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KMID : 0948320040040020221
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Konyang Medical Journal
2004 Volume.4 No. 2 p.221 ~ p.225
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Unusual Manifestation of Erdheim-Chester Disease - A Case Report -
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Hwang Cheol-Mog
Cho Young-Jun
Lee Jong-Joo
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Abstract
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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.
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KEYWORD
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Erdheim-Chester disease, Musculoskeletal, MR
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