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 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.

NEUROPSYCHIC POLYMORPHISM OF JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS

2021 ◽  
Vol 7 (3) ◽  
pp. 28-34
Author(s):  
Yu. Minakova ◽  
M. Silenko ◽  
O. Ivanova
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Nervous System ◽  
Lupus Erythematosus ◽  
Neuropsychiatric Symptoms ◽  
Clinical Manifestations ◽  
Pathogenetic Mechanism ◽  
Systemic Lupus ◽  
Juvenile Systemic Lupus Erythematosus ◽  
Wide Range ◽  
Prognostic Criterion

Damage to the nervous system (neurolupus) is one of the most common clinical manifestations of systemic lupus erythematosus (SLE) in childhood, and is also considered as an unfavorable prognostic criterion for the course of this disease. Neurolupus is characterized by a wide range of clinical manifestations in both children and adult patients, which is due in most cases to a common pathogenetic mechanism - the formation of systemic microvasculitis. The non-specificity and variability of neuropsychiatric symptoms, which may appear already at the onset of the disease, significantly complicate the early diagnosis of SLE and necessitate a close acquaintance of the pediatrician with neurolupus polymorphism in children.

scholarly journals Systemic Lupus Erythematosus with Unusual Manifestations

2020 ◽  
Vol 17 (6) ◽  
pp. 43-53
Author(s):  
Lorena Manea ◽  
Cătălin Mihai Popescu ◽  
Raluca Popescu ◽  
Daniela Adriana Ion ◽  
Andreea Alexandra Nicola ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Nasal Septum ◽  
Disease Patient ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Septal Perforation ◽  
Systemic Lupus ◽  
Extended Defect ◽  
Nasal Septal Perforation

Abstract Systemic lupus erythematosus (SLE) is a chronic autoimmune disease of unknown etiology, presenting with variable clinical picture. Having a high heterogeneity and lacking pathognomonic features, very often the diagnosis poses a great challenge for the clinician. Several unusual clinical manifestations such as nasal septal perforation and digital gangrene can occur in LES patients. Case report. We report the case of a 42-year-old woman, known with SLE, hospitalized in our department for a clinical presentation consisting of a recent major epistaxis, physical asthenia and acral necrosis of the upper limbs. Physical examination revealed an afebrile patient, with a cushingoid facies, facial telangiectasias, and necrotic scars localized on the distal phalanges, bilaterally. A diagnostic nasal endoscopy showed a large septal perforation with the absence of the cartilaginous nasal septum. CT highlighted an extended defect at the level of the cartilaginous part of the nasal septum. Conclusion. Nasal septal perforation remains an underdiagnosed invalidating complication of lupus and treated and discovered early could have an important impact on the general health of an already burdened by disease patient.

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 Case of a Сombination of Lupus Erythematosus, Antiphospholipid Syndrome and Myocardial Infarction

Kardiologiia ◽  
2019 ◽  
Vol 59 (12) ◽  
pp. 92-96
Author(s):  
N. A. Kosheleva ◽  
N. M. Nikitina ◽  
E. U. Andreeva
Keyword(s):  
Myocardial Infarction ◽  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
The Body ◽  
Systemic Autoimmune Disease ◽  
Unknown Etiology ◽  
Systemic Lupus ◽  
Wide Range

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology characterized by a wide range of clinical manifestations with damage to various organs and systems of the body. There are bad prognostic factors for SLE: damage to the heart, kidney, central nervous system, the development of hematological crises and secondary antiphospholipid syndrome. A number of authors consider systemic lupus erythematosus a “new” risk factor for atherosclerosis. The overall risk of myocardial infarction (MI) in patients with SLE is 10 times higher than in the general population. The article presents clinical case report of the development of myocardial infarction in a woman with SLE, receiving therapy for secondary antiphospholipid syndrome.

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

Systemic lupus erythematosus and related disorders

2020 ◽  
pp. 4499-4513
Author(s):  
Anisur Rahman ◽  
David A. Isenberg
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immune System ◽  
Lupus Erythematosus ◽  
Severe Disease ◽  
Skin Lesions ◽  
The Body ◽  
Rheumatic Disorder ◽  
Systemic Lupus ◽  
Mild Disease ◽  
The Complement System

Systemic lupus erythematosus is an autoimmune rheumatic disorder that can present with symptoms in almost any organ or system of the body. It is 10 times commoner in women than men, and commoner in Afro-Caribbeans than in other ethnic groups. Its aetiology is multifactorial, incorporating genetic, hormonal, and environmental elements. No single abnormality of the immune system can be considered responsible, pathogenesis depending on the interplay of several different factors, including autoantibodies, T lymphocytes, cytokines, the complement system, and apoptosis. Common symptoms are constitutional (fatigue, anorexia), musculoskeletal (arthralgia/arthritis, myalgia), dermatological (alopecia, butterfly rash, vasculitic skin lesions, purpura), cardiopulmonary (breathlessness, pleurisy), and neurological (migraine, seizures, depression, psychosis). Treatment for mild disease is NSAID, analgesics and hydroxychloroquine, more severe disease requires corticosteroid and immunosuppressant drugs.

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.

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