scholarly journals Cutaneous Manifestations of Systemic Lupus Erythematosus

1970 ◽  
Vol 8 (2) ◽  
pp. 44-48 ◽  
Author(s):  
Nahid Parveen ◽  
Dipak Kumar Das ◽  
FRM Mahbubul Haque ◽  
Mahamudun Nabi Mohammad Momtazul Haque

Systemic lupus erythematosus (LE) is a heterogeneous, multisystem, autoimmune disease characterized by the production of auto-antibodies against several cell constituents. The skin is one of the target organs most variably affected by the disease can be yield valuable diagnostic & prognostic information. The present study was conducted to evaluate cutaneous manifestations of SLE in Bangladeshi patients. 100 cases of SLE were collected from the Department of Dermatology, BSMMU, Dhaka. The diagnosis was based on American Rheumatism Association Criteria. Cutaneous changes were recorded on a predevised pro forma. LE specific cutaneous changes noted were: Malar rash or butterfly rash (70.0%), maculopapular rash (19.0%), discoid rash (10.0%), and bullous LE (10.0%). Non specific LE lesions noted were: Photosensitivity (75.0%), alopecia (47.0%), oral mucosal lesion (34.0%), hyperpigmentation (20.0%), nail change (15.0%), Raynauds phenomenon (12.0%), Palmar erythema, genital lesion, periungual talangiectesia, leg ulcers, livedo reticularis, erythema multiform were rare. Systemic lupus erythematosus is a multisystem disease that predominantly affects women. Cutaneous lesions are the most important diagnostic tools as reflected by the fact that they account for four of the 11 revised American Rheumatism Association criteria of SLE. The pattern of skin changes may vary from place to place. Key Words: SLE, LE specific and non specific cutaneous lesions   DOI = 10.3329/jom.v8i2.1405 J MEDICINE 2007; 8 : 44-48

2021 ◽  
Vol 49 ◽  
Author(s):  
Isabel Rodrigues Rosado ◽  
Julia Perinotto Picelli ◽  
Juliana Gonzaga da Silva ◽  
Marina Cazarini Madeira ◽  
Taís Teixeira Zambarda ◽  
...  

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.


Lupus ◽  
2017 ◽  
Vol 27 (1) ◽  
pp. 134-138 ◽  
Author(s):  
Z Ekinci ◽  
K Ozturk

Treatment and outcome of systemic lupus erythematosus (SLE) in C1q deficient patients are rarely reported. The aim of this report is to share our experience about the course of management of three cases diagnosed as SLE with C1q deficiency, in light of present literature. Initial and dominant complaints of three cases from two different families were cutaneous manifestations. One patient was also diagnosed with arthritis and thrombocytopenia. Antinuclear antibody was positive in all cases, whereas anti-dsDNA was negative with normal levels of complement C3, C4 and decreased CH50 activity. C1QA gene of two patients had homozygous nonsense mutation (c.622 > T/p.Gln208Ter). Previously, all of them had been treated with steroids, hydroxychloroquine and methotrexate or azathioprine. It was learned that they had responded only to high dosage prednisolone and their symptoms flared up during dosage reduction even under methotrexate or azathioprine. All symptoms of all three cases improved by daily fresh frozen plasma (FFP) infusions, and once cutaneous lesions subsided, the infusions were reduced to a frequency that would prevent the flare up of the symptoms. Literature search revealed seven reports on fresh frozen plasma treatment in SLE with C1q deficient patients. In this report, it is concluded that severe cutaneous lesions, as seen in these C1q deficient SLE patients, cannot be controlled with conventional immunosuppressive treatment. Instead, regular fresh frozen plasma infusions are proposed as a more reasonable method of treatment.


Lupus ◽  
2019 ◽  
Vol 29 (1) ◽  
pp. 74-78
Author(s):  
N Akdogan ◽  
C Demircan ◽  
E C Bolek ◽  
O Gokoz ◽  
A Karaduman

Indeterminate cell histiocytosis (ICH) is an extremely rare clonal proliferative disorder of dendritic cells which presents with skin lesions in the majority of cases. Although extra-cutaneous manifestations are very rare, ICH may involve the mucosa, cornea, and conjunctiva as well as the visceral organs. Since the clinical appearance of cutaneous lesions of ICH is not distinctive, it is diagnosed with histopathological and immunohistochemical findings after clinical suspicion. Herein, we report a 27-year-old man with a two-year history of asymptomatic reddish papules and papulonecrotic lesions on his face, arms and buttocks. He was previously diagnosed with systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS), and he had been treated with hydroxychloroquine and low-dose aspirin. Diffuse dermal infiltration of a mixture of histiocytes and lymphocytes accompanied with multinuclear giant cells, the positive CD68 and Factor XIIIa and negative Langerin immunoreactions, along with the positive staining with CD1a and S100, led us to the diagnosis of ICH. To the best of our knowledge, this is the first case of ICH associated with SLE and APS.


2012 ◽  
Vol 2012 ◽  
pp. 1-15 ◽  
Author(s):  
Luís Uva ◽  
Diana Miguel ◽  
Catarina Pinheiro ◽  
João Pedro Freitas ◽  
Manuel Marques Gomes ◽  
...  

Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. The skin is one of the target organs most variably affected by the disease. The American College of Rheumatology (ACR) established 11 criteria as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. Cutaneous lesions account for four of these 11 revised criteria of SLE. Skin lesions in patients with lupus may be specific or nonspecific. This paper covers the SLE-specific cutaneous changes: malar rash, discoid rash, photosensitivity, and oral mucosal lesions as well as SLE nonspecific skin manifestations, their pathophysiology, and management. A deeper thorough understanding of the cutaneous manifestations of SLE is essential for diagnosis, prognosis, and efficient management. Thus, dermatologists should cooperate with other specialties to provide optimal care of SLE patient.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1041.1-1041
Author(s):  
V. Agarwal ◽  
S. Kakati ◽  
P. Debbaruah

Background:SNP rs7574865, located within the third intron of STAT4 gene at chromosome 2, has been associated with susceptibility to SLE among different ethnic groups.1,2 Interestingly, we recently have documented an association between this gene and susceptibility to systemic lupus erythematosus (SLE) in Indian population.3Objectives:To determine whether the STAT4 (rs7574865) SNP is associated with clinical and immunological manifestations in SLE.Methods:The study was carried out on 100 unrelated SLE (SLICC criteria 2012) patients from North-East India. Genotyping of STAT4 rs7574865 SNP was done using Taqman probe and Real-Time Polymerase chain reaction. An association study was performed between the alleles and genotypes of STAT4 rs7574865 with the clinical and immunological manifestations included in the SLE SLICC classification criteria. For all analysis, the statistical significance was fixed at 5% level of significance (p < 0.05).Results:The mean duration of illness was 2.69±2.55 years. Cases and Controls remained in Hardy-Weinberg equilibrium.The occurrence of Photosensitivity and hyperpigmentation was significantly higher in TT genotype group (97.22% and 77.77%, respectively) with p <0.001 in each case.SLE patients with nephritis (Albuminuria >500mg/24 hours) and elevated serum creatinine were both significantly higher in TT genotype group as compared to GT and GG (p< 0.001 and p=0.001 respectively).The Anti-dsDNA antibody was significantly associated with TT genotype (p <0.001).Conclusion:Our study provides evidence regarding the association between STAT4 rs7574865 gene polymorphism is risk factor for cutaneous manifestations, Lupus nephritis and Anti ds-DNA positivity in SLE. So, our findings reinforce the need for further association studies including prospective studies with larger subjects in order to replicate such findings.References:[1]Graham RR, Ph D, Hom G, Ph D, Behrens TW, Bakker PIW De, et al. and the Risk of Rheumatoid Arthritis and Systemic Lupus Erythematosus. N Engl J Med. 2007;357(10):977–86.[2]Yuan H, Feng JB, Pan HF, Qiu LX, Li LH, Zhang N, et al. A meta-analysis of the association of STAT4 polymorphism with systemic lupus erythematosus. Mod Rheumatol. 2010;20(3):257–62.[3]Gupta V, Kumar S, Pratap A, Singh R, Kumari R, Kumar S, et al. Association of ITGAM, TNFSF4, TNFAIP3 and STAT4 gene polymorphisms with risk of systemic lupus erythematosus in a North Indian population. Lupus. 2018;27(12):1973–9.Disclosure of Interests:None declared


2003 ◽  
Vol 7 (3) ◽  
pp. 232-235
Author(s):  
Audrey Dupéré ◽  
Yves Poulin

Background: Acquired partial lipodystrophy is a rare disorder. An association with systemic lupus erythematosus has been reported. In these cases, an immunologic basis is suggested by the presence of C3 nephritic factor and hypocomplementemia. Objective: The following report presents the case of a woman who developed a rapid loss of facial subcutaneous fat a few months after complete spontaneous resolution of cutaneous lesions of lupus. Conclusion: Absence of C3NeF in this case suggests that other immunological factors may be involved in the pathogenesis.


2020 ◽  
Vol 4 (3) ◽  
pp. 260
Author(s):  
Luke J Maxfield ◽  
Laura S Tanner ◽  
Chelsea Schwartz

Systemic lupus erythematosus (SLE) is a multi-system disease with a myriad of mucocutaneous and systemic findings. One of the atypical cutaneous manifestations is palisaded neutrophilic granulomatous dermatitis (PNGD). This uncommon condition presents as tender or asymptomatic, flesh-colored, red to violaceous subcutaneous nodules. The diagnosis may be suspected clinically but is confirmed by biopsy. The impact of the disease may be the direct result of pain, psychosocial, cosmetic concerns, or be the initial presentation of an underlying systemic disease. We present a patient with known SLE who developed PNGD. We also review similar clinical and microscopic disease entities with a summative comparison of neutrophilic dermatoses in patients with autoimmune connective tissue diseases. 


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.


Lupus ◽  
2021 ◽  
pp. 096120332110300
Author(s):  
Evandro Mendes Klumb ◽  
Morton Scheinberg ◽  
Viviane Angelina de Souza ◽  
Ricardo Machado Xavier ◽  
Valderilio Feijo Azevedo ◽  
...  

Purpose The objective of this review is to address the barriers limiting access to diagnosis and treatment of systemic lupus erythematosus (SLE) and lupus nephritis (LN) in Brazil, specifically for patients in the public healthcare system, arguably those with the least access to innovation. Design A selected panel of Brazilian experts in SLE/LN were provided with a series of relevant questions to address in a multi-day conference. During the conference, responses were discussed and edited by the entire group through numerous drafts and rounds of discussion until a consensus was achieved. Results The authors propose specific and realistic recommendations for implementing access to innovative diagnostic tools and treatment alternatives for SLE/LN in Brazil. Moreover, in creating these recommendations, the authors strived to address barriers and impediments for technology adoption. The multidisciplinary care required for SLE/LN necessitates the collective participation of all involved stakeholders. Conclusion A great need exists to expand the adoption of innovative diagnostic tools and treatments for SLE/LN not only in Brazil but also in most countries, as access issues remain an urgent demand. The recommendations presented in this article can serve as a strategy for new technology adoption in other countries in a similar situation.


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