Cutaneous manifestations of Hughes syndrome occurring in the context of lupus erythematosus

Lupus ◽  
1997 ◽  
Vol 6 (2) ◽  
pp. 139-144 ◽  
Author(s):  
C. Francès ◽  
J-C. Piette
Keyword(s):  
Lupus Erythematosus ◽  
Cutaneous Manifestations ◽  
Hughes Syndrome

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 >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 AB0013 ASSOCIATION BETWEEN STAT4 POLYMORPHISM AND MANIFESTATIONS OF SLE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1041.1-1041
Author(s):  
V. Agarwal ◽  
S. Kakati ◽  
P. Debbaruah
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Indian Population ◽  
Association Studies ◽  
Statistical Significance ◽  
Elevated Serum ◽  
Systemic Lupus ◽  
Cutaneous Manifestations ◽  
Genotype Group ◽  
Dsdna Antibody

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

Cutaneous manifestations of lupus erythematosus

1990 ◽  
pp. 27-48
Author(s):  
C. R. Lovell ◽  
P. J. Maddison ◽  
G. V. Campion
Keyword(s):  
Lupus Erythematosus ◽  
Cutaneous Manifestations

scholarly journals Extensive Palisaded Neutrophilic Granulomatous Dermatitis with Systemic Lupus Erythematosus

2020 ◽  
Vol 4 (3) ◽  
pp. 260
Author(s):  
Luke J Maxfield ◽  
Laura S Tanner ◽  
Chelsea Schwartz
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Disease ◽  
Connective Tissue Diseases ◽  
Direct Result ◽  
Systemic Lupus ◽  
Cutaneous Manifestations ◽  
Neutrophilic Dermatoses ◽  
Granulomatous Dermatitis ◽  
The Impact

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. 

scholarly journals Cutaneous Manifestations of “Lupus”: Systemic Lupus Erythematosus and Beyond

2021 ◽  
Vol 2021 ◽  
pp. 1-19
Author(s):  
Elizabeth E. Cooper ◽  
Catherine E. Pisano ◽  
Samantha C. Shapiro
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Treatment ◽  
Treatment Options ◽  
Cutaneous Lupus Erythematosus ◽  
Laboratory Studies ◽  
Systemic Lupus ◽  
Cutaneous Manifestations ◽  
Distinct Disease ◽  
Cutaneous Lupus

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.

scholarly journals Clinical profile of cutaneous manifestations of connective tissue diseases in North-East India

2021 ◽  
Vol 7 (5) ◽  
pp. 702
Author(s):  
Deepa Mala Subba ◽  
Nandakishore Thokchom ◽  
Linda Kongbam ◽  
Erika Salam ◽  
Deepa Yumnam
Keyword(s):  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Cutaneous Lupus Erythematosus ◽  
Connective Tissue Diseases ◽  
Skin Lesions ◽  
Discoid Lupus Erythematosus ◽  
Serological Tests ◽  
Cross Sectional ◽  
Cutaneous Manifestations ◽  
North East

<p class="abstract"><strong>Background:</strong> Connective tissue diseases (CTDs) are a heterogeneous group of autoimmune disorders having overlapping clinical features. Skin is often involved and it may be the earliest sign of the disease. This study highlighted the various cutaneous manifestations of common CTDs.</p><p class="abstract"><strong>Methods:</strong> A hospital-based cross-sectional study was carried out for a period of two years in 83 patients with CTDs in dermatology OPD, RIMS, Imphal. Detailed history taking, examination and relevant serological tests were performed.<strong></strong></p><p class="abstract"><strong>Results:</strong> The mean age was 39.78±17.29 years with female to male ratio of 4.5:1. Majority of the patients had lupus erythematosus (LE) (N=45) followed by systemic sclerosis (SSc) (N=25), rheumatoid arthritis (RA) (N=6), mixed connective tissue disease (MCTD) (N=4) and morphea (N=3). The most common presentation was raised skin lesions (45.8%) followed by Raynaud’s phenomenon (36.1%), photosensitivity (27.7%), skin tightness (26.5%) and joint pain (19.3%). Among LE patients, chronic cutaneous lupus erythematosus (CCLE) was the commonest variant and localised discoid lupus erythematosus (DLE) (22.9%) was the commonest presentation followed by malar rash and annular subacute lupus erythematosus (SCLE). Skin induration, microstomia and sclerodactyly were seen in most patients of SSc. Antinuclear antibodies were positive in 89.1% of patients. Anti-dsDNA and anti-Sm antibodies were positive in 62.2% and 33.3% of LE patients, anti-Scl 70 antibody was positive in 68% of SSc patients.</p><p class="abstract"><strong>Conclusions:</strong> CTDs are rare but potentially life-threatening. Proper understanding of the spectrum of cutaneous manifestations of CTDs is therefore necessary for early diagnosis and efficient management.</p>

scholarly journals Complement deficiency in pediatric-onset systemic lupus erythematosus

2018 ◽  
Vol 10 (02) ◽  
pp. 232-236 ◽  
Author(s):  
Parisa Afzali ◽  
Anna Isaeian ◽  
Peyman Sadeghi ◽  
Bobak Moazzami ◽  
Nima Parvaneh ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Musculoskeletal Symptoms ◽  
Complement C3 ◽  
Complement Deficiency ◽  
Control Group ◽  
Systemic Lupus ◽  
Cutaneous Manifestations ◽  
Pediatric Onset ◽  
Onset Systemic Lupus Erythematosus

Abstract BACKGROUND: Pediatric-onset systemic lupus erythematosus (pSLE) accounts for about 10%-20% of all patients with SLE. Deficiencies in early complement components of the classical pathway are the strong genetic risk factor for the development of SLE. In this study, clinical and laboratory manifestations of both complement-deficient and normal complement pSLE patients were compared. MATERIALS AND METHODS: To investigate clinical and immunological manifestations of pSLE in Iran, 36 consecutive pSLE patients (onset before 18 years) who were followed up over a period of 2 years, were studied. Complement C1q and C2 levels were measured using radial immunodifusion assay and complement C3 and C4 levels were measured using nephelometry. Medical records were retrospectively evaluated from patient database of Children Medical Center Hospital. Data were assessed through descriptive analysis (confidence interval = 95%), paired t-test, and Pearson correlation test. RESULTS: Twenty-one patients (58%) had at least one component of complement deficiency. Ten patients (27%) had low C1q level, 11 patients (30.5%) had low C2, nine patients (25%) had low C3, and four patients (11%) had low C4 level. Serum level of complement in pSLE was significantly lower than the control group, except C4 (P = 0.005). The low C1q patients had an earlier age of onset of disease (P < 0.0001). The cutaneous manifestations were more frequent and much more severe in pSLE with low complement (100% vs. 73%). The frequency of renal and musculoskeletal symptoms was equal, but renal morbidity was more common in pSLE with low complement. Positivity for anti-ds-DNA was less common in pSLE with low complement (71% vs. 86%). CONCLUSION: In pSLE patients with early disease onset and more aggressive SLE manifestations and negative anti-ds-DNA test, complement deficiency should be considered.

scholarly journals Cutaneous Manifestations of Zika in Peru

2019 ◽  
Vol 24 (1) ◽  
pp. 33-40
Author(s):  
Willy Ramos ◽  
Miguel Luna ◽  
Tania Alarcón ◽  
Gerardo Jiménez ◽  
Jesús Díaz ◽  
...  
Keyword(s):  
Pregnant Women ◽  
Lupus Erythematosus ◽  
Viral Infections ◽  
Inflammatory Diseases ◽  
Maculopapular Rash ◽  
Medical Advice ◽  
The Body ◽  
Cutaneous Manifestations ◽  
Primary Care Health ◽  
Care Health

This article aims to describe the cutaneous manifestations observed in the Zika epidemic in Peru during 2016 and 2017, as well as discuss the potential differential diagnoses. During the outbreak, the main reason for seeking medical advice was the development of a pruriginous maculopapular rash with a marked papular component, which started on the chest and later generalized to the rest of the body. Similar manifestations were noted in adults, children, and pregnant women. Other manifestations such as conjunctivitis, edema, or petechiae on the palate were rare. We suggest that in areas that are endemic for arboviral infections, in the differential diagnosis of a rash one must consider infections such as dengue, Zika, or chikungunya viruses. In nonendemic areas, the diagnosis is more difficult, as the rash may result from other viral infections not transmitted by arthropods and/or reactive or inflammatory diseases (urticaria, atopic dermatitis, systemic lupus erythematosus). We recommend that primary care health personnel are trained in the recognition of the mucocutaneous lesions caused by Zika virus infection, which could contribute to the identification of suspicious cases, particularly pregnant women.

scholarly journals Interleukin 23 is elevated in the serum of patients with SLE

Lupus ◽  
2020 ◽  
Vol 29 (14) ◽  
pp. 1943-1947 ◽  
Author(s):  
Milena Vukelic ◽  
Anita Laloo ◽  
Vasileios C Kyttaris
Keyword(s):  
Disease Activity ◽  
Lupus Erythematosus ◽  
Standard Of Care ◽  
Systemic Lupus ◽  
Current Standard ◽  
Cutaneous Manifestations ◽  
Autoantibody Profile ◽  
Dsdna Antibody ◽  
Antibody Positivity ◽  
Interleukin 23

Aim: Interleukin-23 (IL-23) is a cytokine that promotes the differentiation of T cells into pro-inflammatory Th17. We have previously shown that IL-23 is upregulated in systemic lupus erythematosus (SLE) patients and lupus prone mice. As SLE is highly heterogeneous, we asked whether IL-23 production correlates with different manifestations of the disease. Methods: We recruited 56 subjects who fulfilled the ACR criteria for SLE. Interleukin-23 was measured in the serum by ELISA. Results: IL-23 levels were positively correlated with the overall SLE disease activity as measured with the SLEDAI. Moreover, IL-23 correlated with the skin, renal domains of SLEDAI and arthritis but not with cytopenias or serositis. IL-23 did also correlate with anti-dsDNA antibody positivity and inversely correlated with C3 levels. We found no relationship between patients’ demographics, prior disease manifestations, medications, or autoantibody profile and IL-23 levels. No immunomodulatory medication seemed to be affecting IL-23 levels suggesting that current medications used in SLE are not as effective in shutting down the IL-23/IL-17 axis. Conclusions: IL-23 levels track SLE disease activity mostly in the renal, skin and musculoskeletal domains. Our data suggest that IL-23 inhibitors may be helpful in combination with current standard of care in alleviating arthritis, renal and cutaneous manifestations of the disease.

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