Department of Dermatology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam-si, Korea.
J Dermatol. 2013 Oct;40(10):824-8. doi: 10.1111/1346-8138.12232. Epub 2013 Aug 20.
Epidemiological study of Langerhans cell histiocytosis (LCH) has been limited due to its rarity and multisystemic involvement. The aim of this study was to investigate the epidemiological features of LCH via the clinical data warehouse (CDW). Clinical data of 30 LCH patients from the all departments of a tertiary referral hospital between 2003 and 2012 were analyzed retrospectively by searching the CDW. The male-to-female ratio was 2.8:1. The age of onset ranged 7 days to 57 years with a median of 13 years. Of the patients, 36.7% presented initial symptoms before the age of 10 years. The involved organs at diagnosis were: bone (66.7%), skin (16.7%), lungs (13.3%) and lymph node (3.3%). For all of the 30 cases, there were 31 disease sites because of a single case of multisystemic disease involving both skin and bone. Of the 96.7% of patients with single-system disease, 69.0% had bony involvement. This study elucidated the clinical features of LCH from all the departments of a tertiary hospital via the CDW, which suggests a potential role of the CDW as a new epidemiological approach for rare diseases.
朗格汉斯细胞组织细胞增生症（LCH）的流行病学研究受到其罕见性和多系统受累的限制。本研究旨在通过临床数据仓库（CDW）调查 LCH 的流行病学特征。通过搜索 CDW，回顾性分析了 2003 年至 2012 年间来自一家三级转诊医院所有科室的 30 例 LCH 患者的临床数据。男女比例为 2.8:1。发病年龄为 7 天至 57 岁，中位数为 13 岁。66.7%的患者在 10 岁前出现首发症状。诊断时受累器官为：骨骼（66.7%）、皮肤（16.7%）、肺（13.3%）和淋巴结（3.3%）。由于单个多系统疾病同时累及皮肤和骨骼，30 例患者中共有 31 个疾病部位。96.7%的单系统疾病患者中，69.0%有骨骼受累。本研究通过 CDW 阐明了来自三级医院所有科室的 LCH 的临床特征，这表明 CDW 可能成为罕见病的一种新的流行病学研究方法。
