Tsutsumi Satoshi, Nakajima Shintaro, Oda Hisayuki, Yasumoto Yukimasa
Department of Neurological Surgery, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba, 279-0021, Japan.
Department of Pediatrics, Juntendo University Urayasu Hospital, Urayasu, Chiba, Japan.
Childs Nerv Syst. 2016 Jul;32(7):1337-41. doi: 10.1007/s00381-016-3026-1. Epub 2016 Jan 27.
Langerhans cell histiocytosis (LCH) is a multisystem disorder of unknown etiology and characterized by accumulation of histiocytes in various tissues.
A 3-year-old, previously healthy girl presented with progressive flattening of the parietal convexity for 6 months and seborrheic eczema of the scalp. At presentation, the patient showed no neurological deficit. The eczemas were extensively distributed over the scalp, but not found in any other site of the body. Blood examination revealed a marked increase in soluble interleukin-2 receptor levels. Neuroimages revealed multiple calvarial defects that were replaced by well-demarcated, enhancing extracerebral masses. A biopsy surgery confirmed the diagnosis as LCH.
LCH may cause progressive calvarial defects. If seborrheic eczemas are concurrent, they may suggest prompt histological verification and treatments be initiated.
朗格汉斯细胞组织细胞增多症（LCH）是一种病因不明的多系统疾病，其特征是组织细胞在各种组织中积聚。
一名3岁、此前健康的女孩出现顶叶凸面进行性扁平6个月，伴有头皮脂溢性湿疹。就诊时，患者无神经功能缺损。湿疹广泛分布于头皮，但身体其他部位未发现。血液检查显示可溶性白细胞介素-2受体水平显著升高。神经影像学检查发现多个颅骨缺损，被边界清晰、强化的脑外肿块取代。活检手术确诊为LCH。
LCH可能导致进行性颅骨缺损。如果同时出现脂溢性湿疹，可能提示需及时进行组织学检查并开始治疗。
