scholarly journals Case Report: The First Reported Case of Bullous Lichen Planus-Systemic Lupus Erythematosus Overlap Syndrome

2021 ◽  
Vol 8 ◽  
Author(s):  
Yi Liu ◽  
Xuelei Liang ◽  
Haixuan Wu ◽  
Fenglin Zhuo
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lichen Planus ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Immunosuppressive Drugs ◽  
Overlap Syndrome ◽  
Systemic Lupus ◽  
Delayed Treatment ◽  
Multiple Biopsies

Introduction: Lichen planus/lupus erythematosus overlap syndrome is rarely seen in the clinic and has the characteristic clinical manifestations, histopathology, and immunopathology of lichen planus (LP) and lupus erythematosus (LE). This is the first reported case of bullous lichen planus (BLP)/systemic lupus erythematosus (SLE) overlap syndrome with hair loss as the first symptom.Case Presentation: A 48-year-old female presented with alopecia for half a year, and skin lesions accompanied by itching on her face, trunk, and limbs for 3 months. She had a history suggestive of photosensitivity. Laboratory tests and histopathology were performed for diagnosis. Histopathologic features of the upper arm and back of the hand were consistent with BLP, whereas the scalp lesion indicated LE. Laboratory examination indicated positive for antinuclear antibody (ANA) (1:160), leukopenia, increased urinary protein, decreased C3/C4, and normal BP180. The patient was given glucocorticoid combined with acitretin and immunosuppressive therapy after a definite diagnosis of BLP/SLE overlap syndrome. The lesions of the patient disappeared and some hair had regrown during the two years of follow-up.Conclusion: This is the first reported case of BLP/SLE overlap syndrome which responded well to glucocorticoids, retinoids, and immunosuppressive drugs. Multiple biopsies from characteristic lesions will guide doctors to avoid misdiagnoses and delayed treatment.

scholarly journals Secondary angle-closure glaucoma as an ocular presentation of systemic lupus erythematosus: a case report

2020 ◽  
Vol 48 (9) ◽  
pp. 030006052095949
Author(s):  
Xincen Hou ◽  
Wenping Pan ◽  
Anli Wang ◽  
Tao Yu ◽  
Aiping Song
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Immunosuppressive Drugs ◽  
Acute Angle ◽  
Angle Closure Glaucoma ◽  
Angle Closure ◽  
High Dose ◽  
Systemic Lupus

Systemic lupus erythematosus (SLE) is a chronic idiopathic autoimmune disease. SLE can involve almost any part of the eyes. However, bilateral angle-closure glaucoma due to lupus choroidopathy that is accompanied by polyserositis and nephropathy is rare. We report a 21-year-old woman whose clinical manifestations were diagnosed as bilateral angle-closure glaucoma caused by ciliochoroidal effusion. Subsequently, SLE and lupus nephritis were diagnosed on the basis of malar rash, photosensitivity, proteinuria, positive anti-Smith and anti-DNA antibodies, and a renal histopathological biopsy. After 1 month of treatment with steroids and immunosuppressive drugs, the patient’s intraocular pressure returned to normal, visual acuity improved, and lupus nephritis was effectively controlled. Bilateral secondary acute angle closure caused by SLE choroidal disease can be an ocular manifestation of SLE, and is usually accompanied by polyserositis and nephropathy. High-dose steroids and immunosuppressive therapy should be immediately and actively provided for this condition.

scholarly journals Characteristics of systemic lupus erythematosus and lupus nephritis in children in the Republic of Belarus and the Republic of Tatarstan

2019 ◽  
Vol 64 (5) ◽  
pp. 199-203
Author(s):  
T. P. Makarova ◽  
A. V. Sukalo ◽  
I. A. Kazyro ◽  
Yu. S. Melnikova ◽  
N. N. Firsova ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Immunosuppressive Drugs ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
Severe Manifestation ◽  
Extrarenal Manifestations ◽  
The Republic

Systemic lupus erythematosus is an autoimmune disease characterized by a pronounced polymorphism of clinical manifestations. Lupus nephritis is the most severe manifestation of the disease. The article presents a retrospective analysis of the cases of systemic lupus erythematosus and assessment of the clinical manifestations of the disease and variants of lupus nephritis in children in the Republics of Belarus and Tatarstan. The authors analyzed 60 cases of systemic lupus erythematosus, lupus nephritis. All patients had at least 4 of the 11 diagnostic criteria proposed by the American College of rheumatology (ACR, 1997), and 35 patients had a morphologically verified nephritis. It was found that the disease in children developed very actively with fast multi-organ involvement and it required aggressive therapy with several immunosuppressive drugs. During follow-up, the percentage of patients with renal damage increased, so renal function should be controlled in all patients with systemic lupus erythematosus, especially with early onset. Lupus nephritis is combined with extrarenal manifestations and it is difficult to diagnose when it begins with kidney damage. The overall survival rate of children with systemic lupus erythematosus is closely related to the severity of renal manifestations. Lupus nephritis is a serious problem that requires early aggressive intervention and continuous maintenance therapy.

Młodzieńczy toczeń rumieniowaty układowy – opis przypadku

2019 ◽  
Vol 22 (1) ◽  
Author(s):  
Katarzyna Odzimkowska-Łata ◽  
Aleksandra Rybkowska ◽  
Edyta Olesińska
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Unknown Etiology ◽  
Systemic Lupus ◽  
Juvenile Systemic Lupus Erythematosus ◽  
Mild Disease ◽  
Pediatric Diseases ◽  
Treatment Knowledge

Juvenile systemic lupus erythematosus is an autoimmune, chronic, multisystemic inflammatory disease with an unknown etiology. The average age of juvenile systemic lupus erythematosus (jSLE) diagnosis is 11-14 years old, with a significant female predominance. Clinical manifestations of JSLE are extremely variable, from a relatively mild disease characterised by facial rash, joint pains, fever, fatigue, weight loss, alopecia and arthralgias to a severe life threatening illness.These and other symptoms of diffuse generalized inflammation including lymphadenopathy and hepatosplenomegaly occur both at onset and during disease flares.The authors present the case of a girl hospitalized in the Rheumatology Clinic due to fevers, skin lesions and haematological disorders. The diagnostic difficulty may have been the fact that the symptoms appear also in the course of many pediatric diseases. Finally, based on an in-depth medical history, physical examination and the results of immunological tests, they were recognized at the outset. Juvenile systemic lupus erythematosus is a disease with a diverse clinical picture, therefore, early diagnosis is not easy, but it is necessary to implement effective treatment. Knowledge and experience of the spectrum of paediatric and adolescent disease is important as well as recognition of when features merit further investigation.

scholarly journals Disseminated cryptococcosis presenting as cutaneous cellulitis in an adolescent with systemic lupus erythematosus

2015 ◽  
Vol 7 (2) ◽  
Author(s):  
Ellen Simionato Valente ◽  
Mauricio Costa Lazzarin ◽  
Bruno Lopes Koech ◽  
Ralph Vighi da Rosa ◽  
Rafael De Almeida ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Complete Remission ◽  
Fungal Infection ◽  
Lupus Erythematosus ◽  
Skin Lesions ◽  
Immunosuppressive Drugs ◽  
Systemic Lupus ◽  
Cryptococcal Infection ◽  
Chronic User ◽  
Appropriate Therapy

We report here the case of a 17-year-old girl from Pelotas, Brazil, with systemic lupus erythematosus and disseminated cryptococcal infection. Prior to diagnosis, she was a chronic user of corticosteroids and other immunosuppressive drugs. Her first symptoms were skin lesions that simulated bacterial cellulitis. Upon suspicion, we performed a biopsy and fungal infection was confirmed. Appropriate therapy was established, and the patient was discharged after 42 days of treatment in complete remission.

scholarly journals POS0780 SKIN LESIONS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND ANTIPHOSPHOLIPID SYNDROME

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 643.2-644
Author(s):  
A. Shumilova ◽  
F. Cheldieva ◽  
T. Reshetnyak ◽  
A. Lila
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Past History ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Response To Therapy ◽  
Systemic Lupus ◽  
Skin Manifestations ◽  
Increased Risk

Background:In more than 40% of cases, systemic lupus erythematosus (SLE) is associated with the presence of highly positive antiphospholipid antibodies, with 50-70% of patients developing antiphospholipid syndrome (APS) over the next 10 years of the disease. Both diseases have similar and different clinical manifestations of skin lesions. The variety of skin lesions in SLE and APS requires a differential diagnosis and can make it difficult to diagnose a systemic autoimmune disease in a timely manner.Objectives:To study the frequency of skin manifestations in SLE and APS, depending on the positivity of aPL.Methods:The study included 116 patients with SLE (104 women and 12 men), mean age 37.9±12.9, disease duration 8.5 [1.15-13.0]; 40 patients with APS (33 women and 7 men), mean age 36.2±9.39. All patients were evaluated for skin lesions, and patients with APS were determined by IgG/IgM-aCL and IgG/IgM-aß2HP1 by enzyme immunoassay (ELISA), 19 of them were determined by IgA-aCL, IgA-aß2HP1 and IgG-aß2HP1-D1 chemiluminescence analysis (CMA).Results:Acute skin lesions in past history were noted in 58 (50%) patients, chronic lesions -I n 21 (18.1%) patients; photosensivity and alopecia were indicated in 46 (39.6%) patients, mucosal lesions were noted in 36 (31%) of 116 patients, which corresponds to the literature data on the frequency of skin lesions and its appendages in SLE. At the time of inclusion in the study, skin lesions were detected in 20 patients. Score according to the CLASI index in patients with skin lesions: activity index=1.55 [0-22]; damage index=1.81 [0-36].Skin lesions are the second most common signs of SLE onset (debut in 26 (22.4%) patients), second only to arthritis (38 (32.5%) patients), while the detection of immunological disorders (highly positive ab to dsDNA) was observed in 7 patients (6%) with reliable APS and probable SLE, who may not have had time to develop a clinic for reliable SLE.Livedo, as one of the most frequent skin manifestations of APS, was detected in 60 patients and was significantly associated with IgM-aCL and IgM-aß2HP1 positivity (p<0.0001). Significantly, positivity for IgG-aCL, IgG-aß2HP1 and IgG-aß2HP1-D1 (p=0.0001) and IgA-aCL (p=0.008) was associated with the development of comminuted hemorrhages, which occurred in 7 patients with APS and was associated with positivity of IgG-aCL, IgG-aß2HP1, IgG-aß2HP1-D1 (p=0.0001) and IgA-aCL (p=0.008).The development of ulcerative-necrotic vasculitis with deep skin necrosis was observed in 3 patients, 2 of them were highly positive for IgG-aCL, IgG-aß2HP1, IgG-aß2HP1-D1. Melanoma was detected in the past history in 2 patients with highly positive for IgG-aCL, which is not a manifestation of the underlying disease, but confirms an increased risk of malignancy with aPL-positivity.Conclusion:More than half of the patients had acute skin lesions, and about a quarter of the cases had skin lesions at the onset of the disease. The presence of comminuted hemorrhages was associated with positivity of IgG-aCL, IgG-aß2HP1, IgG-aß2HP1-D1) and IgA-aCL. Assessment of skin activity and damage (in particular, according to the CLASI index) is necessary for a comprehensive analysis of the dynamics of the disease and the response to therapy. The detection of aPL is necessary not only for the purpose of predicting thrombotic catastrophes, but also for the development of skin manifestations of APS.Disclosure of Interests:None declared

scholarly journals Netosis in the pathogenesis of antiphospholipid syndrome and systemic lupus erythematosus

2021 ◽  
Vol 15 (5) ◽  
pp. 96-102
Author(s):  
K. S. Nurbaeva ◽  
T. M. Reshetnyak ◽  
A. M. Lila
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Space Form ◽  
Clinical Manifestations ◽  
Immunosuppressive Drugs ◽  
Systemic Lupus ◽  
Extracellular Traps ◽  
Activated Neutrophils

Antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) are autoimmune diseases. In recent years, APS has been considered as an autoimmune thrombo-inflammatory disease. It has been established that clinical manifestations of APS can persist, progress over time, or debut during an adequate anticoagulant therapy and, in some cases, require administration of immunosuppressive drugs, which indicates the role of autoimmune inflammation in their development. The formation of extracellular neutrophil traps (neutrophil extracellular traps, NETs) is one of the connecting links of inflammation and thrombosis. Netosis is the process by which activated neutrophils in the extracellular space form netlike structures (NETs). This review examines the role of neutrophils and netosis in the pathogenesis of APS and SLE.

Systemic lupus erythematosus children in Egypt: Homeland spectrum amid the global situation

Lupus ◽  
2021 ◽  
pp. 096120332110430
Author(s):  
Nahla N Eesa ◽  
Hend Abdel Nabi ◽  
Rasha El Owaidy ◽  
Iman Khalifa ◽  
Ahmed R Radwan ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Disease Onset ◽  
Damage Index ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Systemic Lupus ◽  
Disease Characteristics ◽  
Egyptian Children

Objectives This study aims to present the manifestations of juvenile systemic lupus erythematosus (JSLE) across Egypt, to focus on age at onset and gender-driven influence on disease characteristics, and to compare findings to other countries. Methods The study included 404 Egyptian children with systemic lupus erythematosus (SLE) presenting to one of the specialized rheumatology centers corresponding to 13 major governorates. Juvenile cases age was ≤ 16°years at the time of recruitment. The SLE Disease Activity Index (SLEDAI) and damage index (DI) were assessed. Results The mean age was 13.2 ± 2.4°years; 355 females and 49 males (7.2:1), and the disease duration was 2.3 ± 1.6 years, while age at disease onset was 11.1 ± 2.5°years. Their SLEDAI was 13.5 ± 12.3, and DI, 0.36 ± 0.78. The overall estimated prevalence of childhood-SLE patients in the recruited cohort in Egypt was 1/100,000 population (0.24/100000 males and 1.8/100000 females). 7.4% developed pre-pubertal SLE (≤ 7 years); 73.3%, peri-pubertal; and 19.3% during early adolescence. The differences according to age group were equal for gender and clinical manifestations except skin lesions present in 59.3% of pre-pubertal onset, 74.6% of peri-pubertal, and 84.2% of adolescents ( p = 0.029), and renal involvement in 73.8% of peripubertal, 62.1% of pre-pubertal and 58.9% of adolescents ( p = 0.03). Laboratory investigations, SLEDAI, and DI were similar among age categories. Lupus nephritis was more common in Egypt compared to JSLE from other countries. Conclusion Our large multicenter study identified that female gender influenced disease characteristics with more frequent skin involvement. Skin lesions were significantly higher in adolescents, while renal involvement in peri-pubertal children.

Diagnostic algorithm for Raynaud’s phenomenon and vascular skin lesions in systemic lupus erythematosus

Lupus ◽  
2010 ◽  
Vol 19 (9) ◽  
pp. 1087-1095 ◽  
Author(s):  
JG Richter ◽  
O. Sander ◽  
M. Schneider ◽  
P. Klein-Weigel
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Correct Diagnosis ◽  
Clinical Manifestations ◽  
Diagnostic Algorithm ◽  
Skin Lesions ◽  
Raynaud's Phenomenon ◽  
Systemic Lupus

Skin discolorations and skin lesions due to vascular pathologies are common clinical features in systemic lupus erythematosus. A variety of clinical manifestations such as Raynaud’s phenomenon, acrocyanosis, livedo patterns, erythematous or violaceous macules and papules or necrosis are triggered by heterogeneous pathophysiological mechanisms such as vasospasm, vasculitis or thromboembolism. A standardized macro- and microvascular assessment is necessary to establish the correct diagnosis. We describe and illustrate common clinical features of vascular skin manifestations in systemic lupus erythematosus and present a diagnostic algorithm. Lupus (2010) 19, 1087—1095.

scholarly journals Vitamin D Status in Patients with Systemic Lupus Erythematosus in Serbia: Correlation with Disease Activity and Clinical Manifestations

2015 ◽  
Vol 3 (2) ◽  
pp. 256-261 ◽  
Author(s):  
Rada Miskovic ◽  
Aleksandra Plavsic ◽  
Sanvila Raskovic ◽  
Zikica Jovicic ◽  
Jasna Bolpacic
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Vitamin D ◽  
Disease Activity ◽  
Vitamin D Deficiency ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Vitamin D Status ◽  
Systemic Lupus ◽  
The Impact

BACKGROUND: Numerous studies indicate potential role of vitamin D as an important factor in the development of many autoimmune diseases including systemic lupus erythematosus (SLE). Patients with SLE are especially prone to the development of vitamin D deficiency due to the nature of their illness.AIM: The aims of our study were to determine the prevalence of vitamin D insufficiency and deficiency in patients with SLE in Serbia, to identify clinical variables associated with vitamin D status and to examine the impact of vitamin D status on disease activity and presence of specific lupus autoantibodies.MATERIAL AND METHODS: The study included 46 patients with SLE. Serum 25(OH)D concentration was measured by electrohemiluminiscent immunoassay.RESULTS: The mean serum concentration of 25(OH)D was 11.9 ± 7.3 ng/ml. The prevalence of insufficiency was 32.6%, while the prevalence of deficiency was 67.4%. There was no association between vitamin D status and photosensitivity, skin lesions, arthritis and lupus nephritis. Vitamin D status was not associated with the presence of specific autoantibodies. There was no correlation between disease activity assessed by SLEDAI scale with the concentration of 25(OH)D. Patients who used vitamin D supplements and calcium did not have a significantly higher concentration of 25(OH)D.CONCLUSION: In conclusion, vitamin D deficiency is common in patients with SLE.

scholarly journals Cutaneous Manifestations of Systemic Lupus Erythematosus

2012 ◽  
Vol 2012 ◽  
pp. 1-15 ◽  
Author(s):  
Luís Uva ◽  
Diana Miguel ◽  
Catarina Pinheiro ◽  
João Pedro Freitas ◽  
Manuel Marques Gomes ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Unknown Etiology ◽  
Systemic Lupus ◽  
Cutaneous Lesions ◽  
Optimal Care ◽  
Cutaneous Manifestations ◽  
American College Of Rheumatology

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.

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