증례 : 다양한 진단도구와 반복적인 피부조직검사를 통해 확진한 원발성 전신성 유전분증 1예

분야
의약학 > 피부과학
저자
고범준 ( Bum Joon Ko ) , 이강모 ( Gang Mo Lee ) , 최인호 ( In Ho Choi ) , 박영림 ( Young Lip Park ) , 이성열 ( Sung Yul Lee ) , 황규왕 ( Kyu Uang Whang )
발행기관
대한피부과학회
간행물정보
대한피부과학회지 2013년, 제51권 제9호, 713~717페이지(총5페이지)
파일형식
27728962.pdf [무료 PDF 뷰어 다운로드]
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    영문초록
    Amyloidosis is a group of disorders resulting from the extracellular deposition of amyloid fibrils in tissues and organs. Primary systemic amyloidosis may be myeloma-associated or idiopathic. It involves the kidney, heart, liver,peripheral nerves, autonomic nervous system and skin. We report a case of a 76 year-old woman with primary systemic amyloidosis who suffered from ecchymotic purpura on the periorbital, flexural area with hemorrhagic bulla,and macroglossia for two years. She showed typical symptoms of AL amyloidosis, and while primary systemic amyloidosis was suspected from electrophoresis results, no amyloid was found in the skin, tongue, and bone marrow. Upon her admission due to panperitonitis from diverticulitis, she was diagnosed with primary systemic amyloidosis after amyloid deposition was confirmed in the skin and colon biopsy. She had been treated with bortezomib, but she expired from methicillin-resistant Staphylococcus aureus septic shock.
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