scholarly journals Juvenile lupus, cytomegalovirus infection and cardiac tamponade: case report

2022 ◽  
Vol 40 ◽  
Author(s):  
Levi Coelho Maia Barros ◽  
Matheus Eugênio de Sousa Lima ◽  
Roseny Marinho Mesquita Pereira ◽  
Lia Arcanjo Alves Vasconcelos ◽  
Willenne Campelo Rabelo
Keyword(s):  
Cardiac Tamponade ◽  
Lupus Erythematosus ◽  
Hemodynamic Instability ◽  
Severe Form ◽  
Cmv Infection ◽  
Cutaneous Manifestations ◽  
Diagnostic Confusion ◽  
History Of ◽  
Treatment Changes ◽  
Water Seal

ABSTRACT Objective: To describe a rare case of cardiac tamponade in a pediatric patient with systemic lupus erythematosus (SLE) and cytomegalovirus (CMV) infection, and to discuss the relationship between these morbidities, the diagnostic approach, and the possible treatments. Case description: A 9-year-old girl presented to the emergency department with severe dyspnea, muffled heart sounds, jugular vein distention, hemodynamic instability, and intense pallor. She had previously been followed up at the outpatient clinic for a six-month history of mild respiratory distress, polyarthritis, fever, and various cutaneous manifestations. Doppler echocardiogram revealed pericardial effusion. The patient was submitted to pericardiocentesis followed by water seal pleuropericardial drainage, with no complications. The investigation continued, with fulfillment of clinical and laboratory SLE criteria plus CMV antigenemia of 15/200,000 cells. Medications to control CMV infection and SLE were then initiated, with good clinical and laboratory response. Comments: Pediatric SLE commonly manifests in a more severe form, accounting for high morbimortality. Cardiac tamponade could be one of the first manifestations of SLE, which can also be precipitated by infectious agents, such as CMV, leading to diagnostic confusion and misleading the treatment. Changes in therapeutics must also be considered in the presence of both conditions. This study presents a juvenile SLE case aggravated by a CMV infection with the unusual manifestation of cardiac tamponade.%

Unilateral facial erythematous patch as the presenting sign of systemic lupus erythematosus

Lupus ◽  
2020 ◽  
Vol 29 (14) ◽  
pp. 1968-1970
Author(s):  
Dae-Lyong Ha ◽  
Gi-Wook Lee ◽  
Kihyuk Shin ◽  
Hoon-Soo Kim ◽  
Hyun-Chang Ko ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Autoimmune Disorder ◽  
Left Cheek ◽  
Systemic Lupus ◽  
Diagnostic Challenge ◽  
Cutaneous Manifestations ◽  
Oral Ulcers ◽  
Rare Manifestation ◽  
History Of

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that can have many cutaneous manifestations including malar rash, discoid rash or oral ulcer. Isolated unilateral involvement of face is uncommon in SLE. It lacks typical clinical features of LE, and may impose a diagnostic challenge for clinicians. Herein we report a case of 62-year-old woman presenting with a 2-year history of erythematous patches on left cheek and eyelid. Initially, she was diagnosed as having recurrent blepharitis or cellulitis that did not respond to conventional treatment with ophthalmic medicaments. As time went by, the patches spread to her left cheek, and she was referred to our dermatologic department. Histopathologic examination was consistent with LE. Further physical and laboratory tests have found that she had oral ulcers, proteinuria, thrombocytopenia and abnormal titer of anti-nuclear antibody satisfying the diagnosis of SLE. From this case, we think unilateral erythematous patches on face could be a rare manifestation of SLE and more intention should be paid to this type of patients, because unilateral facial symptom may mimic other dermatoses.

scholarly journals Systemic Lupus Erythematosus Associated with Neurological Manifestations in a Border Collie Bitch

2021 ◽  
Vol 49 ◽  
Author(s):  
Isabel Rodrigues Rosado ◽  
Julia Perinotto Picelli ◽  
Juliana Gonzaga da Silva ◽  
Marina Cazarini Madeira ◽  
Taís Teixeira Zambarda ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Urinary Incontinence ◽  
Lupus Erythematosus ◽  
Clinical Signs ◽  
Neurological Manifestations ◽  
Systemic Lupus ◽  
Cutaneous Lesions ◽  
Cutaneous Manifestations ◽  
Immune Mediated ◽  
History Of

Background: Systemic lupus erythematosus (SLE) is an immune-mediated and multisystemic disorder which etiology is believed to be multifactorial. Its clinical signs vary accordingly to affected organs, cutaneous lesions being the most frequently observed. There are few reports of SLE in dogs with neurological manifestations. Therefore, the aim of this report is to describe a case of SLE in a dog with indicative signs of nervous system involvement.Case: A 6-year-old Border Collie bitch was referred to the Veterinary Hospital (HVU) of the University of Uberaba (UNIUBE) with a history of  with cluster seizures, inappetence and urinary incontinence. Erythema and flaking of nasal plan were noted on physical examination, and splenomegaly on abdominal palpation. Thrombocytopenia and slightly increased ALT were found on blood tests. Ehrlichiosis was suspected and doxycycline was prescribed together with phenobarbital for the control of seizures. In the follow-up visit, the dog was still presenting urinary incontinence, thrombocytopenia and splenomegaly. Also, an ulcer on the nasal mucocutaneous junction was observed. The patient went through a neurological examination which indicated thalamocortical lesion. Cerebrospinal fluid samples were obtained for cytology, culture and canine distemper test, and serology tests for leishmaniasis, toxoplasmosis and neosporosis were done. No alterations were found in these exams. The histopathology of the nasal lesion was proceeded and showed results consistent with lupus erythematosus. It was prescribed a 15-day course of prednisolone at immunosuppressive dose. The patient showed clinical improvement with this treatment. Azathioprine was started along with gradual removal of prednisolone. After twenty days of discontinuation of this drug, the dog presented epileptic seizures, urinary incontinence, thrombocytopenia, increased ALT and worsened nasal lesion. Prednisolone at immunosuppressive dose was reintroduced and the dose of azathioprine, increased. One week past this, the patient showed inappetence and an extensive hematoma in the thoracic region. Lab exams confirmed drug-induced acute pancreatitis. All medications were interrupted, the patient was hospitalized, and treatment for pancreatitis was initiated, but the dog passed away.Discussion: For involving multiple body systems and for presenting varied clinical signs, diagnosing SLE can be challenging in clinical routine. The dog from this report was a Border Collie; this breed is considered to be predisposed to this disease. The animal had a history of being exposed to solar radiation for a large part of the day, had dyspigmentation of nasal plan and had no application of sunscreen, predisposing the occurrence of SLE. Neurological signs are uncommon in SLE, but the seizures and the urinary incontinence were the main reasons for the dog’s guardian to look for medical assistance. The suspicion for SLE was raised due to cutaneous manifestations and persistent thrombocytopenia along with splenomegaly. Histopathological findings are essential for diagnosing SLE, as well as antinuclear antibody tests. Nonetheless, due to financial limitations, this last test was not performed. Azathioprine is an immunomodulating drug largely used along with glucocorticoids when treating SLE; however, this medication is prone to induce side effects as the ones presented by the dog from this report. Therefore, it is concluded that SLE should be considered as a differential diagnosis in patients showing cutaneous, hematological, systemic and neurological manifestations, considering the variety of signs caused by this disorder.Keywords: seizures, dog, immune-mediated encephalopathy. Lúpus eritematoso sistêmico associado a manifestações neurológicas em cadela da raça Border CollieDescritores: crises epilépticas, cão, encefalopatia imunomediada.

scholarly journals Acute Vascular Reactive Inflammatory Acral Skin Lesions and SARS-CoV-2

2021 ◽  
Vol 79 (4) ◽  
pp. 315-320
Author(s):  
Renan Minotto ◽  
Joana R. Fitz ◽  
Bruna M. Bauer ◽  
Ana Helena Choi
Keyword(s):  
Lupus Erythematosus ◽  
Acute Infection ◽  
Previous History ◽  
Weather Conditions ◽  
Skin Lesions ◽  
Distal Margin ◽  
Cutaneous Manifestations ◽  
Diagnosis And Prognosis ◽  
History Of ◽  
Hot Weather

Introduction: Coronavirus disease 2019 (COVID-19) predominantly affects the respiratory system, but there have been many reports of skin involvement. Several cutaneous manifestations associated with COVID-19 have been identified, including acral lesions (pernio-like), which we intend to better characterize in this study. Methods: Demographic, clinical and major histopathologic characteristics of acral skin lesions occurring during the acute infection stage of COVID-19 disease were evaluated in this survey conducted in the Dermatology Division of Santa Casa Hospital Complex, Federal University of Health Sciences, Porto Alegre, Brazil. Results: In the present study, we evaluated eight female patients with acute SARS-CoV-2 infection confirmed by PCR with acral skin lesions including pernio-like. The mean age was 43.6 and with no other acute disease, who had a good outcome with need for hospitalization only in 2 cases. Pernio-like lesions were observed under hot weather conditions in patients who denied any previous history of similar cutaneous manifestations. Some acral lesions resembled those of lupus erythematosus and of herpes simplex paronychia, but laboratory tests were negative for those conditions. Unusual nail involvement was present, characterized by half-moon shaped erythematous bands at the distal margin of the lunula. Conclusion: Recognition of these skin lesions is important in that they could be potential markers, aiding in the diagnosis and prognosis of COVID-19 disease.

Clinical Characteristics of Patients Diagnosed with Strongyloidiasis in a United States Urban Outpatient Dermatology Department: A Case Series

2017 ◽  
Vol 1 (3) ◽  
pp. 156-160
Author(s):  
Jacqueline Watchmaker ◽  
Sean Legler ◽  
Dianne De Leon ◽  
Vanessa Pascoe ◽  
Robert Stavert
Keyword(s):  
United States ◽  
Case Series ◽  
The United States ◽  
Screening Tools ◽  
Dermatology Department ◽  
Cutaneous Manifestations ◽  
Serologic Testing ◽  
Patient Reported ◽  
History Of ◽  
Endemic Areas

Background: Although considered a tropical disease, strongyloidiasis may be encountered in non-endemic regions, primarily amongst immigrants and travelers from endemic areas.  Chronic strongyloides infection may be under-detected owing to its non-specific cutaneous presentation and the low sensitivity of commonly used screening tools. Methods: 18 consecutive patients with serologic evidence of strongyloides infestation who presented to a single urban, academic dermatology clinic between September 2013 and October 2016 were retrospectively included.  Patient age, sex, country of origin, strongyloides serology titer, absolute eosinophil count, presenting cutaneous manifestations, and patient reported subjective outcome of pruritus after treatment were obtained via chart review.  Results: Of the 18 patients, all had non-specific pruritic dermatoses, 36% had documented eosinophila and none were originally from the United States. A majority reported subjective improvement in their symptoms after treatment. Conclusion:  Strongyloides infection and serologic testing should be considered in patients living in non-endemic regions presenting with pruritic dermatoses and with a history of exposure to an endemic area.Key Points:Chronic strongyloidiasis can be encountered in non-endemic areas and clinical manifestations are variableEosinophilia was not a reliable indicator of chronic infection in this case series Dermatologists should consider serologic testing for strongyloidiasis in patients with a history of exposure and unexplained pruritus

scholarly journals AB0420 RISK OF DEVELOPING TYPE 2 DIABETES MELLITUS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1510.2-1510
Author(s):  
L. Kondrateva ◽  
T. Popkova ◽  
E. Nasonov ◽  
A. Lila
Keyword(s):  
Abdominal Obesity ◽  
Lupus Erythematosus ◽  
Antihypertensive Drugs ◽  
Control Group ◽  
Systemic Lupus ◽  
Type 2 Dm ◽  
History Of ◽  
Systemic Rheumatic Diseases ◽  
Very High

Background:Patients with systemic lupus erythematosus (SLE) have higher than in general population prevalence of diabetes mellitus (DM). Hyperinsulinemia is a predictor of developing type 2 DM, however routine measurement of insulin levels for DM risk assessment is uncomfortable in daily clinical practice. International Diabetes Federation recommends the use of patient questionnaires to quickly identify people who may be at a higher risk of DM development.Objectives:To determine the 10-years risk of developing type 2 DM in SLE patients using dedicated questionnaire - Finnish Type 2 Diabetes Risk Assessment Form (FINDRISK) data.Methods:The study included 92 SLE patients without DM (83 women, 9 men, 39 [34; 47] years old). The median disease duration was 6 [2,14] years, SLEDAI-2K was 4[2;8]. SLE pts were treated with glucocorticoids (GC) (89%) and hydroxychloroquine (78%), immunosuppressive drugs (28%) and biological agents (10%). The control group consisted of 88 subjects without systemic rheumatic diseases, inflammatory arthritis or DM, matched by age and sex with SLE patients. Eight items of FINDRISK questionnaire (age, overweight, abdominal obesity, family history of diabetes, physical inactivity, eating habits, history of antihypertensive drugs treatment, history of hyperglycemia) were taken into account to calculate the total risk score (TS). The risk of developing DM within following 10 years is regarded as low (1%) or slightly elevated (4%) with TS ≤11 points, as moderate (17%), high (33%) or very high (50%) with TS ≥12 points.Results:The risk of developing DM was low or slightly elevated in 65 (71%) SLE pts and moderate, high or very high in 27 (29%) pts. The difference was significant compared with the control group, in which 76 (86%) subjects had a low or slightly elevated risk and 12 (14%) had a moderate, high or very high risk (p=0,01). The number of risk factors (4[2;5]) and the median TS of SLE pts (9[5;12] points) were higher than values in control subjects (3[2,4] factors and 6[3;9] points, respectively) (p<0,01 for both). DM risk factors profiles were similar in two groups, except for higher prevalence of abdominal obesity (66% vs 41%, p<0,01) and history of antihypertensive drugs treatment (57% vs 17%, p<0,01) in SLE. There were positive correlations between TS and CRP levels (r=0,25, p=0,02), SLICC (r=0,36, p<0,01), HAQ (r=0,29, p<0,01), and negative correlations between TS and SLEDAI-2K (r= -0,32, p<0,01), glomerular filtration rate by CKD-EPI (r=-0,23, p=0,03). Current GC use had no influence on TS values in SLE.Conclusion:Patients with SLE were more likely than individuals without systemic rheumatic diseases to have a moderate, high and very high risk of developing DM, and therefore, required interventions to prevent the metabolic disease. Increased risk of developing DM was associated with most common traditional factors, especially by abdominal obesity and regular use of antihypertensive drugs that can be considered a kind of equivalent to the presence of hypertension. Curtain contribution of inflammation, lupus activity and irreversible damage index can’t be ignored. Clarification of SLE-specific phenomena in DM pathogenesis requires further research.Disclosure of Interests: :None declared

scholarly journals Atypical and Rare Forms of Cutaneous Lupus Erythematosus: The Importance of the Diagnosis for the Best Management of Patients

Dermatology ◽  
2021 ◽  
pp. 1-10
Author(s):  
Astrid Herzum ◽  
Giulia Gasparini ◽  
Emanuele Cozzani ◽  
Martina Burlando ◽  
Aurora Parodi
Keyword(s):  
General Practitioner ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Cutaneous Lupus Erythematosus ◽  
Cutaneous Manifestations ◽  
Skin Manifestation ◽  
Systemic Involvement ◽  
Best Management ◽  
Wide Range ◽  
Cutaneous Lupus

Lupus erythematosus (LE) is an autoimmune disease with a wide range of clinical and cutaneous manifestations. Along with the well-known typical cutaneous manifestations of LE, some cutaneous manifestations are rarer, but still characteristic, enabling the dermatologist and the general practitioner who know them to suspect cutaneous LE (CLE) and investigate a possible underlying systemic involvement. Indeed, not infrequently a skin manifestation is the first presentation of systemic LE (SLE), and &#x3e;75% of SLE patients show signs of skin disease during the course of the illness. Especially, SLE involvement occurs in cases of acute CLE, while it is uncommon in subacute CLE and rare in chronic CLE. This review aims to concentrate especially on atypical cutaneous manifestations of LE to enable the clinician to diagnose even the rarest forms of CLE.

scholarly journals OP0102 GENE SCREENING OF PRIMARY IMMUNODEFICIENCY DISEASES IN PATIENTS WITH JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 66.2-66
Author(s):  
J. LI ◽  
W. Wang ◽  
C. Y. Wang ◽  
J. Y. Pan ◽  
H. Song
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Primary Immunodeficiency ◽  
Lupus Erythematosus ◽  
Gene Sequencing ◽  
Primary Immunodeficiency Disease ◽  
Type I ◽  
Cmv Infection ◽  
Systemic Lupus ◽  
Genetic Tests ◽  
Immunodeficiency Disease

Background:Systemic lupus erythematosus (SLE) is one of the most common auto-immune diseases in childhood. Primary immunodeficiency disease (PID) patients may present or combine with autoimmune diseases.Objectives:This study aimed to perform gene sequencing via high-throughput sequencing technology in a series of Chinese pediatric SLE patients, and investigate the concomitant situation of PIDs and SLE. Gene sequencing results may help clarify the pathogenesis of SLE.Methods:This was a retrospective case series of SLE children who referred to the Peking Union Medical College Hospital between 01/2016 and 09/2019. Genetic tests were performed in patients who met the inclusion criteria. We then collected demographic, clinical, and treatment information of all involved patients. Descriptive statistics were used.Results:Seventy-one patients were finally included (eighteen boys and fifty-three girls). The median age at the time of disease onset was 9.5 (range, 3-15) years. It is notable that five patients experienced their first attack before the age of five. Twenty-seven patients showed a persistent increase in ESR during treatment, while thirteen cases presented with repeated CMV infection, thirty-four cases with persistent low complement levels, seven with basal ganglia calcification showed in skull CT or MRI, four with special type of rash (i.e., frostbite-like rash, discoid erythema, reticular erythema), two with obvious hepatosplenomegaly, and one case with type I diabetes. Gene sequencing results showed that about ten patients combine with primary immunodeficiency disease, including Aicardi-Goutières Syndrome (AGS) (n=4), Spondyloenchondro-dysplasia with immune dysregulation (SPENCDI) (n=1), STING-associated vasculopathy with onset in infancy (SAVI) (n=1), lysinuric protein intolerance (LPI) (n=1), Ras-associated autoimmune leukoproliferative disorder (RALD) (n=2).Conclusion:SLE patients who present atypical or refractory manifestations should attach importance to the existence of primary immunodeficiency disease. Genetic tests are recommended for patients with early-onset SLE, especially those with recurrent frostbite-like rash or persistent CMV infection since childhood.References:[1]T Tarr, B Dérfalvi, N Győri, et al. Similarities and differences between pediatric and adult patients with systemic lupus erythematosus[J]. Lupus. 2015, 24: 796–803.[2]Gupta S, Louis A G. Tolerance and Autoimmunity in Primary Immunodeficiency Disease: a Comprehensive Review[J]. Clinical Reviews in Allergy & Immunology, 2013, 45(2):162-169.Disclosure of Interests:None declared

scholarly journals “It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case

Lupus ◽  
2021 ◽  
pp. 096120332098176
Author(s):  
Sarah J van der Lely ◽  
Jeffrey Boorsma ◽  
Marc Hilhorst ◽  
Jesper Kers ◽  
Joris Roelofs ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Gestational Trophoblastic Disease ◽  
Grand Round ◽  
Placental Site Trophoblastic Tumor ◽  
Trophoblastic Tumor ◽  
Anchoring Bias ◽  
Placental Site ◽  
History Of

Introduction: Placental site trophoblastic tumor (PSTT) is a rare subtype of gestational trophoblastic disease. Association of PSTT and nephrotic syndrome is exceedingly rare and has been described in 8 cases thus far. In all cases hysterectomy was performed within months after onset of symptoms, leading to immediate remission of nephrotic syndrome, except for one patient who died of complications of PSTT. Case: We describe the history of a woman in which PSTT was discovered years after onset of nephrotic syndrome. Kidney biopsy revealed lupus-like mesangiocapillary nephritis and over time the patient developed additional symptoms mimicking systemic lupus erythematosus (SLE). Discussion: We provide an overview of the literature on this clinical entity and elaborate on its pathophysiology. In addition, we reflect on the phenomenon of anchoring bias, that led physicians to assume the patient had SLE without questioning this diagnosis in the light of the unexplained finding of increased tumor markers.

“Protenuria in SLE: Is it always lupus?”

Lupus ◽  
2021 ◽  
pp. 096120332098345
Author(s):  
Alessandra Ida Celia ◽  
Roberta Priori ◽  
Bruna Cerbelli ◽  
Francesca Diomedi-Camassei ◽  
Vincenzo Leuzzi ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Fabry Disease ◽  
Histological Examination ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Kidney Involvement ◽  
History Of ◽  
Genetic Assay

Proteinuria is one of the most typical manifestations of kidney involvement in Systemic Lupus Erythematosus (SLE). We report the case of a 23-year-old woman with a 6-year-long history of SLE presenting with proteinuria after a three-year remission on hydroxychloroquine. Kidney histological examination showed alterations inconsistent with lupus nephritis and suggestive of hydroxychloroquine toxicity or Fabry disease. The latter was confirmed by genetic assay.

scholarly journals Atypical cutaneous and musculoskeletal manifestation of SARS-CoV-2: ‘COVID-19 toes’ and spasticity in a 48-year-old woman

2021 ◽  
Vol 14 (3) ◽  
pp. e241410
Author(s):  
Avery Kopacz ◽  
Cameron Ludwig ◽  
Michelle Tarbox
Keyword(s):  
Nervous System ◽  
Adult Patient ◽  
Early Identification ◽  
Lower Limbs ◽  
Rare Occurrence ◽  
Cutaneous Manifestations ◽  
Crucial Component ◽  
History Of ◽  
Screening Questions ◽  
Insight Into

Establishing accurate symptomatology associated with novel diseases such as COVID-19 is a crucial component of early identification and screening. This case report identifies an adult patient with a history of clotting dysfunction presenting with rare cutaneous manifestations of COVID-19, known as ‘COVID-19 toes’', previously described predominantly in children. Additionally, this patient presented with possible COVID-associated muscle spasticity of the lower limbs, as well as a prolonged and atypical timeline of COVID-19 infection. The rare occurrence of ‘COVID-19 toes’' in this adult patient suggests that her medical history could have predisposed her to this symptom. This supports the coagulopathic hypothesis of this manifestation of COVID-19 and provides possible screening questions for patients with a similar history who might be exposed to the virus. Additionally, nervous system complaints associated with this disease are rare and understudied, so this novel symptom may also provide insight into this aspect of SARS-CoV-2.

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