Katz A M, Rosenthal D, Jakubovic H R, Pai R K, Quinonez G E, Sauder D N
Division of Dermatology, McMaster University Medical Center, Hamilton, Canada.
J Am Acad Dermatol. 1991 Jan;24(1):32-7. doi: 10.1016/0190-9622(91)70005-m.
Langerhans cells histiocytosis, one of a group of histiocytosis syndromes characterized by Langerhans cell infiltration, has many clinical manifestations. In the past 30 years, numerous cases of presumed Letterer-Siwe disease, the acute multiorgan variant, have been reported in twins and siblings. Only recently has the Histiocyte Society established a criterion for a "definitive diagnosis" of Langerhans cell histiocytosis--the presence of Birbeck granules within the cells of the histiocytic infiltrate. We report the fatal outcome of Langerhans cell histiocytosis in monozygotic twin infants. There is no satisfactory explanation why Langerhans cell histiocytosis occurs concurrently in twins. We suggest that cytokines may provide an endogenous signal that triggers the pathologic proliferation of Langerhans cells.
朗格汉斯细胞组织细胞增多症是一组以朗格汉斯细胞浸润为特征的组织细胞增多症综合征之一，有多种临床表现。在过去30年里，双胞胎和兄弟姐妹中报告了许多疑似勒-雪病（急性多器官型）的病例。直到最近，组织细胞协会才确立了朗格汉斯细胞组织细胞增多症“确诊”的标准——组织细胞浸润细胞内存在伯贝克颗粒。我们报告了一对单卵双胞胎婴儿患朗格汉斯细胞组织细胞增多症的致命结局。对于朗格汉斯细胞组织细胞增多症为何会在双胞胎中同时发生，目前尚无令人满意的解释。我们认为细胞因子可能提供了一个触发朗格汉斯细胞病理性增殖的内源性信号。
