Adib O, Baroth E, Perard L, Scoazec J-Y, Vervueren L, Aubé C, Willoteaux S
Département de radiologie, centre hospitalier universitaire d'Angers, 4, rue Larrey, 49933 Angers cedex 09, France.
J Radiol. 2011 Jul-Aug;92(7-8):671-80. doi: 10.1016/j.jradio.2011.04.013. Epub 2011 Jul 5.
Erdheim-Chester disease is a rare form of systemic non-Langerhans cell histiocytosis characterized by infiltration by lipid-laden or foamy histiocytes. Osseous involvement, major diagnostic criteria, is constant and characteristic. It presents as metaphyseal and diaphyseal osteosclerosis, mainly affecting the long bones of the lower limbs. A few cases with axial skeleton involvement have been reported. Extra-osseous lesions may affect the retroperitoneum, lungs, skin, heart, brain and orbits. Prognosis depends mainly on the extra-osseous disease, mainly heart and lung involvement. Diagnosis is based on the combination of radiographic features, nuclear medicine features and nearly pathognomonic immunohistochemical profile.
厄尔德海姆-切斯特病是一种罕见的系统性非朗格汉斯细胞组织细胞增多症，其特征为富含脂质的组织细胞或泡沫状组织细胞浸润。骨受累是主要诊断标准，具有持续性和特征性。表现为干骺端和骨干骨质硬化，主要累及下肢长骨。已有少数累及中轴骨骼的病例报道。骨外病变可累及腹膜后、肺、皮肤、心脏、脑和眼眶。预后主要取决于骨外病变，主要是心脏和肺部受累情况。诊断基于影像学特征、核医学特征以及几乎具有诊断意义的免疫组化表现的综合判断。
