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Erdheim-Chester병의 비특이성 발현: 1례보고

Unusual Manifestation of Erdheim-Chester Disease - A Case Report -

건양의대학술지 2004년 4권 2호 p.221 ~ 225
황철목, 조영준, 이종주,
소속 상세정보
황철목 ( Hwang Cheol-Mog ) - 건국대학교 의과대학 영상의학교실
조영준 ( Cho Young-Jun ) - 건양대학교 의과대학 영상의학교실
이종주 ( Lee Jong-Joo ) - 건양대학교 의과대학 신경외과학교실

Abstract


Erdheim-Chester disease belongs to the group of lipoid granulomatosis and a rare non-Langerhans cell histiocytosis of unknown etiology. Most frequently, symmetric sclerosis of the metaphyses and diaphyses of the long tubular bones of the upper and lower extremities are pathognomic radiologic changes. The disease process usually spares the epiphyses and rarely affects the axial skeleton. Additionally, other skeletal segments can be affected. Erdheim-Chester disease ranges from a focal asymptomatic process to a multisystemic fatal condition. Signs and symptoms are the result of histiocytic infiltration of various tissues. The sites of involvement can include bone, lung, pancreas, liver, skin, orbit, central nervous system, pituitary gland, retroperitoneum, pericardium and superficial soft tissues. We present the case of a 37-year-old woman with unusual manifestation of Erdheim-Chester disease as bilateral axillary masses and diametaphyseal and epiphyseal involvement of long bones of the lower extremities.

키워드

Erdheim-Chester disease;Musculoskeletal;MR

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