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口腔表现可能是朗格汉斯细胞组织细胞增多症的首发症状。

Oral manifestations may be the first sign of Langerhans cell histiocytosis.

作者信息

Erdem Arzu Pinar, Kasimoglu Yelda, Sepet Elif, Gencay Koray, Sahin Songul, Dervisoglu Sergulen

机构信息

Department of Paedodontics, Istanbul University, Istanbul, Turkey.

出版信息

Oral Health Prev Dent. 2013;11(1):57-9. doi: 10.3290/j.ohpd.a29376.

DOI:10.3290/j.ohpd.a29376
PMID:23507682
Abstract

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of histiocytes characterised by a proliferation of abnormal and clonal Langerhans cells. LCH most commonly occurs as a localised solitary bone lesion and appears predominantly in paediatric patients. This case report presents clinicopathological features of this disorder in a 2-year-old boy. The presenting features of the patient included fever, bleeding gums and seborrheic dermatitis-like rash. Intraoral examination and radiographs revealed deep pockets and severe bone loss around all primary molars. Due to severe periodontal destruction, a gingival biopsy was taken. The biopsy specimen revealed heavy infiltration of Langerhans histiocytes. Chemotherapy was administered. Although no clear cause of LCH has been identified, the disease can be triggered by environmental agents and viruses, in particular Epstein-Barr virus. Oral manifestations may be the first or only signs of LCH.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的组织细胞增殖性疾病,其特征是异常克隆性朗格汉斯细胞增殖。LCH最常表现为局限性单发性骨病变,主要见于儿童患者。本病例报告介绍了一名2岁男孩患该疾病的临床病理特征。患者的临床表现包括发热、牙龈出血和脂溢性皮炎样皮疹。口腔检查和X光片显示所有乳牙周围有深牙周袋和严重骨质流失。由于严重的牙周破坏,进行了牙龈活检。活检标本显示朗格汉斯组织细胞大量浸润。给予了化疗。虽然尚未确定LCH的确切病因,但该疾病可由环境因素和病毒引发,尤其是爱泼斯坦-巴尔病毒。口腔表现可能是LCH的首发或唯一症状。

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