기관지 천식을 동반하지 않은 Churg-Strauss 증후군 1예
An atypical case of Churg-Strauss syndrome without asthma
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초록
본 증례에서는 18세 남자 환자가 우측 손가락의 변색과 우측 족하수를 주소로 내원하여 기관지 천식의 동반 없이 말초 혈액의 호산구 증다증, 폐침윤, 다발성 단일 신경병증, 혈관외 호산구침윤, 다발성 혈관염 등의 임상양상을 근거로 Churg-Strauss 증후군으로 진단하였다. Churg-Strauss 증후군의 경우 진단이 늦어질 경우 치명적일 수 있으므로 원인을 알 수 없는 호산구 증다증, 폐침윤, 신경학적 증상 등을 주소로 내원한 환자에서 천식이 동반되지 않을지라도 초기 감별 진단으로 Churg-Strauss 증후군을 반드시 고려하고 필요할 경우 조직 검사를 조기에 적극적으로 시행해야 할 것이다.
Abstract
Churg-Strauss syndrome is a rare systemic necrotizing vasculitis that is characterized by asthma, peripheral eosinophilia, non-fixed pulmonary infiltrates, neuropathy and histopathologic features of extravascular eosinophilic infiltration and granuloma formation. Churg-Strauss syndrome is accompanied by asthma in nearly all cases. However, a few atypical cases of Churg-Strauss syndrome without asthma or eosinophilia have been reported. An 18-year-old male was referred for discoloration of right second finger, and right foot drop. He developed peripheral eosinophilia, bilateral pulmonary infiltrates, mononeuritis multiplex, extravascular eosinophilic infiltration on lung biopsy, and vasculitis on medium to small sized arteries of upper and lower extremities without asthma. We report an atypical case of an 18-year-old male patient diagnosed as Churg-Strauss syndrome without preexisting asthma.
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