Figure - available from: Clinical Reviews in Allergy & Immunology
This content is subject to copyright. Terms and conditions apply.
Discoid cutaneous lupus erythematosus. Scarring alopecia. Inflammatory areas with erythema, depigmentation, and follicular hyperkeratosis are visible
Source publication
Systemic lupus erythematosus is a complex autoimmune disease with a wide spectrum of clinical and immunopathological features. Cutaneous and articular manifestations are the most common signs in patients with systemic lupus erythematosus. We here review the pathogenesis and the new classification of cutaneous lupus erythemathosus with a discussion...
Similar publications
In this case report, we present a patient with systemic lupus erythematosus who has both refractory discoid lupus erythematosus lesions and refractory cytopenia under antimalarial, glucocorticoid and untarget immunosuppressant drugs. Rituximab, a monoclonal antibody directed against the CD20 antigen on the surface of B-lymphocytes has been used as...
Citations
... Though CLE may be part of the generalized autoimmune condition known as systemic lupus erythematosus, it frequently remains restricted to the skin. CLE may present with slightly different histopathologic lesions, which allows division into three types: acute, subacute, or chronic lupus erythematosus (CCLE), and this latter type accounts for 80% of the cases of CLE [2]. The majority of cases of CCLE are predominantly present on the scalp or face and when it is limited to this location, it is referred to as discoid lupus erythematosus (DLE) [3]. ...
There are many therapies to treat discoid lupus erythematous (DLE) which provide mild to moderate beneficial effect. However, none of these suggested therapies are ideal, and thus research continues exploring the immunological pathways responsible for DLE hopefully leading to the development of monoclonal antibody therapy to disrupt a key pathway leading to the pathological state. New brand-named drugs are usually associated with a hefty price and frequently there are significant side effects. Thus, ideally it would be very beneficial to re-purpose an inexpensive generic drug already on the market that is effective with little side effects. One such drug for DLE already exists, e.g., the anti-malarial hydroxychloroquine which frequently can be moderately successful. Another potential drug fulfilling these ideals may be a dopaminergic drug, e.g., dextroamphetamine sulfate, normally used for attention- deficit hyperactivity disorder which has been used to successfully treat other skin disorders e.g., urticaria, eczema, and bullous pemphigoid. The hypothesized mechanism involves releasing more dopamine from sympathetic nerve fibers. Dopamine diminishes cellular permeability, thus, theoretically inhibiting absorption of irritating elements into the dermis, which sets off an immune cascade. The case of DLE presented did not respond to standard therapy for DLE, but had a 100% remission with dextroamphetamine sulfate, which has lasted one and a half years so far.
... There are still no universal criteria for various subtypes of skin manifestation in SLE. 10 Skin findings are histopathologically and clinically classified into specific and non-specific, according to Gillam. 11 LE-specific lesions showed the characteristic of skin manifestation of SLE. ...
... It is a common finding in SLE patients and only occurs during the active phase of the disease. [10][11][12] The CLE spectrum is reported to differ between populations and races. 13 Unfortunately, only a few published reports show the spectrum of CLE in SLE patients from Indonesia. ...
Background
Cutaneous involvement is common in systemic lupus erythematosus (SLE) patients and may be essential to the disease activity. This study aimed to describe cutaneous manifestations spectrum and determine the association of cutaneous lesions with the disease activity and systemic involvement among SLE patients in Malang, Indonesia.
Methods
A cross-sectional study was conducted using 54 SLE patients from rheumatology outpatient clinic at Saiful Anwar General Hospital Malang, Indonesia. Cutaneous features were classified according to Gilliam and Sontheimer classification of cutaneous lupus. Disease activity and clinical manifestations were documented according to Mexican-SLE disease activity index (Mex-SLEDAI).
Results
Among 54 subjects, 50% of the patients had cutaneous manifestations. Subacute cutaneous lupus erythematosus (SCLE) was observed in 11.1% of patients, and malar rash in 20.4%. Subjects with cutaneous lesions had significantly higher Mex-SLEDAI scores, especially those who had SCLE (p<0.001), malar rash (p=0.002), alopecia (p=0.002), and photosensitivity (p=0.032). Six patients (11.1%) had skin infections with higher disease activity (9[8–11]vs.2[0–4];p<0.001). SCLE was significantly associated with malar rash (OR 11.7[1.8–76.5]), vasculitis (OR 43.0[4.1–445.6]), and fatigue (OR 15.0[2.1–108.8]). Malar rash was associated with photosensitivity (OR 8.4[1.6–44.0]), while oral or nasal ulcer was associated with fatigue (OR 8.6 [1.4–54.6]). Vasculitis (OR 5.9[1.0–35.1]) and nephritis (OR 11.7 [1.8–76.5]) were associated with the presence of skin infection.
Conclusion
SCLE and malar rash are the most common cutaneous lesions among subjects. Subjects with cutaneous lesions have relatively higher disease activity. Several skin lesions are also associated with SLE patients’ systemic manifestations.
... The classic histopathologic pattern in SCLE is epidermal thinning, vacuolization of the basal cell layer, scattered cytoid bodies, and a perivascular lymphocytic interface dermatitis confined to the upper dermis. Dyskeratotic keratinocytes extending into the upper spinous layers is another highly characteristic finding (15,16). ...
Immune checkpoint inhibitor (ICI) use has been associated with numerous autoimmune side effects, known as immune related adverse events (irAEs). Cutaneous irAEs are common and affect up to 50% of patients treated with ICIs. There have been an increasing number of cases reported in the literature regarding ICI-induced subacute cutaneous lupus erythematosus (SCLE). ICI-induced SCLE is important to recognize as it can result in a delayed and/or prolonged skin reaction despite treatment discontinuation. We describe a patient with gastro-esophageal adenocarcinoma who developed SCLE following one cycle of nivolumab treatment. A 75-year-old man presented to our clinic with a new photo-distributed rash composed of oval scaly pink papules and plaques involving his chest and arms. Despite treatment with topical corticosteroids, he presented to the emergency department 1 week later with worsening rash. Skin biopsy showed vacuolar interface pattern, along with superficial perivascular lymphocytic infiltrate, consistent with a drug eruption. The clinicopathological presentation was consistent with ICI-induced SCLE. Nivolumab treatment was discontinued due to the severity of the rash. The rash remitted with systemic corticosteroids, high potency topical steroids, and hydroxychloroquine. Unfortunately, the patient developed intraperitoneal metastatic disease, and was enrolled in hospice care. In this paper, we highlight the importance of early identification and treatment of this irAE. A review of the literature, including a discussion on the management of ICI-induced SCLE is also provided.
... Discoid lupus erythematosus (DLE), a subtype of chronic cutaneous lupus, confers significant morbidity [1][2][3]. Clinical presentation includes scarring alopecia, marked dyspigmentation of plaques, erythematous papules with scaling and telangiectasia, and facial lesions, especially in photo-exposed areas [2,3]. Discoid lupus erythematosus with linear configurations that follow the lines of Blaschko are atypical and rare. ...
... Discoid lupus erythematosus (DLE), a subtype of chronic cutaneous lupus, confers significant morbidity [1][2][3]. Clinical presentation includes scarring alopecia, marked dyspigmentation of plaques, erythematous papules with scaling and telangiectasia, and facial lesions, especially in photo-exposed areas [2,3]. Discoid lupus erythematosus with linear configurations that follow the lines of Blaschko are atypical and rare. ...
... Discoid lupus erythematosus with linear configurations that follow the lines of Blaschko are atypical and rare. However, reports of DLE mimicking en coup de sabre morphea do exist [3]. In addition, there are associated rare immunodeficiencies linked to DLE such as the chronic granulomatous disease (CGD) state. ...
... SLE is a severe inflammatory disease characterized by diverse clinical and immunopathological manifestations (80,81). Dysregulated activation of immune cells and aberrant secretion of autoantibodies and proinflammatory cytokines participate in the pathogenesis of SLE (82, 83). ...
The skin is exposed to environmental challenges and contains heterogeneous cell populations such as epithelial cells, stromal cells, and skin-resident immune cells. As the most abundant type of stromal cells, fibroblasts have been historically considered silent observers in the immune responses of the cutaneous epithelial immune microenvironment (EIME), with little research conducted on their heterogeneity and immune-related functions. Single-cell RNA sequencing (scRNA-seq) and spatial transcriptomics (ST) have overcome the limitations of bulk RNA sequencing and help recognize the functional and spatial heterogeneity of fibroblasts, as well as their crosstalk with other types of cells in the cutaneous EIME. Recently, emerging single-cell sequencing data have demonstrated that fibroblasts notably participate in the immune responses of the EIME and impact the initiation and progression of inflammatory skin diseases. Here, we summarize the latest advances in the role of fibroblasts in the cutaneous EIME of inflammatory skin diseases and discuss the distinct functions and molecular mechanisms of activated fibroblasts in fibrotic skin diseases and non-fibrotic inflammatory skin diseases. This review help unveil the multiple roles of fibroblasts in the cutaneous EIME and offer new promising therapeutic strategies for the management of inflammatory skin diseases by targeting fibroblasts or the fibroblast-centered EIME.
... Nem jogtalanul hívják a lupust és luest is nagy imitátoroknak, hiszen mind a két betegség igen széleskörű és heterogén tünetekkel járhat együtt. A különböző szervrendszereket érintő tünetek hasonlósága mellett a diagnosztika során elvégzett vizsgálatokban is találunk átfedéseket (1,2). Esetünkkel szeretnénk rávilágítani a szifilisz és szisztémás lupus erythematosus differenciáldiagnosztikai kihívásaira, valamint segítséget nyújtani elkülönítésükben. ...
The authors report the case of a 29-year-old male patient presenting with erythematous maculopapular rash, painful oral and genital erosions, dysuria, scleroconjunctivitis, painful palmoplantar hyperkeratotic papules, asymmetric, migratory oligoarthritis, recurrent fever and alopecia parvimaculata. Based on the clinical symptoms the first suspected diagnoses were secondary syphilis and reactive arthritis, however the performed laboratory tests did not confirmed the diagnoses. The autoimmune serology tests, the clinical symptoms, and the results of further investigations established the diagnosis of systemic lupus erythematosus (SLE). By presenting this case the authors highlight the differential diagnostic challanges of secondary syphilis and SLE.
... Regarding the diagnosis of the two conditions, Table 1 presents the main investigations used, together with the criteria that must be met to certify their positivity. [17][18][19][20][21][22][23]. ...
Celiac disease (CD) and systemic lupus erythematosus (SLE) are two diseases intensively studied in all age groups, with an increasing incidence at the global level, possibly due to the increased awareness of the diseases and their accurate diagnosis and as a consequence of the new research and innovation technologies that have appeared in medicine. The first is a controllable condition found in approximately 1% of the entire population in the form of a reaction to environmental stimuli affecting individuals with genetic susceptibility, causing gluten intolerance, gastrointestinal and extradigestive symptoms, starting from subclinical stages and culminating in severe malabsorption. On the other hand, lupus is an autoimmune disease with chameleon-like symptoms and found mainly in the female sex, which leaves its clinical mark on most organs, from the skin, eyes, and kidneys to the cardiovascular, pulmonary, neurological, osteoarticular, and hematological systems. Current studies focus on the correlation between celiac disease and other autoimmune pathologies such as autoimmune thyroiditis (Hashimoto and Graves-Basedow), type I diabetes, and systemic lupus erythematosus. The current review aims to present a summary of the data from the specialized literature regarding the intercurrents between celiac disease and lupus by analyzing the most recent studies published on PubMed.
... El área afectada en la mayoría de los casos es el rostro, seguido del cuello, el tronco y las extremidades. Por otra parte, cuando hay compromiso del cuero cabelludo, puede causar alopecia cicatricial, que es irreversible según el tiempo de evolución (4,8) . El diagnóstico diferencial debe establecerse con enfermedades que sigan la distribución en las líneas de Blaschko, como el liquen plano lineal, liquen estriado, granuloma anular lineal, psoriasis lineal, morfea o nevo epidérmico verrugoso lineal inflamatorio (3) . ...
El lupus eritematoso discoide lineal es una enfermedad crónica de etiología desconocida. Se caracteriza por placas eritematosas que siguen las líneas de Blaschko y constituye una variante inusual del lupus eritematoso discoide. En la presente revisión, se describe el caso de una paciente femenina que consultó por lesiones en la cara, área alopécica en el cuero cabelludo asociado a manifestaciones de lupus eritematoso sistémico, quien obtuvo una adecuada respuesta al tratamiento farmacológico. La búsqueda de los artículos científicos para el desarrollo de este reporte se realizó en las siguientes bases de datos: ClinicalKey, Ovid y PubMed. En la actualidad, es el primer reporte de caso clínico descrito de lupus eritematoso discoide lineal asociado a lupus eritematoso sistémico; se realizará una revisión de la literatura.
... It determines our contact with the environment and is an important protection against external pathogens. Up to 80% of patients with SLE present symptoms of the disease expressed as skin lesions, and in 25% of them skin lesions are the first manifestation of the disease [58]. Zhou et al. revealed that the skin microflora of SLE patients presents significant differences when compared to healthy controls. ...
... Interestingly, a comparison between remission and active SLE groups revealed that the family Caulobacteraceae was positively correlated with SLE disease activity index (SLEDAI) and negatively with complement C3. Additionally, Aerococcaceae was negatively correlated with SLEDAI and immunoglobulin G [58]. These observations provided some suggestive evidence for further exploration of skin microbiota in SLE patients. ...
Lupus nephritis (LN) is one of the most common and serious complications of systemic lupus erythematosus (SLE). The risk factors for developing LN by SLE patients are not fully understood. They are considered to be a mix of genetic and environmental variables, one of them being dysbiosis, proposed recently to interfere with autoimmunity. As of yet, the relations between the human microbiome, its genetic determinants, individual variability and clinical consequences remain to be established. One of the major obstacles in studying them is the magnitude of confounders, such as diet, drugs, infections or antibiotics use. They also make comparison between the studies extremely complicated. We reviewed the available evidence for the interplay between microbiome, dysbiosis and mechanisms triggering the autoimmune responses and potentially contributing to LN development. One such mechanism is the stimulation of autoimmune responses by bacterial metabolites that can mimic autoantigens and cause antibody production. These mimicking microbial antigens seem to be a promising target for future interventions.
... The management of dermatologic findings in SLE typically involves avoiding sun exposure, using a broadspectrum sunscreen, and wearing protective clothing. Topical and systemic medications, such as corticosteroids and antimalarials, may also be used to treat skin symptoms [5]. Close monitoring and management of SLE by a healthcare professional are essential to prevent complications and improve quality of life. ...
Systemic lupus erythematosus (SLE) is an autoimmune disease that has a wide range of manifestations and can affect nearly every organ system. Skin manifestations are a common finding in SLE. They are often photosensitive and can be exacerbated by exposure to ultraviolet light. Here, we discuss the case of a 34-year-old African American woman who presented with periorbital edema while 12 weeks pregnant. This case highlights the importance of avoiding sun exposure in patients with SLE and the challenge of treating SLE during pregnancy.
