• 제목/요약/키워드: Systemic vasculitis

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소아에서 출혈성 수포를 동반한 $Henoch-Sch{\"{o}}nlein$ 자반병 1례 (A Case of $Henoch-Sch{\"{o}}nlein$ Purpura with Hemorrhagic Bullae in a Child)

  • 오연미;정미림;최혜정;차희정;정진영
    • Childhood Kidney Diseases
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    • 제10권1호
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    • pp.40-44
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    • 2006
  • [ $Henoch-Sch{\"{o}}nlein$ ] 자반증은 소혈관의 혈관염으로 오는 전신적 혈관 장애로 자반, 위장 증세, 관절 증세, 신 증세를 주 증세로 하는 질환이다. HSP의 흔한 피부증상은 주로 궁둥이와 하지의 자반 증상이며 성인에서는 출혈성 수포를 동반하는 경우가 흔하나 소아에서는 드문 것으로 알려져 있다. 저자들은 관절통과 복통을 호소하는 환아에서 출혈성 수포가 동반되어 있어 피부조직 검사를 하였고 진피의 leukocytoclastic vasculitis와 면역 형광법상 소혈관의 IgA와 fibrinogen의 침착을 관찰하였다. HSP로 진단하여 스테로이드 치료를 하였고 수일 내로 관절통과 복통이 호전되어 문헌고찰과 함께 보고하는 바이다.

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A Case Report of Sweet's Syndrome with Parotitis

  • Jo, Myoung-Soo;Lim, Young-Bin;Shin, Hea-Kyeong;Choe, Joon;Seul, Jung-Hyun;Jang, Tae-Jung
    • Archives of Plastic Surgery
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    • 제39권1호
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    • pp.59-62
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    • 2012
  • Sweet's syndrome is characterized by clinical symptoms, physical features, and pathologic findings which include fever, neutrophilia, tender erythematous skin lesions, and a diffuse infiltrate of mature neutrophils. This is a report of our experience of Sweet's syndrome with parotitis. A 57-year-old man initially presented with tender swelling on the right cheek similar to parotitis. His symptoms relapsed despite the use of an oral antibiotic agent for 3 weeks. He additionally presented with erythematous papules and plaques on the periocular area and dorsum of both hands. Histiopathologic findings on punch biopsy of the right dorsum of the hand showed superficial perivenular histiocytic infiltration without vasculitis. We confirmed this as histiocytoid Sweet's syndrome and used systemic corticosteroid. After initiation of treatment with systemic corticosteroids, there was a prompt recovery from both the dermatosis-releated symptoms and skin lesions. Sweet's syndrome should be considered in patients with therapy-refractory parotitis and unclear infiltrated nodules. We present a confusing case who initially appeared to have parotitis but turned out to have histiocytoid Sweet's syndrome.

가와사끼병에 동반된 Gas Forming Enterocolitis 1례 (A Case of Gas Forming Enterocolitis Associated with Kawasaki Disease)

  • 김지영;양태진;송민섭;김철호
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제4권2호
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    • pp.233-237
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    • 2001
  • 저자들은 발열, 구토 및 설사를 주소로 내원한 14개월된 여아의 대변배양검사에서 Aeromonas hydrophilia가 배양된 gas forming enterocolitis를 동반한 가와사끼병 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

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가와사끼병의 심혈관계 합병증 및 치료 (Cardiovascular complications after Kawasaki disease and its management)

  • 장기영
    • Clinical and Experimental Pediatrics
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    • 제51권5호
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    • pp.462-467
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    • 2008
  • Kawasaki disease is a systemic vasculitis of unknown etiology, usually occurring in infants and young children. Although the etiology of Kawasaki disease remains uncertain, its serious complicationssuch as giant aneurysm formation, coronary arterial stenotic lesions, and thrombotic occlusionhave been proven to cause myocardial ischemia or infarction in patients with Kawasaki disease. To prevent and treat these complications, several modes of therapyincluding long-term anticoagulation, interventional catheterization, and surgical treatmenthave been gradually developed. In this article, we review the cardiovascular complications following Kawasaki disease and the management thereof, which includes thrombolytic therapy, catheter intervention, and coronary artery bypass graft.

Update of genetic susceptibility in patients with Kawasaki disease

  • Yoon, Kyung Lim
    • Clinical and Experimental Pediatrics
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    • 제58권3호
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    • pp.84-88
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    • 2015
  • Kawasaki disease (KD) is an acute systemic vasculitis that predominantly affects children, and can result in coronary artery lesions (CAL). A patient with KD who is resistant to treatment with intravenous immunoglobulin (IVIG) has a higher risk of developing CAL. Incomplete KD has increased in prevalence in recent years, and is another risk factor for the development of CAL. Although the pathogenesis of KD remains unclear, there has been increasing evidence for the role of genetic susceptibility to the disease since it was discovered in 1967. We retrospectively reviewed previous genetic research for known susceptibility genes in the pathogenesis of KD, IVIG resistance, and the development of CAL. This review revealed numerous potential susceptibility genes including genetic polymorphisms of ITPKC, CASP3, the transforming growth factor-${\beta}$ signaling pathway, B lymphoid tyrosine kinase, FCGR2A, KCNN2, and other genes, an imbalance of Th17/Treg, and a range of suggested future treatment options. The results of genetic research may improve our understanding of the pathogenesis of KD, and aid in the discovery of new treatment modalities for high-risk patients with KD.

정맥투여용 면역글로불린에 반응한 심한 복통을 가진 $Henoch-Sch\"{o}nlein$ 자반증 3례 (IV-gamma Globulin Therapy for Severe Abdomlnal Pain Refractory to Steroid Therapy in $Henoch-Sch\"{o}nlein$ Purpura)

  • 허영옥;이창연
    • Childhood Kidney Diseases
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    • 제1권2호
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    • pp.176-178
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    • 1997
  • Henoch-Scholein purpura is a systemic vasculitis of small blood vessels. It is characterized by nonthrombocytopenic purpura, abdominal pain, arthralgia and renal involvement. In Henoch-Scholein purpura, severe abdominal pain may be relived by steroid, but occasionally unresponsive to steroid and conventional analgesics therapy. We tried IV-gamma globulin for severe abdominal pain, unresponsive to steroid, analgesics, and antispasmodics therapy in HSP, and experienced dramatic symptomatic improvement. So we report three cases of IVIG therapy against severe abdominal pain in HSP with a brief review of literatures

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$Henoch-Sch{\ddot{o}}lein$ Purpura 환자 치험 2례 (A Clinical Review of $Henoch-Sch{\ddot{o}}lein$ Purpura - two cases)

  • 연경진;노석선;김창훈
    • 한방안이비인후피부과학회지
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    • 제18권3호
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    • pp.127-134
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    • 2005
  • The Henoch-Schnlein Purpura (HSP) is a systemic small vasculitis, characterized by palpable purpura, abdominal pain, hematuria or proteinuria, and arthritis. The etiology is unknown but it is manifested by allergic reaction, so it is called Anaphylactoid Purpura. The prognosis of disease is usually good, but about 50% of patients have experienced recurrences. Oral corticosteroid is treatment of choice, but it cannot prevent recurrence of Disease. We experienced two case of young patients who suffered from the Henoch-Schnlein purpura. We prescripted Mokyangbinrang-hwan, Insamyang-witang, Gwibi-tang. For both two patient, symptoms were much relieved.

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Safety of radiotherapy in patients with Behcet's disease: a case report and review of the literature

  • Ko, Dahui;Kim, Young Suk;Choi, Yunseon
    • Journal of Medicine and Life Science
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    • 제18권2호
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    • pp.35-39
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    • 2021
  • Exaggerated acute and late toxicities following radiotherapy have been reported in patients with pre-existing connective tissue diseases, such as systemic lupus and scleroderma. Behcet's disease (BD) is a relapsing multisystem connective tissue disease characterized by vasculitis in the mucocutaneous, ocular, gastrointestinal, respiratory, neurologic, urogenital, articular, and cardiovascular systems. Data concerning the relationship between radiotherapy toxicity and BD are limited in the literature. Here, we report a case of lung cancer treated with radiotherapy (60 Gy) in a patient with BD. No severe radiation-induced toxicity was observed. Radiation-induced toxicity in patients with BD has also been discussed.

Henoch-Sc$\ddot{o}$lein 자반증에서 출혈성 물집을 동반한 9세 소아 1례 (Hemorrhagic Bullous Lesions in a 9-year-old Girl with Henoch-Sch$\ddot{o}$lein Purpura)

  • 김문규;박성은;이준호
    • Childhood Kidney Diseases
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    • 제16권1호
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    • pp.51-53
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    • 2012
  • 헤노흐-쇤라인 자반증(HSP)은 소아에서 가장 흔한 혈관염으로 피부 자반증, 관절염, 복통, 그리고 신염 등을 특징으로 한다. HSP 소아에서 물집을 동반하는 경우는 매우 드물다. 관절염을 동반한 HSP를 보인 9세 여아에서 경구용 스테로이드 치료를 시작했음에도 불구하고 피부 자반이 빠르게 출혈성 물집 병변으로 진행되었다. 치료 7일 후 몇몇 궤양성 병변은 경미한 흉터로 남았으나 관찰 6개월 후 흉터는 완전히 소멸되었다. 현미경적 혈뇨는 관찰 6개월간 지속되었으나 단백뇨는 관찰되지 않았다. HSP 소아에서 물집이 동반되었을 경우, 신장의 예후에는 직접적인 연관은 없었다. 임상적으로 HSP 진단기준을 만족한다면 HSP 진단을 위해 피부조직검사는 필요 없을 것으로 보이며, 더 많은 증례가 모이면 정확한 결론을 내릴 수 있을 것으로 사료된다. 관절염을 동반한 HSP 9세 여자 소아에서 경구용 스테로이드 치료를 시작했음에도 불구하고 피부 자반이 빠르게 출혈성 물집 병변으로 진행되는 HSP 증례를 경험하였기에 보고하는 바이다.

장천공이 동반된 Churg-Strauss 증후군 1예 (A Case of Churg-Strauss Syndrome with Interstinal Perforation)

  • 박정훈;정윤석;김양기;이영목;황정화;김기업;김동원;어수택;김재준
    • Tuberculosis and Respiratory Diseases
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    • 제66권5호
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    • pp.374-379
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    • 2009
  • 천식조절 중이던 환자에서 임상증상과 조직검사를 바탕으로 진단된 Churg-Strauss 증후군을 진단하고 치료 중 발생한 장천공을 경험하였기에 문헌고찰과 함께 보고하는 바이다.