Fink M G, Levinson D J, Brown N L, Sreekanth S, Sobel G W
Department of Pathology, Humana Hospital-Michael Reese, Chicago, Ill. 60616.
Arch Pathol Lab Med. 1991 Jun;115(6):619-23.
Erdheim-Chester disease is a rare pathologic entity characterized by symmetrical radiodensities in the metaphyseal and the diaphyseal portions of the long bones. Fibrosis, osteoblastic cortical bone deposition, and fibroxanthomatous granulomas with lipid-laden macrophages and multinucleated giant cells, which have a particular tropism for connective and adipose tissues, are the pathologic hallmarks. To our knowledge, 27 cases have been reported in the literature since the entity was first described in 1930. Protean clinical features range from a focal and asymptomatic process to a multisystemic infiltrative disease. We describe the clinical course of a new case and review the extensive pathologic findings at autopsy, including those demonstrated by light and electron microscopy and cytochemical and immunocytochemical studies.
厄德里希-切斯特病是一种罕见的病理实体，其特征是长骨干骺端和骨干部分出现对称性放射密度影。纤维化、成骨细胞性皮质骨沉积以及含有充满脂质的巨噬细胞和多核巨细胞的纤维黄色瘤性肉芽肿，这些病变对结缔组织和脂肪组织具有特殊的嗜性，是其病理特征。据我们所知，自1930年首次描述该实体以来，文献中已报道了27例病例。其临床表现多样，从局部无症状病变到多系统浸润性疾病不等。我们描述了一例新病例的临床过程，并回顾了尸检时广泛的病理发现，包括光镜、电镜以及细胞化学和免疫细胞化学研究结果。
