Chronic Cutaneous Lupus Erythematosus Research Articles

Chronic Cutaneous Lupus Erythematosus in a Patient with a History of Kikuchi–Fujimoto Disease

Chronic Cutaneous Lupus Erythematosus in a Patient with a History of Kikuchi–Fujimoto Disease

Immunohistochemical Expression of Immune Regulatory Proteins in Interface Dermatoses.

Cutaneous immune-related adverse events (irAEs) of immunotherapies, such as anti-programmed cell death protein-1 (PD-1), suggest that immune checkpoint factors may contribute to the pathobiology of lichenoid interface dermatitis in immunotherapy-naïve patients. Our study aimed to describe innate and adaptive immune markers via immunohistochemical (IHC) staining of lichenoid interface dermatoses. We studied the staining patterns of PD-L1, STING, IL-36 gamma, CD8, PD-1, and LAG-3 in five interface dermatoses: oral lichen planus (LP) (n = 10), cutaneous LP (n = 10), chronic cutaneous lupus erythematosus (CLE) (n = 11), erythema multiforme (EM) (n = 11), and toxic epidermal necrolysis (TEN) (n = 13), by immunohistochemistry (IHC) analysis. Expression was evaluated semi-quantitively according to the percentage of keratinocytes and dermal lymphocytes stained compared to keratinocytes and resident pericapillary lymphocytes in normal human skin. All interface dermatoses evaluated showed increased expression of PD-L1 on keratinocytes and LAG-3 in lymphocytes. STING was increased on the keratinocytes of most specimens. Expression of IL-36 gamma, in basal layer keratinocytes was more extensive in oral LP and cutaneous LP and varied in CLE, EM, and TEN. Lymphocytic infiltration expressing PD-1 was elevated in oral LP, cutaneous LP, and CLE. Current thinking is that interface dermatitis is the result of a cell-mediated immune reaction involving cytotoxic CD8+ T-cell-mediated apoptosis of keratinocytes. The findings of this study suggest that in addition to cell-mediated immunity, innate immune factors may contribute to pathobiology.

Effect of iberdomide on cutaneous manifestations in systemic lupus erythematosus: a randomized phase 2 clinical trial

Iberdomide, a cereblon modulator, promotes degradation of transcription factors Ikaros and Aiolos. Evaluate iberdomide efficacy and safety in cutaneous lupus erythematosus (CLE) in a phase 2 study. Patients were randomized (2:2:1:2) to iberdomide 0.45 (n=81), 0.30 (n=82), or 0.15 mg (n=42) or placebo (n=83) daily while continuing background lupus medications. The mean (SD) baseline Cutaneous Lupus Area and Severity Index Activity (CLASI-A) score was 6.9 (7.0); 28% of patients had a score ≥8; 56% had acute CLE, 29% chronic CLE, and 16% subacute CLE. Mean CLASI-A improvement in patients with baseline score ≥8 was 39.7% for iberdomide 0.45 mg versus 20.1% for placebo at week 4 (P=0.032), with continued improvement through week 24 (66.7% vs 54.2%; P=0.295). Proportions of patients achieving ≥50% CLASI-A reduction from baseline at week 24 were significantly greater for iberdomide 0.45 mg versus placebo for patients with subacute (91.7% vs 52.9%, P=0.035) and chronic (62.1% vs 27.8%; P=0.029) CLE but not for the overall population (55.6% vs 44.6%) or patients with baseline CLASI-A ≥8 (66.7% vs 50.0%). Small patient subgroups of CLE subtypes. Iberdomide showed beneficial effects when added to background lupus medications in patients with subacute and chronic CLE.

Successful Treatment of Refractory Discoid Lupus Erythematosus with Deucravacitinib

Discoid lupus erythematosus (DLE) is the most common subtype of chronic cutaneous lupus erythematosus (CLE) that may present with or without systemic lupus erythematosus (SLE). Treatment for DLE remains limited, as there are no medications specifically approved to treat DLE. Deucravacitinib, an oral, selective, allosteric tyrosine kinase 2 (TYK2) inhibitor, is currently being studied for adults with moderate-to-severe discoid and/or subacute CLE. We report a case of successful treatment of refractory DLE with deucravacitinib. A 65-year-old female with a longstanding history of systemic lupus erythematosus presented with prominent discoid lesions involving the nose, cheeks, and mid upper cutaneous lip. Having tried topical medications, multiple courses of systemic steroids were attempted, but the patient flared shortly after the tapers. Despite the patient experiencing some improvement on mycophenolate mofetil and prednisone, the decision to switch to deucravacitinib was made due to the patient developing multiple infections while on the former regimen. Three months after starting deucravacitinib, the patient returned with their skin completely clear. A better understanding of the molecular pathogenesis of CLE is informing the development of more targeted therapeutic strategies. TYK2 and Janus Kinases (JAKs) both mediate the signaling of cytokines, some of which play a role in the pathogenesis of SLE. Deucravacitinib binds to TYK2 at the regulatory domain, which is unique to its respective kinase unlike conventional Janus Kinase (JAK) inhibitors that bind to the active domain conserved across all JAKs. Deucravacitinib offers more targeted inhibition with a potentially improved safety profile compared to conventional JAK inhibitors.

Cutaneous spectrum of lupus erythematosus: A cohort of patients from a referral-center in Colombia

IntroductionTo date, few epidemiological studies compare the incidence and prevalence of systemic lupus erythematosus (SLE) and isolated cutaneous lupus erythematosus (CLE). The information is even more scarce in Latin America. The aim of this study is to describe the phenotype of CLE in a reference center in Colombia. Materials and methodsA retrospective cross-sectional study was carried out. SLE was confirmed according to EULAR/ACR 2019 classification criteria and patients were simultaneously evaluated in the rheumatology and dermatology clinics. A descriptive and bivariate analysis was carried out. The institutional committee approved the study. ResultsThirty participants were diagnosed with SLE and CLE. Most patients were female (83.3%), with a mean age of 37±13 years. Chronic CLE was the most prevalent subtype (46.7%), followed by acute (30%) and subacute (16.7%) CLE. There were statistically significant differences when comparing acute CLE and reduced C4 (p=.032); in subacute CLE and interstitial lung disease (p=.010); and in lymphopenia (p=.012) and thrombocytopenia (p=.046). Finally, there was a difference in patients with chronic CLE and the use of topical corticosteroid (p=.026), methotrexate (p=.036), and SLEDAI>3 points (p=.025). ConclusionThis study provides valuable insights into the phenotypic characteristics and associations of CLE with systemic manifestations in the Colombian population, contributing to the understanding and managing of this complex autoimmune disease.

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.

Chronic ulcerative paronychia: a possible further clinical manifestation of mucocutaneous lupus erythematosus in four dogs

In this study, the signalment, clinical signs, histopathological findings and treatment outcome of four dogs with chronic ulcerative paronychia, that presented clinical and microscopic characteristics of the mucocutaneous variant of canine chronic cutaneous lupus erythematosus are described. The Greyhounds in the case series had a symmetric claw fold involvement, either exclusively or as major clinical presentation.

Skin Changes in Lupus Erythematosus: A Concise Review Lupus Erythematosus and Skin-demystifying the Features

Lupus erythematosus is an autoimmune disease characterised by the formation of various autoantibodies, leading to the involvement of numerous organ systems like the nervous system, renal system, and cutaneous involvement. Cutaneous involvement is the second most common manifestation after musculoskeletal involvement and can range from minor involvement to severe disabling sequelae. The type of skin manifestation can sometimes predict the underlying systemic involvement, as most patients of acute lupus erythematosus have concomitant systemic involvement in contrast to limited chronic cutaneous LE, which has a low incidence of systemic involvement. The cutaneous manifestations have been included in the Systemic Lupus International Collaborating Clinics (SLICC) and American College of Rheumatology criteria for ease in diagnosing lupus erythematosus patients. Herein, we present a review of various cutaneous manifestations seen in association with systemic lupus erythematosus (SLE) patients and their specific management, which can ease the early diagnosis of the patients and triage the patients that will need close follow-up for systemic involvement.

The realistic positioning of UVA1 phototherapy after 25 years of clinical experience and the availability of new biologics and small molecules: a retrospective clinical study

BackgroundSince the early 1990s, Ultraviolet (UV) A1 phototherapy has been described as an effective and safe treatment of a multitude of skin disorders. However, after 30 years, its use has remained limited to few dermatological centers.ObjectiveTo analyze the changes over the years and the current position of UVA1 phototherapy through a Real-World Evidence (RWE) study at a single tertiary referral center.MethodsWe reviewed the medical files of 740 patients treated between 1998 and 2022. Treatment results were collected, efficacy was assessed by a grading scale and acute adverse effects were registered.ResultsWe treated patients with 26 different diseases. We registered marked improvement (MI) or complete remission (CR) in 42.8% of patients with morphea, 50% with Urticaria Pigmentosa, 40.7% with Granuloma annulare and 85.7% with skin sarcoidosis. Good results were obtained also in the treatment of chronic Graft Versus Host Disease (GVHD), Eosinophilic Fasciitis, Sclero-atrophic Lichen, skin manifestations of systemic lupus erythematosus and psoriasis of HIV+ patients. Systemic Sclerosis, Romberg’s Syndrome, Bushke’s Scleredema, Nephrogenic Fibrosing Dermopathy, REM Syndrome, Follicular Mucinosis, Pretibial Myxedema, Scleromyxedema, pemphigus foliaceus, chronic cutaneous lupus erythematosus, erythroderma of Netherton Syndrome and Necrobiosis Lipoidica were no or poorly responsive. In clinical indications where UVA1 was used as a second line phototherapy after narrow-band (NB)-UVB, we saw good MI or CR rates in Mycosis Fungoides (57% of patients), Atopic Dermatitis (33.9%), Pitiryasis Lichenoides chronica (50%), Pityriasis Lichenoides et varioliformis acute (75%) and Lymphomatod Papulosis (62.5%). Short-term adverse events were uncommon and mild.ConclusionOver the past decade, the annual number of treated patients has progressively declined for several reasons. Firstly, UVA1 phototherapy has taken a backseat to the cheaper and more practical NB-UVB phototherapy, which has proven effective for common indications. Secondly, the emergence of new, safe, and effective drugs for conditions such as atopic dermatitis, GVHD, and connective tissue disorders. Finally, our research has shown that UVA1 therapy is often ineffective or minimally effective for some rare diseases, contrary to previous case reports and small case series. Nonetheless, UVA1 continues to be a valuable treatment option for patients with specific skin disorders.

Treatment of Patient with Cutaneous Lupus Erythematosus with Roflumilast Cream 0.3%

Introduction: Cutaneous lupus erythematosus (CLE) is the cutaneous form of a common autoimmune disease, lupus erythematosus, driven by a dysfunction within the adaptive and innate immune system. CLE is subcategorized further into acute, subacute and chronic disease. Here we report a case of a patient with systemic and refractory cutaneous disease with improvement in manifestations demonstrating efficacy following treatment with roflumilast cream 0.3% in a complex chronic CLE patient with uncontrolled disease, intense pruritus and scarring. Case Report: A 48-year-old Asian female with history of smoking, chronic refractory SLE since 2008 with a new onset of pruritic rash. Previous therapies including thalidomide, hydroxychloroquine, methotrexate, mycophenolate mofetil, numerous research trials, oral and intramuscular steroids that failed to control her systemic LE. A Rheumatology colleague referred the patient for new onset cutaneous manifestations. Initial presentation revealed lesions involving 2% of the occipital scalp. Pathology confirmed the diagnosis of discoid lupus. Intralesional triamcinolone , plus topical betamethasone dipropionate 0.05% twice daily (BID) and timolol 0.5% OP solution for wound healing were initiated. Fifteen months later the patient had disease progression of her CLE on to her scalp, face, legs and arms, with the chief complaint of persistent pruritus despite oral prednisone, hydroxychloroquine and thalidomide. The patient’s rheumatologist requested alternative treatment for cutaneous disease without use of long term topical high potency steroids. A second series of intralesional triamcinolone injections plus betamethasone dipropionate 0.05% BID for 3-4 weeks and titrate down to 2-3 applications per week, and addition of roflumilast cream 0.3% was initiated once daily to her face, scalp, and lower legs. On the third office visit, four weeks later, the patient reported pruritus and cutaneous manifestations in lower legs controlled and scalp and face have marked improvement. After 8 weeks on this regimen, the patient return reported that scalp and face continued to improve and disease on the legs continue to be controlled despite not using topical treatment for four weeks. Roflumilast cream 0.3% once daily was continued as monotherapy. Two months later, the patient reported that her skin was fully controlled was starting to repigment with monotherapy. Conclusions: This case report of a 48-year-old female with refractory treatment resistant chronic SLE and CLE treated with roflumilast cream 0.3%, a phosphodiesterase-4 (PDE-4) inhibitor, resulted in disease control, the symptom improvement of improved pruritus as well as repigmentation of the skin. The results observed in this patient suggest this topical intervention, if implemented at the beginning of treatment for cutaneous onset of disease, may have the potential to halt the progression of cutaneous involvement. This case report serves to highlight a promising intervention that may lead to better cutaneous control without long term side effects of topical steroid use.

Diffuse skin lesions in a patient with systemic lupus erythematosus: A case report

Systemic lupus erythematous (SLE) is a persistent autoimmune disease with systemic and life- threatening manifestations, in which cutaneous disease is the second most common manifestation, after joint inflammation. Skin lesions in SLE is originally divided into two groups: LE-specific lesions and LE-nonspecific lesions. The term “LE-specific lesions” includes 3 distinct categorizes: Acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE) and chronic cutaneous lupus erythematosus (CCLE) [4]. By another method of classification, specific skin lesions can also be classified as localized or generalized lesions. The most common localized lesion is malar, or butterfly rash, which is reported in 20-60% of patients with SLE. In comparision to localized lesions, generalized lesions, which are indiscriminately referred to as lupus maculopapular rash, are relatively rare. This form of lesion is characterized by well-demarcated erythematous macules and/or papules located on the extensor side of limbs, sparing the knuckles. In this article, we report a case of a female patient having generalized cutaneous lesions. She was initially diagnosed with SLE and received treatment at Department of Allergy, Center of Dermato-Venerology and Allergy, 108 Military Central Hospital.

Lupus mastitis and antiphospholipid syndrome treated with anticoagulation and immunosuppression: a case report

BackgroundSystemic lupus erythematosus is an autoimmune disease that can have cutaneous and systemic manifestations. Lupus panniculitis, also known as lupus mastitis, is a subset of chronic cutaneous lupus erythematosus that involves inflammation of the subcutaneous fat. The pathogenesis of lupus mastitis is not fully understood. Diagnosis involves a combination of skin manifestations, imaging, and pathologic confirmation. Treatment typically includes steroids and antimalarials, with more severe disease requiring additional immunosuppressive medications. This report highlights a case of lupus mastitis treated with rituximab and a possible relationship between this disease process and thrombotic disease.Case presentationA 48-year-old African American female with systemic lupus erythematosus and antiphospholipid syndrome presented with new breast lesion. Mammography revealed calcifications and increased density with coarse trabecular pattern. Breast biopsy showed features of cutaneous lupus and occlusive vasculopathy. The patient was diagnosed with lupus mastitis and treated with anticoagulation, rituximab, mycophenolate mofetil, and quinacrine with resolution of her symptoms.ConclusionThis patient experienced improvement in her breast symptoms with combination therapy including rituximab. There are only two other cases reported in literature of patients with lupus mastitis responding to rituximab, highlighting the possible role of B cell depleting therapy for those who have contraindications to standard treatments for lupus mastitis. While the pathophysiology of lupus mastitis is thought to be immune driven, some literature suggests that associated thrombosis commonly seen may be due to a physiologic overlap similar to antiphospholipid syndrome. The possible relationship between antiphospholipid syndrome and lupus mastitis and the use of antiplatelet and anticoagulation therapy is discussed and may warrant further investigation.

A Rare Case of Butterfly Target Rash

A 25-year-old female was admitted under Internal medicine for fever and anemia. The patient had a history of fever, joint pain, photosensitivity, and rash over the malar area of the face. On examination, the patient had targetoid lesions over the malar area of the face, ulcers in the oral cavity, and chilblain lesions. The presence of a targetoid lesion in association with features suggestive of systemic lupus erythematosus (SLE) raised suspicion of Rowell syndrome (RS). Further evaluation revealed pancytopenia, proteinuria, positive antinuclear antibodies, and RA factor, confirming our suspicion. RS is chronic cutaneous LE, wherein erythema multiforme occurs in the setting of LE. Heretofore, only 18 cases of RS have been reported from India. Our patient had targetoid lesions limited to the malar area of the face, which is not the characteristic malar rash of SLE. The patient also had a rare finding of chilblain lesions. This distinctive array of clinical and immunological features diagnostic of RS prompted us to present the case.

CPC05 A case of extensive hypertrophic lupus erythematosus responsive to topical steroids

Abstract Hypertrophic lupus erythematosus (HLE) is a rare variant of lupus erythematosus, occurring in 2% of cases of chronic cutaneous lupus erythematosus (CCLE). It is an exaggerated proliferative epithelial response manifesting as verrucous-appearing hypertrophic indurated lesions occurring on sun-exposed sites. We present a case of the development of extensive HLE in a patient with subacute cutaneous lupus erythematosus (SCLE) with underlying pulmonary malignancy. A 74-year-old woman with Sjögren syndrome presented to dermatology with a 2-month history of a rash affecting her face and décolletage. Laboratory studies illustrated elevated antinuclear and anti-Ro antibodies. Biopsy from her chest confirmed SCLE. Malignancy screening revealed T1aN0 pulmonary lepidic adenocarcinoma, which was successfully managed with a lower lobectomy. Hydroxychloroquine was initially trialled but required discontinuation following intolerable side-effects. Mepacrine was then initiated, which allowed clearance of the rash, alongside yellow discoloration of the skin. Six months later, she developed a raised pruritic rash on her arms, legs and buttocks, resistant to current management alongside a trial of mycophenolate mofetil. Examination revealed violaceous hypertrophic plaques affecting 40% of her body-­surface area. The differential included hypertrophic lichen planus, nodular prurigo and HLE. Repeat lesional biopsies highlighted a lichenoid inflammatory infiltrate with overlying scale, favouring HLE. Owing to patient choice, topical mometasone furoate 0.1% was used to manage cutaneous disease, with a reduction in lesion size. HLE is a distinct subset of CCLE, regarded as a separate entity to SCLE. Twenty per cent of patients with SCLE also exhibit other types of cutaneous lupus erythematosus (CLE) lesions. No cases in the literature describe progression from SCLE to CCLE, or HLE, in particular. Hallmarks of HLE include verrucous lesions, chronicity and treatment resistance. Early recognition and follow-up are essential as malignancy may arise in long-standing lesions. Lung adenocarcinomas and hypertrophic lupus are both associated with p53 mutations and decreased granzyme B expression. Mutated p53 results in constitutive cell proliferation and DNA damage can accumulate in cells. Granzyme B is a protease in cytotoxic T cells involved in immune-mediated cell lysis. Both processes result in the potential to produce exaggerated hyperproliferative lesions, such as those observed in HLE and the lepidic adenocarcinomas. HLE is a rare clinical entity that can be easily misdiagnosed clinically and histopathologically. Increased awareness of this condition is essential to highlight the variety of clinical presentations of uncommon variants of CLE, to ensure they are considered while approaching clinical diagnosis.

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.

Changing Trends of Cutaneous Lupus Erythematosus (CLE) in a Tertiary Care Hospital in Northern Odisha, India.

Cutaneous lupus erythematosus (CLE) is a chronic autoimmune inflammatory skin disorder. Several studies have been published regarding its prevalence, demographic details, clinical spectrum, and various associated factors. In our out patient department (OPD), we noticed an increase in the number of cases of CLE in our area in the last few years. Therefore, the current cross-sectional study was conducted to assess the trends of CLE among patients who reported to a tertiary care hospital. The current study is a record-based cross-sectional study of 81 patients of CLE, who attended the dermatology OPD of a tertiary care hospital. Data were collected from 2017 to 2022 and were divided into three different periods of time (2017-2018, 2019-2020, and 2021-2022). Demographic details, clinical examination findings, and laboratory investigation reports were also collected. There was a rising trend in the cases of CLE. Females outnumbered males (2:1, 66.67%). The increase in cases from 2017-2018 to 2019-2020 was 157% and from 2019-2020 to 2021-2022 was 204%. In 2021-2022, 52% of cases of chronic CLE (CCLE) were males. Photosensitivity was the most common finding. The majority of patients were addicted to smoking. The current study noticed an increasing trend in all types of CLE. So, this rising trend should be investigated for possible triggering factors like climatic changes,infections, and drug factors with a larger sample size.

Clinical characteristics of palmoplantar discoid lupus erythematosus: A multicenter retrospective review of 43 patients

AbstractBackgroundPalmoplantar discoid lupus erythematosus (ppDLE) is a rare manifestation of chronic cutaneous lupus erythematosus that can result in painful, hyperkeratotic, and/or erosive lesions on the palms and soles. Despite this penchant to cause significant morbidity, the natural history of ppDLE and its response to conventional treatment approaches remain poorly understood.ObjectivesTo characterize the initial presentation, treatment response, and association with systemic lupus erythematosus (SLE) of ppDLE.MethodsWe performed an International Classification of Diseases (ICD‐9 and ICD‐10) code and natural‐language query for medical records from Brigham and Women's Hospital and Massachusetts General Hospital to identify all cases of ppDLE based on biopsy and/or expert opinion from January 2000 through October 2022. Demographics, clinical features, and treatment data were analysed.ResultsForty‐three patients (median age, 29.5 years [range, 8−70 years]; 36 female [83.7%]) with ppDLE were identified. Mean time from initial clinical presentation of ppDLE symptoms to diagnosis was 14.5 months (n = 34). Of the 43 patients, 74.4% developed coexistent SLE. A mean of 5.9 (standard deviation, 2.7) lines of therapy were required to achieve control of ppDLE, with the majority of patients requiring the initiation of at least two concomitant systemic medications.ConclusionsHerein, we present the largest cohort of patients with ppDLE in the literature to date. Despite a high incidence of coexistent SLE, patients experienced lengthy diagnostic delays. Even with appropriate diagnosis, an overwhelming trend towards treatment‐refractory disease was observed.

Comedonic lupus, a rare variant of chronic cutaneous lupus erythematosus (CCLE): case series and literature review.

Cutaneous lupus erythematosus (CLE) is an autoimmune disease with several clinical presentations. The chronic form predominantly presents as discoid rashes but may present with less common morphological findings that can sometimes make diagnosis difficult. Comedonic lupus is a rare and underdiagnosed variant, with unknown etiology and still poorly defined treatment. The report illustrates a series of five cases of patients diagnosed with comedonic lupus, and it reviews 18 cases previously published in the literature. The clinical presentation is of comedonal lesions, mostly located on the face, making a differential diagnosis with other benign conditions such as acne vulgaris, Favre-Racouchot syndrome, and syringoma, emphasizing the importance of clinical practice and histopathology for diagnostic confirmation. There is scarcity in the literature regarding the condition and therapeutic possibilities for these cases of comedonic lupus.

Skin involvement in systemic lupus erythematosus: a review article

Cutaneous disease is one of the most frequent manifestations of systemic lupus erythematosus (SLE), being classified as LE-specific and LE-nonspecific. LE-specific skin lesions are divided into acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE) and chronic cutaneous lupus erythematosus (CCLE). The association with systemic involvement varies between each clinical subtype, with non-specific lesions being more frequent associated with active SLE than cutaneous specific lesions. The treatment consists of topical agents (glucocorticoids, topical calcineurin inhibitors) as well as systemic therapies (glucocorticoids, hydroxychloroquine, quinacrine, methotrexate, retinoids, dapsone, mycophenolate mofetil or even biologics). In the presence of strictly cutaneous involvement, periodic patient follow-up and monitoring for the progression to systemic disease remains an important mission for the dermatologist and the rheumatologist.

Panoramic view of clinical features of lupus erythematosus: a cross-sectional multicentre study from China

ObjectiveLupus erythematosus (LE) is a complicated disease with highly heterogeneous clinical manifestations. Previous studies have rarely included all subgroups of patients with lupus and have overlooked the importance of the...