[朗格汉斯细胞组织细胞增多症与厄尔德海姆-切斯特病：可能并非偶然关联]
[Langerhans-cell histiocytosis and Erdheim-Chester disease: probably not a fortuitous association].
作者信息
Boralevi F, Léauté-Labrèze C, Tison F, Bioulac-Sage P, Vital C, Delbrel X, Cony M, Géniaux M
机构信息
Clinique Dermatologique, Hôpital Pellegrin, Bordeaux.
出版信息
Ann Dermatol Venereol. 1998 May;125(5):335-8.
BACKGROUND
Erdheim Chester disease (MEC) is a rare non-Langerhans cell histiocytosis characterized by multi-visceral involvement. We report a case of MEC associated with Langerhans cell histiocytosis (HCL).
CASE REPORT
A 46-year-old women presented skin and vulvar localization of HCL associated with typical MEC bone involvement. Despite chemotherapy (vinblastine) and prednisone, the disease progressed to involve the central nervous system, leading to fatal outcome. Post-mortem examination showed HCL in skin, MEC in bones and central nervous system, and intermediate histiocytic proliferation in the encephalon.
DISCUSSION
Usually, MEC and HCL are considered as distinct entities. MEC is characterized by a xanthogranulomatous proliferation of CD 68+ foamy histiocytes nested in fibrosis, and HCL by a proliferation of PS 100+ and CD1a+ Langerhans cells. However, our observation, as well as previous reports, suggests that MEC is part of the HCL spectrum.
背景
厄尔德海姆-切斯特病（MEC）是一种罕见的非朗格汉斯细胞组织细胞增多症，其特征为多脏器受累。我们报告一例与朗格汉斯细胞组织细胞增多症（HCL）相关的MEC病例。
病例报告
一名46岁女性出现HCL的皮肤和外阴定位，伴有典型的MEC骨受累。尽管接受了化疗（长春花碱）和泼尼松治疗，但疾病仍进展至累及中枢神经系统，导致死亡。尸检显示皮肤有HCL，骨骼和中枢神经系统有MEC，脑内有中间型组织细胞增殖。
讨论
通常，MEC和HCL被认为是不同的实体。MEC的特征是CD68+泡沫状组织细胞在纤维化中呈黄色瘤样增殖，而HCL的特征是PS100+和CD1a+朗格汉斯细胞增殖。然而，我们的观察以及既往报告表明，MEC是HCL谱系的一部分。
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