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야간뇨와 호흡곤란으로 발현된 신장을 침범한 특발성 호산구 증가 증후군
정윤진 ( Yun Jin Jeung ),이진영 ( Jin Young Lee ),이가연 ( Ga Yeon Lee ),오미정 ( Mi Jung Oh ),이병재 ( Byung Jae Lee ),최동철 ( Dong Chull Choi ) 대한천식알레르기학회 2010 천식 및 알레르기 Vol.30 No.3
Idiopathic hypereosinophilic syndrome (HES) is characterized by marked (>1,500/μL) and persistent (>6 months) eosinophilia without an identifiable underlying cause. This condition is associated with multiorgan involvements. Renal involvement is rarely reported and little known about renal morphologic changes in this disease. We report a case of HES with renal involvement presenting with nocturia and dyspnea. A 30-year-old man visited to our institution due to nocturia and dyspnea. Peripheral blood tests showed an increased eosinophil count (14,868/㎣). Chest CT showed extensive areas of ground-glass opacities in both lungs. Renal biopsy showed thrombotic microangiopathic nephropathy. Based upon his medical records, physical examination findings and laboratory data, he was diagnosed as having idiopatic HES with renal involvement. We found FIP1-like-1-platelet-derived growth factor receptor-α (FIP1L1-PDGFR α) gene rearrangement on BM aspiration. He was treated with oral glucocorticoid and tyrosine kinase inhibitor (imatinib mesylate). His symptoms were improved and proteinuria disappeared. (Korean J Asthma Allergy Clin Immunol 2010;30:241-245)
심부정맥혈전증이 동반된 Churg-Strauss 증후군
정승민 ( Seung Min Chung ),이진희 ( Jin Hee Lee ),전준석 ( Jun Seok Jeon ),조아진 ( A Jin Cho ),정윤진 ( Yun Jin Jeung ),이병재 ( Byeoung Jae Lee ),최동철 ( Dong Chull Choi ),김태은 ( Tae Eun Kim ) 대한천식알레르기학회 2011 천식 및 알레르기 Vol.31 No.2
Churg-Strauss syndrome (CSS) is a rare multi-system vasculitis. Patients with CSS are at greater risk of venous thromboembolic events. However, venous thrombosis is rarely reported in patients with CSS in Korea. We report 1 patient with CSS who experienced deep vein thrombosis. A 27-year-old man was admitted to our hospital due to fever, cough, skin rash on both lower extremities, and right leg edema. He was diagnosed with CSS based on a history of asthma, peripheral eosinophilia, transient pulmonary infiltrates on chest radiograph, and extravascular eosinophils in the skin biopsy specimens. After steroid pulse therapy and the first cycle of intravenous pulse cyclophosphamide therapy, right leg edema did not resolve. Venous ultrasonography and CT angiography detected deep vein thrombosis involving the right common iliac, femoral and calf veins. The patient received both anticoagulation therapy and intravenous pulse cyclophosphamide therapy. After 6 months of anticoagulation therapy and the 9 cycles of intravenous pulse cyclophosphamide therapy, deep vein thrombosis completely resolved. (Korean J Asthma Allergy Clin Immunol 2011;31:148-152)
자각 증상과 심장비대 없이 심장 침범된 Churg-Strauss 증후군
김수아 ( Su A Kim ),안희경 ( Hee Kyung Ahn ),이수진 ( Su Jin Lee ),이준호 ( Jun Ho Yi ),정윤진 ( Yun Jin Jeung ),이병재 ( Byeoung Jae Lee ),최동철 ( Dong Chull Choi ) 대한내과학회 2010 대한내과학회지 Vol.78 No.2
We report a case of Churg-Strauss syndrome with cardiac involvement presenting without cardiomegaly or cardiopulmonary symptoms. A 47-year-old woman was referred to our institution for myalgia, peripheral numbness, and eosinophilia. She had been diagnosed with bronchial asthma and allergic rhinitis four years ago. The patient exhibited eosinophilia (71%) and elevated cardiac enzymes (cTnI, 2.977ng/mL). Cardiomegaly was not observed on chest radiography, but nonspecific ST segment changes were observed on electrocardiography. A transthoracic echocardiography revealed a dilated left ventricular cavity, a decreased left ventricle (42%), and diastolic dysfunction. Contrast-enhanced cardiac magnetic resonance imaging revealed delayed hyperenhancement 10 minutes after injecting gadolinium. An endomyocardial biopsy showed eosinophilic myocarditis associated with vasculitis. The patient was diagnosed with Churg-Strauss syndrome and received combination therapy with steroid and cyclophosphamide. After the second treatment cycle, the blood eosinophilia disappeared and the vasculitis and infiltration of eosinophils into the endomyocardial tissue had completely resolved. (Korean J Med 78:266-272, 2010)
말초혈액 호산구증다증이 없는 고열을 동반한 Wells 증후군
황지원 ( Ji Won Hwang ),김혜영 ( Hye Young Kim ),오세훈 ( Se Hoon Oh ),이창훈 ( Chang Hoon Lee ),정윤진 ( Yun Jin Jeung ),이병재 ( Byung Jae Lee ),최동철 ( Dong Chul Choi ) 대한천식알레르기학회 2008 천식 및 알레르기 Vol.28 No.4
Wells` syndrome is eosinophilic cellulitis characterized clinically by an acute dermatitis resembling cellulitis. Peripheral eosinophilia is not necessary for the diagnosis but is present in approximately 50% of during active disease. We report a case of Wells` syndrome which was clinically difficult to diagnose without biopsy. A 26-year-old woman presented with high fever and skin lesion mimicking urticaria migrating in chest, abdomen and extremities. The skin lesion was aggravated with rise in temperature and was evanescent. Well-demarcated swollen and tender erythematous plaques were in ear lobes, neck, chest, abdomen and extremities. Viral markers, blood cultures and other laboratory test results related to infections such as EBV, CMV and Limedisease were negative. FANA, ANCA and ENA were also negative. There was no peripheral eosinophilia. Skin biopsy performed from the volar side of right forearm revealed the infiltration of eosinophils with multifocal collagen degeneration in dermis without vasculitic process, consistent with the early stage of Wells` syndrome. The patient was treated with intravenous methylprednisolone and antihistamine with remarkable clinical improvement. Wells` syndrome should be included in the differential diagnosis of any skin lesion mimicking cellulitis even without peripheral eosinophilia. Confirmatory skin biopsy is mandatory for the definite diagnosis of this disease. (Korean J Asthma Allergy Clin Immunol 2008;28:305-308)