She was initially reviewed by oral medicine for ulcers, where erythematous buccal mucosal plaques with white striations were seen. The initial biopsy showed a patchy lichenoid inflammatory infiltrate, raising suspicions for lichen planus; however, repeat biopsy highlighted perivascular inflammation and keratin plugging suggestive of lupus. Full skin examination revealed patchy scarring alopecia with perifollicular scale and erythema. Erythematous infiltrated plaques were seen on the face with similar more indurated lesions on the upper back associated with open comedones. The eponymous Shuster sign was observed in the left conchal bowl, with associated follicular occlusion. Laboratory studies all returned within normal limits, including antinuclear antibody titres and complement levels. Differential diagnosis included lupus erythematosus tumidus, discoid lupus erythematosus and LC. Lesional biopsies taken from her back confirmed LC, highlighting lichenoid inflammation involving follicles and interfollicular skin, with superficial and deep perivascular infiltrate. We commenced oral hydroxychloroquine at 200 mg once daily for management, with topical mometasone furoate 0.1% to be used while awaiting its full effect. Smoking cessation has also been advised given the potentially antagonistic effect of smoking with lupus and hydroxychloroquine. Hypotheses of the pathogenesis of LC include the effect of sun exposure. Ultraviolet radiation is known to not only enhance the activity of CCLE but may also contribute to the formation of comedones. Actinic damage alters collagen of normal skin, promoting sebum retention and follicular plugging. Given its infrequency, LC could be mistaken for benign conditions, including acne vulgaris, naevus comedonicus and Favre–Racouchot disease. It is plausible that the small case numbers are due to current underdiagnoses. It is important for dermatologists to be aware of and accurately diagnose LC. Apart from its potentially destructive, scarring complications, especially in untreated and misdiagnosed cases, it is associated with systemic lupus in 30% of cases. Delay in diagnosis and therapeutic management can be associated with significant potential morbidity and even mortality.

Systemic lupus erythematosus presenting as lupus erythematosus tumidus and lupus nephritis: a case report

BackgroundLupus nephritis and lupus erythematosus tumidus (LET) are uncommon manifestations of systemic lupus erythematosus (SLE), and their coexistence as the initial presentation of SLE is exceedingly rare. Here, we report such a case, emphasizing the diagnostic challenges and therapeutic implications of this unusual association.Case reportA 38-year-old North African woman presented in Nephrology department with a history of lower extremity edema, fatigue, and weight loss of 3 kg in 4 weeks. Physical examination revealed LET lesions on the chest and the Neck. Laboratory investigations showed lymphopenia, low C3 and C4 complement levels, positive antinuclear antibodies, anti-dsDNA antibodies, and anti-SSA/Ro antibodies. Renal function tests showed normal serum creatinine and nephrotic proteinuria. Renal biopsy revealed Class V lupus nephritis. Skin biopsy confirmed the diagnosis of LET, with the presence of lymphohistiocytic infiltrates and dermal mucin. The patient was diagnosed with SLE based on the 2019 EULAR/ACR criteria and treated with prednisone (1 mg/kg/day) and hydroxychloroquine. She showed significant improvement in her cutaneous and renal symptoms at 6 and 12 months follow-up.ConclusionThe rarity of the coexistence of LET and lupus nephritis as the initial manifestation of SLE, especially in the North African population, underscores the need for further research to elucidate the immunopathogenic mechanisms and prognostic factors associated with this association.

A case of lupus erythematosus tumidus misdiagnosed as erysipelas

A patient presented with erythema and oedema on the right eyelid, malar region and left thigh with pruritus and pain. She was diagnosed with erysipelas and had been treated with anti-inflammatory drugs, but the symptoms did not improve. Laboratory investigation and biopsy showed that she was consistent with lupus erythematosus tumidus. It's a typical and rare diease relatively.

Successful treatment of a child's lupus erythematosus tumidus with antimalarials.

Successful treatment of a child's lupus erythematosus tumidus with antimalarials.

Characterization of B cells in lupus erythematosus skin biopsies in the context of different immune cell infiltration patterns.

Cutaneous lesions in lupus erythematosus (LE) subtypes are heterogenous. In line with the heterogeneity of the clinical presentation, the underlying lesional inflammation in LE skin samples is defined by different immune cell infiltrates. Pathophysiologically, lesional inflammation is driven by autoreactive cytotoxic T cells, targeting keratinocytes; plasmacytoid dendritic cells (pDCs), producing large amounts of interferon (IFN); and B cells, whose function in cutaneous LE is still unclear. This study aims to (a) classify inflammatory patterns with regard to the dominating cell type or cytokine expression and (b) investigating the specific role of B cells in LE skin lesions. Therefore, the immunohistological expression of inflammatory surrogates (CD20, CD123, MXA) in skin samples of n = 119 LE (subtypes: subacute cutaneous LE, chronic discoid LE, chilblain LE, LE tumidus, other LE) and n = 17 patients with inflammatory skin diseases (atopic dermatitis, psoriasis) were assessed. Samples were classified with regard to inflammatory groups. In addition multiplex-immunohistochemical analyses of n = 17 LE skin samples focusing on lesional B cells were conducted. In this study, we show that cutaneous lesions present with eight different inflammatory groups dominated by B cells, pDCs, a strong IFN expression, or overlapping patterns. Altogether, LE subtypes show heterogenous infiltration regardless of LE subtype, certain subtypes display a preference for infiltration groups. Furthermore, lesional B cells either form diffuse infiltrates or pseudofollicular structures, wherein they show antigen-presenting and T cell-activating properties. Altogether, in the light of emerging targeted therapeutic options, we suggest histological assessment in regard to B-cell or pDC preponderance to allow tailored treatment decisions.

Dermoscopic Features of Acute, Subacute, Chronic and Intermittent Subtypes of Cutaneous Lupus Erythematosus in Caucasians.

Cutaneous lupus erythematosus (CLE) is divided into the following four clinical subtypes: acute CLE (ACLE), subacute (SCLE), chronic CLE (CCLE) and lupus erythematosus tumidus (LET). The aim of this study was to describe the dermoscopic patterns of CLE by clinical variant. A total of 54 Caucasian patients from Poland (ACLE = 10; SCLE = 11; CCLE = 26; LET = 7) were included. The predefined parameters for dermoscopic assessment in inflammatory dermatoses were analyzed separately by two dermatologists. Under dermoscopy, all the variants of CLE showed predominantly polymorphous vessels on a pink–red background within the lesional skin. Dotted vessels, in association with other vessel morphologies, were observed more frequently in SCLE than in the other subtypes of CLE, but the difference did not reach statistical significance (p = 0.07). The findings associated with hair follicles, including rosettes (p = 0.02), follicular plugs (p = 0.01), follicular red dots (p < 0.01), perifollicular white halos (p < 0.01) and dermoscopic features corresponding to scarring, including white (p = 0.01) and pink (p < 0.01) structureless areas, were significantly more common in CCLE than in other variants of CLE. A lack of scaling, pigmentation, erosions and crusting were observed in all the cases of LET. The role of dermoscopy as an auxiliary tool in the differential diagnosis of CLE needs further elucidation.

Autologous Fat Transfer in Lupus Panniculitis Facial Lipoatrophy.

Lupus panniculitis (LP) often presents with tender nodules and intermittent ulcers that then heal with scarring and lipoatrophy. The current mainstay of treatment is medical treatment. Research regarding the treatment of lipoatrophy from LP with autologous fat grafting is limited. We would like to share our experience in this rare case, which was treated with autologous fat transfer. A 48-year-old female presented with erythematous plaque, tender nodules, and ulcers following by a depression of the lesion at the left temporal area. The patient also had indurated erythematous plaque at her left cheek. Both lesions were aggravated by sunlight exposure. After several investigations, she was diagnosed as LP with secondary lipoatrophy and tumid lupus erythematosus at her left temporal and left cheek, respectively. She received antimalarial drug and topical steroids. The patient underwent two sessions of autologous fat transfer. She was satisfied with the volume and contour improvement in the scar following the injection of 8 and 3.7 mL of fat. Furthermore, the patient reported the remission of tender nodules and ulcers since the first fat graft injection. In conclusion, the autologous fat transfer is a simple and effective treatment for lipoatrophy and scar secondary to LP with promising results.

Lupus Erythematosus Tumidus Clinical Characteristics and Treatment: A Retrospective Review of 25 Patients.

Lupus erythematosus tumidus (LET) is a rare photosensitive der-matosis that was considered a subtype of chronic cutaneous lupus erythematosus (CLE); however, its clinical course and favorable prognosis led to its reclassification into another category called intermittent CLE. Although known for more than 100 years, LET's association with systemic lupus erythematosus (SLE), autoantibody profile, and disease prognosis is not well characterized. The purpose of this study was to describe the demographics, clinical characteristics, autoantibody profile, comorbidities, and treatment of LET.

Diagnosis of Lyme Borreliosis With a Novel, Seminested Real-Time Polymerase Chain Reaction Targeting the 5S-23S Intergenic Spacer Region: Clinical Features, Histopathology, and Immunophenotype in 44 Patients.

Lyme borreliosis (LB) is the most common tick-borne infection in Europe and North America. Polymerase chain reaction (PCR) is an important tool to confirm the diagnosis, but not always successful, especially when organisms are sparse. We developed a novel, seminested real-time PCR assay [target: 5S-23S intergenic spacer region (IGS)] and compared it with 3 well-established conventional PCR assays (IGS/OspA/real-time IGS) on 596 formalin-fixed, paraffin-embedded routine skin biopsies. The seminested real-time assay identified 46 cases of borreliosis while 25, 27, and 38 were identified by the 3 other assays, respectively (P 0.01, P 0.02, and P 0.42; significance P < 0.05). Clinicopathologic and immunophenotypic analysis of PCR-positive cases revealed 38 erythema migrans (EM), 6 Borrelia lymphocytomas, and 2 acrodermatitis chronica atrophicans (ACA). In the 44 PCR-confirmed cases, plasma cells were present in only a third of EM cases. By contrast, CD123-positive plasmacytoid dendritic cells were common (74%) and therefore are unlikely to be helpful in the differential diagnosis between EM and tumid lupus erythematosus. A loss of CD34 in a third of all LB specimens limits its diagnostic value in the differential diagnosis with morphea. Interstitial macrophages were common in cutaneous LB (42/43) forming interstitial granulomas in a third of all cases, and 3/38 EM, 3/6 Borrelia lymphocytomas, and 1/2 ACA were only identified by the new seminested real-time assay, suggesting that it is especially helpful in confirming the diagnosis of Borrelia lymphocytoma.

Lupus Erythematosus Tumidus: Clinical and Pathological Features in a Series of 20 Patients.

We sought to assess the clinicopathologic/immunophenotypical features in patients with lupus erythematosus tumidus (LET). All skin biopsies diagnosed as LET in 16 years were retrieved from our pathology laboratory. Clinical charts were reviewed and immunohistochemical markers were performed. Subpopulations of cells in the infiltrates were studied, as well as the number/distribution of plasmacytoid dendritic cells (PDC), which were compared with two control groups: normal skin and discoid lupus erythematosus. Direct immunofluorescence was performed when available. We found 20 patients (11 men and 9 women; mean age 43.5 years); the mean evolution previous to diagnosis was 20.3 months. They all had erythematous, nonscarring urticarial-like plaques: 8 in the head region only, 8 in trunk/limbs only; both segments were affected in 2 patients; topography was unknown in 2. Except for 1 patient, no one developed systemic lupus erythematosus. A dense perivascular/periadnexal superficial-to-deep lymphocytic infiltrate and stromal mucin deposition were characterized histopathologically; interphase changes were absent. Immunophenotype supported an inflammatory profile. The differential count of CD123 + PDC in 10/20 cases of LET (n = 1180) was notably higher than 5 cases of discoid lupus erythematosus (n = 419) and 5 cases of normal skin (n = 38). No immune deposits were found in 2 cases. LET is a rare, peculiar form of cutaneous lupus erythematosus that only exceptionally evolves to systemic lupus erythematosus . Its clinical-pathologic/immunophenotypical features are very characteristic. The amount of CD123 + PDC is a very helpful feature among the criteria for its diagnosis and seems to be relevant in its pathogenesis.

Diverse molecular signatures observed in patients with cutaneous lupus erythematosus

British Journal of DermatologyVolume 185, Issue 3 p. e147-e147 Plain Language Summary Diverse molecular signatures observed in patients with cutaneous lupus erythematosus First published: 02 September 2021 https://doi.org/10.1111/bjd.20612AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Abstract Linked Article: Zhu et al. Br J Dermatol 2021; 185:563–572. Cutaneous lupus erythematosus (CLE) is an autoimmune connective tissue disease affecting the skin. It has been estimated there are approximately 4·0 patients with the condition per 100,000 people. It affects more women than men and more African Americans than other ethnicities. CLE is a clinically diverse disease and can be divided into several subtypes including subacute CLE, discoid lupus erythematosus and lupus erythematosus tumidus. Although CLE can have different clinical presentations, patients largely receive the same initial treatments. This study was conducted in Dallas, Texas, USA and aimed to assess the molecular diversity found in patients with CLE. Blood samples were collected from a racially and ethnically diverse group of patients with CLE (n = 62) and gene expression was then studied using RNA sequencing. Analysis of data revealed that there were six unique subsets or groups of patients with CLE. The demographics and clinical features of these subsets were compared. It was found that two mostly non-African American and subacute CLE groups had inflammation and neutrophil signatures. Three clusters of predominantly African American patients and patients with discoid lupus erythematosus had increased expression of genes that related to T cells. This study shows that the molecular diversity of patients with CLE may reveal greater variation in CLE than previously recognized from just the clinical presentation of the disease. Future studies to better understand this molecular diversity in CLE can aid in the development of more targeted treatments for these different subsets of patients with CLE. Volume185, Issue3September 2021Pages e147-e147 RelatedInformation

S2k guideline: Diagnosis and management of cutaneous lupus erythematosus - Part1: Classification, diagnosis, prevention, activity scores.

S2k guideline: Diagnosis and management of cutaneous lupus erythematosus - Part1: Classification, diagnosis, prevention, activity scores.

AB023. An atypical clinical presentation of alopecia in two patients with systemic lupus erythematosus

Alopecia in varying patterns is a common feature of lupus erythematosus (LE). Several forms of alopecia that are not specific to LE can occur in the setting of lupus, such as alopecia areata, telogen effluvium, and anagen effluvium. LE-specific alopecias are defined as those with histology consistent with LE, and include forms of acute, subacute, and chronic cutaneous lupus erythematosus (CCLE). Common patterns of LE-specific alopecia include the non-scarring diffuse hair thinning and fragility of acute LE and the scarring, erythematous scaly plaques with follicular keratotic plugs, peripheral hyperpigmentation, and central hypopigmentation of discoid lupus erythematosus (DLE). Lupus erythematosus tumidus (LET) may also present on the scalp as well-defined, non-scarring alopecia without overlying scale, atrophy, and dyspigmentation. We report two male patients with large circular non-scarring alopecic plaques on the scalp without overlying scale or erythema, but with central hyperpigmentation and scarring – findings reminiscent of but also distinct from both DLE and LET. Punch biopsy from the hyperpigmented area of alopecia revealed peri-follicular lymphocytic infiltrate, follicular dropout, and increased dermal mucin consistent with LE; further workup and serologic testing revealed systemic lupus in both patients. These two cases demonstrate a unique clinical presentation of central scarring alopecia within a larger non-scarring alopecic plaque in the setting of SLE that deviates from typical lupus-related alopecia. Teaching point: these two cases underscore the morphologic heterogeneity of CCLE and need for thorough evaluation of systemic disease in patients presenting with both scarring and non-scarring alopecia.

AB006. A modular analysis approach to studying gene expression in cutaneous lupus erythematosus

Previous studies on blood transcription profiles of patients with cutaneous lupus (CLE) have primarily used a gene expression approach. The large amount of data involved in these studies can make data analysis and interpretation cumbersome. Consequently, a novel approach in gene expression analyses was devised that groups genes into transcriptional modules (i.e., apoptosis, protein synthesis, inflammation) to identify important themes and relevant genes. This approach was used to study gene expression in systemic lupus patients and subsequently identified a unique interferon signature. Thus, we aim to use the modular analysis approach to identify unique modules and genes of CLE patients in order to better understand their disease pathophysiology. We conducted RNA sequencing of whole transcriptomes of blood from 66 CLE patients and 10 human controls and subsequently performed modular analyses. Based on module scores, we performed an unsupervised cluster analysis to identify subgroups of CLE patients with distinct gene expression patterns. Statistical analyses comparing module scores of these clusters was performed using the Kruskal-Wallis tests with Bonferroni’s correction. Modules were associated with patient subgroups distinguished by demographic, clinical, and laboratory features such as CLE subtypes, autoantibody profiles (BioPlex® 2200 ANA Screen with MDSS), and cytokine profiles (Thermo Scientific ProCarta xMAP cytokine assay kits). The unsupervised cluster analysis identified eight distinct clusters of CLE patients and controls. A neutrophilic predominant signature governed by azurophilic granule genes (i.e., BPI, LTF, MPO) and neutrophil chemotaxis genes (CEACAM6, CEACAM8) was seen in two groups of patients mostly with subacute lupus erythematosus (SCLE) [n=7 (77.8% of total SCLE)] or lupus erythematosus tumidus (LET) [n=6 (60% of total LET)] and European-American descent [n=28 (53.6% of total European-Americans)]. Four groups consisting of a majority of discoid lupus erythematosus (DLE) patients [n=29 (60% of total DLE)] had a predominant T cell signature driven by genes important for antigen presentation and T cell proliferation and differentiation (i.e., SKAP1, ITK). Our data suggests that unique cell populations may help differentiate CLE subgroups, which can potentially affect their disease course and treatment selection. Further studies examining individual gene and protein levels are necessary to confirm our hypotheses.

A Rare Case of Tumid Lupus Erythematosus with Unilateral Linear Distribution in A Young Child

Tumid lupus erythematosus (TLE) as a rare variant of cutaneous lupus erythematosus (CLE) is characterized by edematous, urticarial-like annular papules and plaques. TLE has similar histopathologic findings to CLE such as periadnexal lymphocytic infiltration and interstitial mucin deposition. Although TLE develops on sun-exposed areas at any age, it is rarely distributed along the Blaschko lines and develops in infancy and childhood. Unlike CLE, skin lesion of TLE heals without leaving scarring or dyspigmentation. Here, we report a rare case of unilateral linear TLE in a 4-year-old girl, which was improved by intralesional corticosteroid injection and oral antimalarial drug with leaving postinflammatory hyperpigmentation.

18178 Clinical characteristics and treatment of lupus erythematosus tumidus

18178 Clinical characteristics and treatment of lupus erythematosus tumidus

Reticular erythematous mucinosis-Aspecial subtype of cutaneous lupus erythematosus?

Reticular erythematous mucinosis (REM syndrome) is arare skin disease that predominantly affects women. It is clinically characterized by flat, partly reticular, irregularly configurated pale erythema located in the region of the central chest and upper back. The skin alterations are usually asymptomatic or associated with slight pruritus or burning. Extracutaneous diseases and involvement of internal organs do not occur in REM syndrome. Histopathological features include perivascular and periadnexal lymphocytic inflammatory infiltrates as well as increased deposition of mucin in the dermis. Due to the similar histology to lupus erythematosus (LE) tumidus, it is discussed whether REM syndrome can be assigned to the spectrum of cutaneous LE or can be assessed as a lupus-like disease. Moreover, both conditions respond well to treatment with hydroxychloroquine.