The association between lupus erythematosus profundus and systemic lupus erythematosus: a single center study and literature review

( Do Ik Kwon ),( Jang Hwan Jung ),( Seol Hwa Seong ),( Kang Hoon Lee ),( Jong Bin Park ),( Kee Suck Suh ),( Min Soo Jang ) 대한피부과학회 2020 대한피부과학회 학술발표대회집 Vol.72 No.1

Background: Lupus erythematosus profundus (LEP) is a variant of chronic cutaneous lupus erythematosus and association between LEP and systemic lupus erythematosus (SLE) has been described as from 10% to 50% in the previous studies. Objectives: To investigate the association of SLE in LEP patients. Methods: This study was conducted on 32 patients diagnosed as LEP in the authors’ hospital and we reviewed previous studies using PubMed searches (Search terms included LEP, lupus erythematosus profundus, lupus erythematosus panniculitis, lupus profundus, lupus panniculitis. Studies describing 3 or more patients with LEP were included). Results: The mean age was 40.8 years (range, 17-73) and the mean follow-up duration was 72.4 months (range, 0- 162). Three patients (9.3%) were diagnosed concurrently SLE with LEP and two patients (6.3%) developed into SLE after 6 to 7 years. In the review of previous studies, 189 patients were histologically confirmed as LEP including 51 patients (26.9%) accompanied by SLE. Of them, 30 patients (15.8%) had been diagnosed as SLE before LEP, and 15 patients (7.9%) were simultaneously diagnosed as SLE by LEP. Only 5 patients (2.6%) developed into SLE during the follow-up period of LEP. Conclusion: The proportion of LEP patients associated with SLE in our center (16.7%) was lower than that of literature review (26.9%). And LEP seems to rarely progresses into the SLE.

개에 있어서 전신성홍반성루프스 1예와 전신성혼반성루프스 의증 1예 및 자가면역성혈소판감소성출혈성자반병 1예

이창우,나기정,임정식,서정욱 한국임상수의학회 1996 한국임상수의학회지 Vol.13 No.1

Systemic lupus erythematosus in a dog, suspected systemic lupus erythematosus in a dog, and autoimmune thrombocytopenic purpura hemmorrhagica in a dog are reported. A fice-year old, female Chihuahua (Case 1) showed initially hemorrhagic diathesis and purpura hemorrhagica. Afterward, it showed polymyositis and polyarthritis. LE-cell was demonstrated on LE-cell preparation trom blood. Systemic lupus erythematosus was diagnosed. This reponded well to the immunosuppressants, but developed iatrogenic Cushing syndrome and steroid hepatopathy. A two-and-half-year old, male toy poodle (Case 2) had chief complaint of red urine. Occult blood test for the urine sediment. This did not respond at all to antibiotics and carbazochrome, which is one of systemic coagulants. LE-cell was demonstrated on LE-cell preparation from blood. This responded relatively well to immunosupressants such as prenisolone, azathioprine and cyclophosphamide. systemic lupus erythematosus is suspected. A nine-year-and-three-month old, female Maltese (Case 3), which had history of congestive heart failure and ovariohysterectomy showed purpura hemorrhagica in the skin of chest. This had severe thrombocytopenia and leukocytosis. As prednisolone was administered before immunological examination or demonstration of LE-cell, it was impossible to diagnose whether purpura hemorrhagica developed as a member of systemic lupus erythematosus or independent of systemic lupus erythematosus. This responded well to prednisolone, and so autoimmune thrombocytopenic purpura hemorrhagica was diagnosed.

Vesiculobullous eruption, the first manifestation of lupus erythematosus

( Su Hyun Park ),( Ki Hwa Choi ),( Ji Yeoun Lee ),( Tae Young Yoon ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Bullous systemic lupus erythematosus is an autoantibodymediated subepidermal blistering disease that occurs in patients with systemic lupus erythematosus. In cases from the literature, the vast majority had a previous history of lupus erythematosus before making the diagnosis of bullous systemic lupus erythematosus. A 46-year-old woman presented with an extensive vesiculobullous eruption on the face, trunk, and both upper extremities for 1 month. She had no other systemic symptoms. Histologic findings showed hydropic degeneration of the basal layer, a subepidermal blister and a predominantly neutrophilic dermal infiltrate. Direct immunofluorescence microscopy for IgG revealed granular bandlike deposition along the basement membrane zone. The patient was also found to have mouth sores, anemia, significant thrombocytopenia, positive antinuclear antibody test, and positive anti-Smith antibody test, confirming the diagnosis of bullous systemic lupus erythematosus. The skin lesions resolved after administrating oral corticosteroids (methylprednisolone 60mg/day) for 2 weeks. Here, we present a case of a 46-year-old woman with bullous systemic lupus erythematosus who presented with vesiculobullous lesions as an initial manifestation of systemic lupus erythematosus.

전신성 홍반성 낭창(SLE)환자 1예(例)에 대한 증례 보고

안창석,강계성,권기록,An, Chang-Suk,Kang, Kye-Seong,Kwon, Gi-Rok 대한약침학회 2000 Journal of pharmacopuncture Vol.3 No.2

After observing a patient diagnosed with Systemic Lupus Erythematosus from the September 21, 2000 to the January 13, 2001, the following results are obtained. Method and Result: We treated one case of Systemic Lupus Erythematosus with the Korean Bee Venom Therapy and the other Oriental Medical treatments. In acupuncture therapy, Korean Bee-venom Therapy is believed to be outstanding way to treat Systemic Lupus Erythematosus. Korean Bee Venom Therapy is treated on the following acupuncture points: ST36(B23(Shinsu:腎兪), BL26(Guanyuanshu:關元兪), ST36(Chok-Samni:足三里), LI4(Hapkok:合谷), LV3(Taechung), SP10(Hyolhae:血海) SP6 (Samumgyo;三陰交). In addition, CFC(Carthami Flos;紅花 and Cervi Pantotrichum Cornu;) Herbal-Acupuncture was applied on the acupucture points of GB20(Pungji: 風池), GB21(Kyonjong:), as well as BUM(It was made of Boviscalculus(牛黃), Moschus and Fel ursi Herbal-Acupuncture was given on CV17(Chonjung). In herbal medication, based on the Sasang Constitution, Taeyumin Chungsimyunja-Tang was given to the patient. As the results of these treatments, general conditions of this patient improved drastically. Conclusion: Based on the clinical results, traditional Korean Medical treatment is believed to be effective for treating Systemic Lupus Erythematosus, and futher studies should be carried out to provide more valuable information.

심한 증식성 루푸스 신염과 혈전성 혈소판 감소성 자반증이 병발한 남성 환자에서 cyclophosphamide 치료 경험

정혜원 ( Hae Won Jung ),신준암 ( Jun Am Shin ),이유지 ( Yu Ji Lee ),강나리 ( Na Ree Kang ),권기영 ( Ghee Young Kwon ),한봉준 ( Bong Jun Han ),김윤구 ( Yoon Goo Kim ) 대한내과학회 2006 대한내과학회지 Vol.71 No.2

Thrombotic thrombocytopenic purpura is a rare but fatal complication of systemic lupus erythematosus. The diagnosis of thrombotic thrombocytopenic purpura as a syndrome distinct from systemic lupus erythematosus may be challenging particularly when thrombotic thrombocytopenic purpura is presented concomitantly with systemic lupus erythematosus. Early diagnosis and aggressive treatment including plasmapheresis would be required. However, recent reports have suggested that the use of cyclophosphamide may have a role. We describe a patient with systemic lupus erythematosus who was first presented with severe thrombotic thrombocytopenic purpura. Diagnosis was based on typical clinical features of thrombotic thrombocytopenic purpura and laboratory findings of active lupus nephritis. Renal biopsy also confirmed the coexistence of thrombotic thrombocytopenic purpura and diffuse proliferative lupus nephritis. Although prompt extensive plasmapheresis and high dose steroid therapy were performed, oliguric renal failure and thrombocytopenia persisted. After addition of cyclophosphamide to the treatment with plasmapheresis and steroid, clinical manifestations of thrombotic thrombocytopenic purpura and lupus nephritis were markedly improved. (Korean J Med 71:214-218, 2006)

Lupus cystitis in Korean patients with systemic lupus erythematosus: risk factors and clinical outcomes

Koh, J H,Lee, J,Jung, S M,Ju, J H,Park, S-H,Kim, H-Y,Kwok, S-K SAGE Publications 2015 Lupus Vol.24 No.12

<P>This study was performed to investigate the clinical characteristics of lupus cystitis and determine the risk factors and clinical outcomes of lupus cystitis in patients with systemic lupus erythematosus (SLE). We retrospectively reviewed 1064 patients at Seoul St. Mary’s Hospital in Seoul, Korea, from 1998 to 2013. Twenty-four patients had lupus cystitis. Lupus cystitis was defined as unexplained ureteritis and/or cystitis as detected by imaging studies, cystoscopy, or bladder histopathology without urinary microorganisms or stones. Three-fourths of patients with lupus cystitis had concurrent lupus mesenteric vasculitis (LMV). The initial symptoms were gastrointestinal in nature for most patients (79.2%). High-dose methylprednisolone was initially administered to most patients (91.7%) with lupus cystitis. Two patients (8.3%) died of urinary tract infections. Sixty-five age- and sex-matched patients with SLE who were admitted with other manifestations were included as the control group. Patients with lupus cystitis showed a lower C3 level (<I>p</I> = 0.031), higher SLE Disease Activity Index score (<I>p</I> = 0.006), and higher ESR (<I>p</I> = 0.05) upon admission; more frequently had a history of LMV prior to admission (<I>p</I> < 0.001); and less frequently had a history of neuropsychiatric lupus (<I>p</I> = 0.031) than did patients with SLE but without lupus cystitis. The occurrence of lupus cystitis was associated with a history of LMV (OR, 21.794; 95% CI, 4.061–116.963). The median follow-up period was 3.4 years, and the cumulative one-year mortality rate was 20%. Complications developed in 33.3% of patients with lupus cystitis and were related to survival (log-rank <I>p</I> = 0.021). Our results suggest that the possibility of lupus cystitis should be considered when a patient with SLE and history of LMV presents with gastrointestinal symptoms or lower urinary tract symptoms. Development of complications in patients with lupus cystitis can be fatal. Thus, intensive treatment and follow-up are needed, especially in the presence of complications.</P>

심근경색증으로 처음 임상발현된 전신성 홍반성 낭창 1 예

김주영(Ju Young Kim),송재관(Jae Kwan Song),이근찬(Geun Chan Lee),두영철(Young Cheoul Doo),김원호(Won Ho Kim),김재중(Jae Joong Kim),박성욱(Seong Wook Park),문희범(Hee Bom Moon),이종구(Jong Koo Lee) 대한내과학회 1994 대한내과학회지 Vol.47 No.1

Systemic lupus erythematosus is a chronic inflammatory disease of unknown cause, characterized by appearance of variable autoantibodies and multiple organ involvement. Cardiac involvement in systemic lupus erythematosus is well recognized and most frequently manifested as pericarditis, myocarditis, and endocarditis, but myocardial infarction during the course of systemic lupus erythematosus is rare and only a few cases have been reported. In this case report, we present a 20-years old young male patients with acute myocardial infarction and active systemic lupus erythematosus. A young male without any past medical history visited emergency room due to sudden anterior chest pain lasting several hours, whose electrocardiography revealed right bundle branch block and myocardial ischemia pattern. Echocardiography showed regional wall motion abnormality suggestive of ischemic insult of left anterior desending artery territory. Total obstruction of distal left anterior descending artery was demonstrated in coronary angiography. The patient described here meets four of the revised criteria for systemic lupus erythematosus: pericardiopleuritis, positive antinuclear anti- body, positive anti-ds-DNA antibodies, and thrombocytopenia. Screening for systemic vasculitis or other connective tissue disease seems to be necessary for etiologic diagnosis of acute myocardial infarction especially in cases without any major risk factors for atherosclerosis.

Self-Reported Physical Activity Is Associated with Lupus Nephritis in Systemic Lupus Erythematosus: Data from KORean Lupus Network (KORNET) Registry

김성규,최정윤,이신석 연세대학교의과대학 2018 Yonsei medical journal Vol.59 No.7

Purpose: The aim of this study was to identify the associations among physical activity, disease activity, and organ damage in patientswith systemic lupus erythematosus (SLE). Materials and Methods: A total of 415 patients with SLE were consecutively enrolled from the KORean lupus Network (KORNET)registry. This registry assessed clinical features, disease activity [Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K)], and organ damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI)] upon enrollment in the study. Self-reported physical activity was measured by the International PhysicalActivity Questionnaire. Statistical analyses were conducted using the Mann-Whitney U test and multivariate logistic regressionanalysis. Results: A significant difference in vigorous activity was noted between patients with lupus nephritis (LN) (n=93) and those withoutLN (n=322) (p=0.012), but not in moderate and walking activities. In contrast, no differences in physical activity, walking,moderate, and vigorous intensity, according to SLEDAI-2K and SDI were found. In addition to younger age (p=0.032), high physicalcomponent summary of SF-36 (p=0.004) and SLEDAI-2K (p=0.038), and less vigorous physical activity were associated withLN (p=0.024). However, cardiovascular disease was not associated with physical activity in SLE patients. Conclusion: This study showed that patients with LN had less vigorous physical activity than patients without LN. The results suggestthat lupus nephritis might be associated with physical activity.

전신홍반루푸스 치료 중 발생한 루푸스 장염

정병수 ( Byoung Soo Chung ),김민성 ( Min Sung Kim ),나찬호 ( Chan Ho Na ),신봉석 ( Bong Seok Shin ) 대한피부과학회 2009 대한피부과학회지 Vol.47 No.8

Systemic lupus erythematosus is a multisystemic autoimmune inflammatory disease with a variety of presenting features and manifestations. Gastrointestinal complications are common in patients with systemic lupus erythematosus, and lupus enteritis is a major cause of acute abdominal pain in the patients suffering with systemic lupus erythematosus. We report here on a 41-year-old-woman with systemic lupus erythematosus and who had developed an acute abdomen during treatment with systemic glucocorticoid and antimalarial drugs. Lupus enteritis was strongly suspected according to the clinical manifestations and the findings of the abdominal computerized tomography scan. She recovered after being treated with intravenous high dose glucocorticoid and conservative therapy. (Korean J Dermatol 2009;47(8):956~959)

Clinical characteristics of pediatric cutaneous lupus erythematosus: experience from a tertiary referral center in Korea

( Shinyoung Song ),( Seha Park ),( Seulki Lee ),( Jin Ok Baek ),( Joo Young Roh ),( Hyangjoon Park ),( Hee Joo Kim ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.2

Background: The clinical characteristics of cutaneous lupus erythematosus (CLE) are well delineated in adults, but pediatric data, particularly in Asian populations, are limited. Objectives: Therefore, we evaluated the characteristics of pediatric cases by retrospectively reviewing the medical records of children with CLE during a 15-year period in a tertiary care dermatology clinic in South Korea. Methods: The study included 21 children (8 males and 13 females), 4 of whom had neonatal lupus erythematosus (NLE). Results: Among 17 patients with CLE, discoid lupus erythematosus (DLE) was most common (47.1%), followed by acute CLE (ACLE, 35.3%). All ACLE cases had systemic lupus erythematosus (SLE). Female predominance was conspicuous in ACLE/SLE (6/11 females versus 0/6 males), as was older age, whereas DLE and NLE showed near equal sex distributions. The median age at the diagnosis of CLE was significantly higher in females than in males (15 years versus 4.5 years, p=0.02). All patients with ACLE/ SLE simultaneously showed skin and systemic symptoms from onset. The kidney was the most commonly involved organ. Conclusion: This study revealed unique characteristics of pediatric CLE, further warranting a comprehensive review among various ethnicities to understand the wide spectrum of CLE in the pediatric population.