一名儿童出现多发性颌骨病变的 Erdheim-Chester 病。
Erdheim-Chester disease in a child presenting with multiple jaw lesions.
作者信息
Nagatsuka Hitoshi, Han Phuu Pwint, Taguchi Koji, Tsujigiwa Hidetsugu, Gunduz Mehmet, Fukunaga Joji, Sugahara Toshio, Asaumi Junichi, Nagai Noriyuki
机构信息
Department of Oral Pathology and Medicine, Graduate School of Medicine and Dentistry, Okayama University, Okayama, Japan.
出版信息
J Oral Pathol Med. 2005 Aug;34(7):420-2. doi: 10.1111/j.1600-0714.2005.00320.x.
BACKGROUND
Erdheim-Chester disease is a rare histiocytic disease entity related to juvenile xanthogranuloma. It is a systemic condition, usually occurs in adult, characterized by infiltration of foamy histiocytes within the bone and soft tissues.
METHODS AND RESULTS
We report a case of 13-year-old female patient who first presented with multiple osteolytic lesions of the jaws followed by bilateral symmetrical bone lesions affecting the lower extremities, as well as brain and abdominal involvement. Histological findings of the jaw lesions showed lipid-storing CD68 (+), CD1a (-) histiocytes with Touton type giant cells.
CONCLUSION
To the best of our knowledge, this is the first case of Erdheim-Chester disease with jaw bone lesions occurring as initial presenting symptom.
背景
Erdheim-Chester病是一种与幼年性黄色肉芽肿相关的罕见组织细胞病实体。它是一种全身性疾病，通常发生于成年人，其特征是泡沫状组织细胞浸润骨骼和软组织。
方法与结果
我们报告一例13岁女性患者，最初表现为颌骨多处溶骨性病变，随后出现累及双下肢的双侧对称性骨病变，以及脑和腹部受累。颌骨病变的组织学检查结果显示为储存脂质的CD68(+)、CD1a(-)组织细胞及图顿型巨细胞。
结论
据我们所知，这是首例以颌骨病变为首发症状的Erdheim-Chester病。
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