Lodhi Umairullah, Sarmast Uzair, Khan Saadullah, Yaddanapudi Kavitha
Department of Radiology, Stony Brook University Hospital, 101 Nicolls Road, Stony Brook, NY 11794, USA.
Case Rep Radiol. 2016;2016:2670495. doi: 10.1155/2016/2670495. Epub 2016 May 31.
Erdheim-Chester Disease is a rare form of multiorgan non-Langerhans' cell histiocytosis that affects individuals between the ages of 50 and 70 with an equal distribution among males and females. It is associated with significant morbidity and mortality that is mostly due to infiltration of critical organs. Some of the sites that Erdheim-Chester Disease affects include the skeletal system, central nervous system, cardiovascular system, lungs, kidneys (retroperitoneum), and skin. The most common presenting symptom of Erdheim-Chester Disease is bone pain although a large majority of patients are diagnosed incidentally during a workup for a different disease process. Diagnosing Erdheim-Chester Disease is challenging due its rarity and mimicry to other infiltrative processes. Therefore, a multimodality diagnostic approach is employed with imaging being at the forefront. As of date, a comprehensive radiologic review of the manifestations of Erdheim-Chester Disease has rarely been reported. Here we present radiologic findings of an individual suffering from Erdheim-Chester Disease.
厄尔德海姆-切斯特病是一种罕见的多器官非朗格汉斯细胞组织细胞增多症,影响50至70岁的个体,男女发病率相等。它与严重的发病率和死亡率相关,主要是由于关键器官的浸润。厄尔德海姆-切斯特病影响的一些部位包括骨骼系统、中枢神经系统、心血管系统、肺、肾脏(腹膜后)和皮肤。厄尔德海姆-切斯特病最常见的症状是骨痛,尽管绝大多数患者是在因其他疾病过程进行检查时偶然被诊断出来的。由于其罕见性以及与其他浸润性疾病的相似性,诊断厄尔德海姆-切斯特病具有挑战性。因此,采用多模态诊断方法,其中影像学检查处于首要位置。截至目前,很少有关于厄尔德海姆-切斯特病表现的全面放射学综述报道。在此,我们展示一名患有厄尔德海姆-切斯特病患者的放射学检查结果。