Kim N R, Ko Y H, Choe Y H, Lee H G, Huh B, Ahn G H
Department of Diagnostic Pathology, Sungkyunkwan University School of Medicine, Samsung Medical Center, Seoul, Korea.
Int J Surg Pathol. 2001 Jan;9(1):73-9. doi: 10.1177/106689690100900115.
Erdheim-Chester disease is a rare systemic disorder characterized by a fibrosing xanthogranulomatous infiltration of multiple organs. We report a case of Erdheim-Chester disease with diffuse necrosis leading to difficulty in making a prompt diagnosis. Radiologically, osteosclerotic lesions with osteolytic element involved metadiaphyses of both proximal tibia, and retroperitoneal infiltrations encasing both kidneys, both adrenals, and aorta were found. A biopsy of the tibia showed diffuse infiltration of foamy histiocytes, Touton-type giant cells, and fibroblastic cells associated with extensive coagulative necrosis. Immunohistochemically, foamy histiocytes were positive for CD68 and peanut agglutinin and negative for S-100 protein. A few Langerhans' cells, which were difficult to identify in hematoxylin-eosin stain, were highlighted by immunostain for S-100 protein. The patient received supportive therapy and was alive 1 1/2 years after diagnosis, with newly developed bilateral retrobulbar lesions and worsened heart failure.
厄德里希-切斯特病是一种罕见的系统性疾病，其特征为多个器官出现纤维性黄色瘤样浸润。我们报告一例伴有弥漫性坏死的厄德里希-切斯特病病例，这导致难以迅速做出诊断。放射学检查发现，双侧胫骨近端干骺端出现伴有骨质溶解成分的骨硬化性病变，以及包绕双肾、双侧肾上腺和主动脉的腹膜后浸润。胫骨活检显示泡沫状组织细胞、图顿型巨细胞和成纤维细胞弥漫性浸润，并伴有广泛的凝固性坏死。免疫组织化学检查显示，泡沫状组织细胞CD68和花生凝集素呈阳性，S-100蛋白呈阴性。少数在苏木精-伊红染色中难以识别的朗格汉斯细胞，经S-100蛋白免疫染色后得以凸显。该患者接受了支持性治疗，诊断后1年半仍存活，但出现了新的双侧球后病变且心力衰竭加重。
