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김일규,최진풍,양정은,장재원,사시카라바라라만,김루시아,Kim, Il-Kyu,Choi, Jin-Ung,Yang, Jung-Eun,Jang, Jae-Won,Sasikala, Balaraman,Kim, Lucia 대한악안면성형재건외과학회 2009 Maxillofacial Plastic Reconstructive Surgery Vol.31 No.5
Pemphigus vulgaris is a chronic autoimmune intraepithelial blistering disease with oral mucosal manifestations that very often precede the skin lesions. The vesicles or bullae are produced by an acantholytic process, detachment of differentiating keratinocytes from one another in the epithelial stratum spino sum or spinous cell layer. The pathogenesis of this disease is initially manifested by IgG(mainly) binding to desmosome(desmoglein 3 or 1) in the intercellular spaces of epithelium. This autoantibody binding caused the release of a plasminogen activator(a proteolytic enzyme) from keratinocytes. This ultimately results in cell to cell separation. The mainstay therapy of pemphigus vulgaris is systemic corticosteroids and immunosuppressive agents to eliminate the pathogenic autoantibodies from circulation. A 41-year old woman presented with a 1.5 year history of oral ulceration. There were no lesions on the skin or other mucosal sites. Histology and immunostaining were consistent with pemphigus vulgaris. Control of oral ulceration and normal oral function were achieved after systemic corticosteroids and immunosuppressive agents were instituted.
전신성 홍반성 낭창 환자에서 상악 치은에 발생한 Kaposi's Sarcoma
김일규,조현영,박승훈,박종원,사시카라바라라만,김준미,Kim, Il-Kyu,Cho, Hyun-Young,Chang, Keum-Soo,Park, Seung-Hoon,Park, Jong-Won,Sasikala, Balaraman,Kim, Joon-Mee 대한악안면성형재건외과학회 2009 Maxillofacial Plastic Reconstructive Surgery Vol.31 No.4
Kaposi's sarcoma was first descrided by Kaposi in 1872 as an idiopathic multiple hemorrhagic sarcoma. Its clinical features revealed to be erythematous red or purple macule started out, and developing into palpable dome-shaped nodules. Etiology is not defined to detail at present. Kaposi's sarcoma is classified to 4 categories; Classical, African, Epidemic and Immunosuppressive type. Epidemic categories is found approximately 20% of all AIDS patients and has strong predilection for head and neck region. The first case of immunosuppresive type Kaposi's sarcoma in patients with kidney transplants was reported in 1969. Kaposi's sarcoma accounts for 5% of all tumors associated with transplanted patients. The most common site of Kaposi's sarcoma in immunosuppressed patients are extremities, but rare in head and neck area. A 42 years old woman who had systemic lupus erythematosus visited to our clinic because of gingival hyperplasia, and excisional biopsy revealed Kaposi's sarcoma. We experienced a case of favorable results using excision and chemotherapy, so we report with review of literatures. 본 증례는 전신성 홍반성 낭창으로 인해 2000년 4월부터 면역 억제제인 azathioprine 75mg과 prednisolne 35mg을 복용 중인 42세 여성 환자가 2004년 12월 상악 협측 치은의 종창, 증식, 동통 및 출혈을 주소로 내원하여 조직 생검 결과 모세 혈관종으로 진단되었으나, 2005년 1월 치은의 재발 병소의 절제 및 조직 생검으로 카포시 육종으로 진단되었고, 흉부의 피부 병변의 조직 생검과 흉부 전산화 단층 촬영 검사에서도 양측 폐에 다수의 결절을 보이는 카포시 육종으로 진단되었다. 의인성 면역억제제에 의한 카포시 육종으로 최종 진단되어, 면역 억제제와 부신피질호르몬제의 중단과, paclitaxel을 이용한 전신적인 항암화학 요법으로, 2008년11월 현재까지 재발의 징후 없이 구강, 피부, 폐의 병소에 대해 양호한 치료 결과를 얻었으나, 향후 전신성 홍반성 낭창의 악화 또는 카포시 육종의 재발 가능성이 있으므로 지속적인 추적 관찰이 요할 것으로 사료된다.