scholarly journals Cutaneous manifestations of systemic lupus erythematosus: experience from a tertiary care hospital of Bangladesh

2021 ◽  
Vol 12 (1) ◽  
pp. 57-61
Author(s):  
Mohammad Asifuzzaman ◽  
Rowsan Ara ◽  
Tonmoy Biswas ◽  
Rashedul Hassan ◽  
Minhaj Rahim Choudhury

Background: Systemic lupus erythematosus (SLE) is a chronic, multisystem disorder that can affect any organ of the body. Approximately 80 percent of patients develop skin disease at some point in their disease course. The association with SLE varies among the subtypes of cutaneous lupus erythematosus (LE). Better understanding of cutaneous manifestations can help in more effective management. This study aimed to evaluate the pattern of cutaneous manifestations of SLE and to find out association with organ involvement. Methods: This cross-sectional observational study was conducted in the Green Life Medical College Hospital from January 2019 to December 2020. Sixty four lupus patients who fulfilled the SLICC 2012 classification criteria and having new onset or preexisting skin complaints were enrolled. Mixed connective tissue disease and other overlap syndromes were excluded. All patients were evaluated by a dermatologist and diagnosis was done as per modified Gilliam Classification criteria. Results: Out of 64 patients, 56 were female and 8 were male. Female and male ratio was 7:1. Mean age was 28.4±9.6 years. Among the cutaneous manifestations, LE specific was 38 (59.4%), LE non-specific was 41 (64.1%). Among LE specific, 66% were acute (ACLE), 42% were sub-acute (SCLE) and 37% patients were chronic (CCLE). Among ACLE, 72% had malar rash and 84% had photosensitivity. Among SCLE, most common was papulosqumous (68%). DLE (86%) was the most common CCLE. Among LE non-specific, 85% had non-scarring alopecia, 52% had vascular abnormalities. Most common organ involvement was musculoskeletal (84%), then renal (56%). DLE had negative association with renal involvement [OR (-0.04)]. No other cutaneous manifestations showed any significant association with any other organ involvement. Conclusion: Cutaneous manifestations are important feature in SLE. LE non-specific was more common than LE specific manifestations in this study. Better understanding can help in efficient diagnosis and management. BIRDEM Med J 2022; 12(1): 57-61

2021 ◽  
Vol 2021 ◽  
pp. 1-19
Author(s):  
Elizabeth E. Cooper ◽  
Catherine E. Pisano ◽  
Samantha C. Shapiro

Lupus, Latin for “wolf,” is a term used to describe many dermatologic conditions, some of which are related to underlying systemic lupus erythematosus, while others are distinct disease processes. Cutaneous lupus erythematosus includes a wide array of visible skin manifestations and can progress to systemic lupus erythematosus in some cases. Cutaneous lupus can be subdivided into three main categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. Physical exam, laboratory studies, and histopathology enable differentiation of cutaneous lupus subtypes. This differentiation is paramount as the subtype of cutaneous lupus informs upon treatment, disease monitoring, and prognostication. This review outlines the different cutaneous manifestations of lupus erythematosus and provides an update on both topical and systemic treatment options for these patients. Other conditions that utilize the term “lupus” but are not cutaneous lupus erythematosus are also discussed.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1051-1052
Author(s):  
D. Lobo Prat ◽  
B. Magallares ◽  
I. Castellví ◽  
H. Park ◽  
P. Moya ◽  
...  

Background:Systemic lupus erythematosus (SLE) is an autoimmune disease with variable clinical features and a complex physiopathology. In 2019, EULAR and ACR have jointly developed new classification criteria with both high sensitivity and specificity. These criteria have the particularity of including the presence of ANA as an obligatory entry criterion and the existence of clinical and immunological domains with weighted scores.Objectives:To evaluate the performance and characteristics of the ACR/EULAR 2019, SLICC 2012 and ACR 1997 classification criteria in a cohort of SLE patients with longstanding disease.Methods:Descriptive observational study that enrolled a cohort of SLE patients with longstanding disease followed in a tertiary level hospital. Demographic and clinical data were gathered along with the fulfillment of classification criteria. The sensitivity of each classification criteria and the statistically significant associations between criteria fulfillment and clinical and immunological data were calculated. Statistical analyses were performed using the Chi2, T-student and ANOVA tests. Statistical significance was assumed in p values <0.05.Results:A total of 79 patients (88.6% women) with a mean age of 51.8±14 years, disease duration of 15.2±11.5 years and SLEDAI of 2.65±2.1 were included. The sensitivity of the different classification criteria was 51.9% for ACR 1997, 87.3% for SLICC 2012 and 86.1% for ACR/EULAR 2019 (Table 1).Table 1.Sensitivity and average scores.ACR/EULAR 2019SLICC 2012ACR 1997Sensitivity (%)86.187.351.9Average score of patients classified as SLE(±SD)18.6±5.85.3±1.45±0.9Average score of patients NOT classified as SLE(±SD)6.1±2.52.8±0.42.8±0.851.9% of patients met all three classification criteria, 29.1% met SLICC 2012 and ACR/EULAR 2019, 5% only met SLICC 2012 and 3.7% exclusively met ACR/EULAR 2019. 11.4% of patients did not meet any classification criteria and were characterized by having a low SLEDAI (0.6±0.9) and fulfilling only skin domains (alopecia or oral ulcers), antiphospholipid antibodies or hypocomplementemia.Statistically significant associations were found between meeting ACR/EULAR 2019 classification criteria and the presence of low C3 and C4 (p<0.04), DNA (p<0.001), lupus nephritis III-IV (p<0.05) and arthritis (p<0.001), highlighting that all patients with arthritis met these criteria.In the SLICC 2012 evaluation, significant associations were found between meeting these criteria and the presence of arthritis (p<0.01), renal involvement (p<0.04), leukopenia/lymphopenia (p=0.05), DNA (p<0.03) and hypocomplementemia (p=0.02).Fullfilment of ACR 1997 was associated to the presence of malar rash (p<0.001), discoid lupus (p<0.05), photosensitivity (p<0.001) and oral ulcers (p<0.04), as well as arthritis (p<0.001), serositis (p=0.02), renal (p<0.05) and hematologic (p=0.05) involvement.The Kappa concordance coefficient among classification criteria is detailed in Table 2.Table 2.Kappa concordance coefficient.ACR/EULAR 2019 - SLICC 2012ACR/EULAR 2019 - ACR 1997SLICC 2012 - ACR 1997Kappa concordance coefficient0.610.270.30Conclusion:The ACR/EULAR 2019 classification criteria maintain a high sensitivity similar to the SLICC 2012 in SLE patients with longstanding disease, both of which are much higher than ACR 1997. Patients with serological, articular or renal involvement are more likely to meet SLICC 2012 or ACR/EULAR 2019 criteria. It is noteworthy the relevance of dermatological manifestations in ACR1997 classification criteria against the increased weight that a better understanding of SLE physiopathology has provided to analytic and immunological criteria in the subsequent classification criteria.Disclosure of Interests:David Lobo Prat: None declared, Berta Magallares: None declared, Ivan Castellví Consultant of: Boehringer Ingelheim, Actelion, Kern Pharma, Speakers bureau: Boehringer Ingelheim, Actelion, Bristol-Myers Squibb, Roche, HyeSang Park: None declared, Patricia Moya: None declared, Ignasi Gich: None declared, Ana Laiz: None declared, Cesar Díaz-Torné: None declared, Ana Milena Millán Arciniegas: None declared, Susana P. Fernandez-Sanchez: None declared, Hector Corominas: None declared


Lupus ◽  
2020 ◽  
Vol 29 (10) ◽  
pp. 1216-1226
Author(s):  
Beatriz Frade-Sosa ◽  
Javier Narváez ◽  
Tarek Carlos Salman-Monte ◽  
Raul Castellanos-Moreira ◽  
Vera Ortiz-Santamaria ◽  
...  

Background The concomitant presence of two autoimmune diseases – systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) – in the same patient is known as rhupus. We evaluated a group of patients with rhupus to clarify further their clinical, serological and immunogenic features in a multi-centre cohort. In addition, the study aimed to explore the utility of the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) SLE classification criteria in our group of patients with rhupus. Methods This was a cross-sectional study. We included rhupus patients from 11 different rheumatology departments, and compared them to SLE and RA patients at a ratio of 2:1. All information was recorded following a pre-established protocol. Results A total of 200 patients were included: 40 rhupus patients and 80 each of SLE and RA patients as controls. Disease duration was similar among SLE and rhupus groups (around 13 years), but the RA group had a significantly lower disease duration. Main clinical manifestations were articular (94.2%), cutaneous (77.5%) and haematological (72.5%). Rhupus patients had articular manifestations similar to those expected in RA. Only 10% of rhupus patients had renal involvement compared with 25% of those with SLE ( p < 0.05), while interstitial lung disease was more common in patients affected by RA. The 2019 EULAR/ACR SLE criteria were met in 92.5% of the rhupus patients and in 96.3% of the SLE cohort ( p > 0.05). Excluding the joint domain, there were no differences between the numbers of patients who met the classification criteria. Conclusion Rhupus patients follow a particular clinical course, with full expression of both SLE and RA in terms of organ involvement, except for a lower prevalence of kidney affection. The new 2019 EULAR/ACR SLE criteria are not useful for differentiating SLE and rhupus patients. A new way of classifying autoimmune diseases is needed to identify overlapping clusters.


Lupus ◽  
2020 ◽  
Vol 29 (14) ◽  
pp. 1854-1865
Author(s):  
Hui Jin ◽  
Tao Huang ◽  
Ruifang Wu ◽  
Ming Zhao ◽  
Haijing Wu ◽  
...  

Although the original purpose of the systemic lupus erythematosus (SLE) classification criteria was to distinguish SLE from other mimic diseases, and to facilitate sample selection in scientific research, they have become widely used as diagnostic criteria in clinical situations. It is not known yet if regarding classification criteria as diagnostic criteria, what problems might be encountered? This is the first study comparing the three sets of classification criteria for SLE, the 1997 American College of Rheumatology (ACR’97), 2012 Systemic Lupus International Collaborating Clinics (SLICC’12) and 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR’19), for their ability to distinguish patients with SLE from patients with pure mucocutaneous manifestations (isolated cutaneous lupus erythematosus without internal disease, i-CLE) in the lupus disease spectrum. 1,865 patients with SLE and 232 patients with i-CLE were recruited from a multicenter study. We found that, due to low specificity, none of the three criteria are adept at distinguishing patients with SLE from patients with i-CLE. SLICC’12 performed best among the original three criteria, but if a positive ANA was removed as an entry criterion, EULAR/ACR’19 would performed better. A review of previous studies that compared the three sets of criteria was presented in this work.


2015 ◽  
Vol 38 (3) ◽  
pp. 124-129
Author(s):  
Shahana A Rahman ◽  
Mohammad Imnul Islam ◽  
Manik Kumar Talukder ◽  
Mohammed Mahbubul Islam ◽  
Syed Saimul Huque ◽  
...  

Background: Systemic lupus erythematosus in children (Paediatric SLE / pSLE) may have a great variability in disease presentation. Any organ system can be involved in pSLE leading to protean clinical manifestations.To evaluate the clinical and serological presentation of pSLE in tertiary renter Bangladesh and to compare it with other populations. Methodology: It was a retrospective cohort study carried out in the department of Paediatrics, Bangabandhu Sheikh Mujib Medical University, Dhaka during the period of January 2005 to December 2013. A total of 70 patients fulfilling the 1997 revised ACR classification criteria for SLE were enrolled in the study. Data was collected in a pre-designed questionnaire. Results: Age range was 2.5 years to 16 years, female: male ratio was 7:1. Duration of disease was 1 month to 18 months. Common clinical presentation included general weakness/fatigue (91%), fever (83%), arthralgia/arthritis (74%), oral ulcer (73%) and skin rash (71%). All the patients were anemic. ANA was positive in 97% cases and anti-ds DNA in 91% cases. More than 65% cases had evidences of renal involvement at the presentation. Among the patients who underwent kidney biopsy, 40% had diffuse proliferative lupus nephritis (WHO class IV) followed by class III in 26% cases. Conclusion: Pediatric SLE patients in our country, compared to other countries had much higher incidence of fever and non-specific complaints such as, general weakness and malaise as presenting symptoms. Thus, a strong index of suspicion should be maintained for early diagnosis of pSLE especially among adolescent girls. DOI: http://dx.doi.org/10.3329/bjch.v38i3.22819 Bangladesh J Child Health 2014; VOL 38 (3) :124-129


Lupus ◽  
2016 ◽  
Vol 26 (6) ◽  
pp. 646-649 ◽  
Author(s):  
E Y Wu ◽  
L E Schanberg ◽  
E C Wershba ◽  
C E Rabinovich

Objective Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide’s efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. Methods We performed a retrospective chart review of 10 adolescents who received lenalidomide for recalcitrant cutaneous lupus erythematosus. Information was gathered at drug initiation and 6-month follow-up. The Wilcoxon matched-pairs signed-rank test was used to assess change in quantitative parameters of disease activity. Results Nine subjects were girls and six were African-American. Indications for lenalidomide treatment included alopecia, nasal and oral ulcers, extensive malar rash, discoid lesions, bullous lesions, panniculitis, cutaneous vasculitis, and Raynaud’s phenomenon with digital ulcerations. Within 6 months, all patients demonstrated complete or near resolution based on physician report. Prednisone dose decreased from a mean 23.5 mg (SD± 13.3) to 12.25 mg (SD± 9.2) ( P= 0.008). Sedimentation rate decreased from a mean 29 mm/hour (SD± 31.5) to 17 mm/hour (SD± 18.1) ( P= 0.004). Lenalidomide was well tolerated. Conclusion Lenalidomide is an effective and safe treatment for a spectrum of dermatological conditions in pediatric systemic lupus erythematosus. Its use may allow a reduction in prednisone dose and decreased disfigurement. Prospective study is needed to clarify lenalidomide’s role in treating cutaneous manifestations of systemic lupus erythematosus.


2020 ◽  
Vol 5 (1) ◽  
pp. 1-4
Author(s):  
Habib N

Systemic lupus erythematosus or SLE is a persistent heterogeneous autoimmune disease that affects multisystem of the body. It is distinguished by acute and chronic inflammation of various tissues and even organs of the body principally the skin and joints. Systemic lupus erythematosus is a multisystem disorder and hence, it can affect any tissues, organs and even systems of the body. There are few categories of lupus for instance, lupus dermatitis or cutaneous lupus erythematosus (CLE) that affects the skin and causes malar rash, discoid lupus erythematosus (DLE) as well as systemic lupus erythematosus that causes damage to single or multiple internal organs. The damage is due to the inflammation that is caused by direct antibody reaction to the body tissues as well the deposition of immune complexes. Glucocorticoids, immunosuppressant, and anti- malarial are the combination therapy used to treat SLE besides providing counseling and awareness. Lupus erythematosus in any form particularly systemic lupus erythematosus (SLE) are prevalent in women compared to men with ratio of 6:1. It has the tendency to affect all ages but most frequently attacks women of aged 20 to 45 years old compared to men. On the other hand, if lupus erythematosus causes damage to internal organs either single or multiple, it is known as systemic lupus erythematosus. The damage is due to the inflammation that is caused by direct antibody reaction to the body tissues as well the deposition of immune complexes.


Lupus ◽  
2018 ◽  
Vol 27 (9) ◽  
pp. 1431-1436 ◽  
Author(s):  
N Leuchten ◽  
B Milke ◽  
B Winkler-Rohlfing ◽  
D Daikh ◽  
T Dörner ◽  
...  

Objective The European League Against Rheumatism and the American College of Rheumatology jointly embarked on a new classification criteria for systemic lupus erythematosus (SLE) project. Its first phase involved generation of a broad set of items potentially useful for classification of SLE. This study was undertaken to add the patient perspective to an expert Delphi approach and an early patient cohort study. Methods A national cross-sectional study was conducted. A self-report questionnaire was published in the “Schmetterling” (Butterfly), the quarterly journal of the German SLE patient association. Individuals with SLE were asked to anonymously complete the questionnaire, which asked for demographic details, organ manifestations, autoantibodies and symptoms. Results A total of 339 completed questionnaires out of 2498 were returned, a response rate of 13.6%; 83.2% reported they were ANA positive and 81.7% reported joint, 66.1% skin and 33.0% renal involvement. For the time before and in the first year after their SLE diagnosis, the majority reported fatigue (89.4%), joint pain (86.7%), photosensitivity (79.4%) and myalgia (76.1%). Of interest, more than half of the patients reported fever as an early symptom (53.7%). Conclusion For a Caucasian European SLE patient population, the overall characteristics suggest meaningful representation. While many symptoms were reported as expected, the high percentage of patients reporting fever and the significant number of patients with unexpected gastrointestinal complaints are of particular interest. These data add to the information on early SLE symptoms informing the development process of new SLE classification criteria.


2020 ◽  
Vol 11 (e) ◽  
pp. e165.1-e165.3
Author(s):  
Manjunath M Shenoy ◽  
Goutham Kilaru ◽  
Ashmiya Razak ◽  
Malcolm Pinto

Systemic lupus erythematosus (SLE) is a multiorgan autoimmune connective tissue disease with many muco-cutaneous manifestations. We report a case of SLE on treatment presented with scaly erythematous plaques on the trunk and extremities and diffuse scaly erythematous macular lesions on the face resembling cutaneous lupus erythematosus. Laboratory evaluation established a diagnosis of tinea corporis with faciei. He responded to long term itraconazole therapy. This case focuses on the modification of tinea in an autoimmune disorder on immunosuppressive therapy and its therapeutic implications.


2020 ◽  
Vol 4 (1) ◽  
pp. 42-46
Author(s):  
Huzairi Sani ◽  
Nada Syazana ◽  
Malek Faris Riza Feisal

Erythema nodosum is a septal panniculitis which is a variant of chronic cutaneous lupus erythematosus (CCLE). It is further classified in the group of Lupus Erythematous Panniculitis (LEP).[1] The most frequent cutaneous manifestations include indurated plaques, subcutaneous nodules and sometimes ulcerations. The lesions occur predominantly on the face, upper arms, upper trunk, breasts, buttocks and thighs.[2] They occur most frequently in adult females and do not typically manifest cutaneously in Systemic Lupus Erythematosus (SLE).[3] In this case report, we discuss a young gentleman who presented with erythema nodosum as a cutaneous feature of SLE.         Keywords: Systemic lupus erythematosus, erythema nodosum, panniculitis, cutaneous lupus


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