Jalil Ajura bt Abdul, Hin-Lau Shin
Stomatology Unit, Cancer Research Centre, Institute for Medical Research, 50588 Jalan Pahang, Kuala Lumpur, Malaysia.
Int J Paediatr Dent. 2009 Sep;19(5):349-53. doi: 10.1111/j.1365-263X.2009.00985.x. Epub 2009 Apr 16.
Oral Langerhans cell histiocytosis is generally seen in children.
To determine the clinicopathological features of oral LCH in Malaysian paediatric patients.
A retrospective study was carried out to determine the clinicopathological features of Langerhans cell histiocytosis (LCH), Letterer-Siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma, and histiocytosis X occurring in the oral cavity in children, diagnosed histologically in the main oral histopathology laboratory in Malaysia from 1967 to 2007.
There were 17 cases (eight girls and nine boys) with age ranging from 1 to 7 years. There were ten Malays, four Chinese, two Indians, and one of other ethnicity. Thirteen cases presented as gingival swellings with six of these cases accompanied with mobility of the teeth. Nine cases involved the mandible, two in the maxilla, and two cases in both the maxilla and mandible. The radiographic findings were mentioned only in nine cases with presence of bony erosion or destruction of the jaw bones. Four cases had punched-out radiolucencies of the skull. The patients also had other systemic signs and symptoms: skin lesions (n = 5), hepatosplenomegaly (n = 2), prolonged fever (n = 2), diabetes insipidus (n = 1), and exophthalmos (n = 1). Two cases were known cases of systemic LCH.
The histopathologic features of LCH are easily recognized; however, with the development of immunostaining, the use of CD1a helps in confirming the diagnosis.
口腔朗格汉斯细胞组织细胞增多症多见于儿童。
确定马来西亚儿科患者口腔朗格汉斯细胞组织细胞增多症（LCH）的临床病理特征。
开展一项回顾性研究，以确定1967年至2007年在马来西亚主要口腔组织病理学实验室经组织学诊断的儿童口腔中发生的朗格汉斯细胞组织细胞增多症（LCH）、勒-雪病、汉-许-克病、嗜酸性肉芽肿和组织细胞增多症X的临床病理特征。
共17例（8名女孩和9名男孩），年龄1至7岁。其中马来人10例，华人4例，印度人2例，其他种族1例。13例表现为牙龈肿胀，其中6例伴有牙齿松动。9例累及下颌骨，2例累及上颌骨，2例上颌骨和下颌骨均受累。仅9例提及影像学表现，可见颌骨骨质侵蚀或破坏。4例颅骨有圆形透亮区。患者还伴有其他全身症状和体征：皮肤病变（5例）、肝脾肿大（2例）、长期发热（2例）、尿崩症（1例）和眼球突出（1例）。2例为系统性LCH已知病例。
LCH的组织病理学特征易于识别；然而，随着免疫染色技术的发展，CD1a的应用有助于确诊。
