Joo Chan Uhng, Go Yang Sim, Kim In Hwan, Kim Chul Seong, Lee Sang Yong
Department of Pediatrics, Chonbuk National University Medical School, 561-712 Jeonbuk, Korea.
Skeletal Radiol. 2005 May;34(5):299-302. doi: 10.1007/s00256-004-0846-y. Epub 2004 Oct 8.
Erdheim-Chester disease is a disseminated xanthogranulomatous infiltrative disease of unknown origin that generally presents in adulthood. A review of the English-language literature demonstrated that pediatric cases were extremely rare, and to our knowledge, only two cases, a 7- and 14-year-old, have been published.
We report a case of Erdheim-Chester disease in a 10-year-old girl evaluated with MR imaging. Radiographs revealed typical bilateral, symmetric osteosclerosis of the metaphyseal regions of long bones of the upper and lower extremities.
A histologic examination demonstrated foamy histiocytes in bone marrow smears. Bilateral symmetric low signal intensities of both proximal tibiae and distal femurs were demonstrated on T1-weighted MR images. After oral steroid therapy for 8 months, follow-up MR imaging showed remarkable restoration of normal high signal intensity in both the tibial and femoral metaphyses.
To our knowledge, this may be the first case of Erdheim-Chester disease that showed normal restoration of the abnormal signal intensities in the metaphyses of long bones after steroid therapy.
Erdheim-Chester病是一种起源不明的播散性黄色瘤性浸润性疾病，通常在成年期出现。对英文文献的回顾表明，儿科病例极为罕见，据我们所知，仅有两例已发表，分别为7岁和14岁。
我们报告一例10岁女孩的Erdheim-Chester病，该病例接受了磁共振成像（MR）评估。X线片显示上下肢长骨干骺端典型的双侧对称性骨硬化。
组织学检查显示骨髓涂片中有泡沫状组织细胞。T1加权MR图像上显示双侧胫骨近端和股骨远端呈对称低信号强度。口服类固醇治疗8个月后，随访MR成像显示胫骨干骺端和股骨干骺端均显著恢复为正常高信号强度。
据我们所知，这可能是首例在类固醇治疗后长骨干骺端异常信号强度恢复正常的Erdheim-Chester病病例。
