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成人起病的朗格汉斯细胞组织细胞增多症——口腔中的“特洛伊木马”:一例罕见临床表现的病例报告

Adult-onset Langerhans cell histiocytosis - A Trojan horse of oral cavity: A case report with rare clinical presentation.

作者信息

Sanjay C J, Patil Karthikeya, Hegde Usha, Panda Romali

机构信息

Department of Oral Medicine and Radiology, JSS Dental College and Hospital, JSS Academy of Higher Education and Research (JSSAHE&R), Mysuru, Karnataka, India.

Department of Oral and Maxillofacial Pathology, JSS Dental College and Hospital, JSS Academy of Higher Education and Research (JSSAHE&R), Mysuru, Karnataka, India.

出版信息

J Oral Maxillofac Pathol. 2023 Feb;27(Suppl 1):S98-S103. doi: 10.4103/jomfp.jomfp_131_22. Epub 2023 Feb 4.

Abstract

Langerhans cell histiocytosis (LCH), earlier recognised as histiocytosis X, is a rare haematological illness involving infants and young children. LCH is caused by unrestrained stimulation and proliferation of usual antigen presenting cells, Langerhans cells (LCs) and the disease demonstrates extensive clinical and radiographic features involving multiple sites. Since the incidence is relatively low limited data is available regarding the epidemiology of LCH, with approximation of 2-5 cases per million populations per year. LCH has male predilection with jaws involved in 10-20% cases and only 1% of the cases affecting maxilla, masquerading as periodontal or periapical pathology. We report a case of 48-year-old female with LCH involving posterior maxilla. This is a unique presentation corresponding to age, gender, location and severity. Dental clinicians should be aware of this and consider it to be a part of their differential diagnosis pertaining to unresolved periodontal pathology as it mimics clinically and radiographically.

摘要

朗格汉斯细胞组织细胞增多症(LCH),早期被称为组织细胞增多症X,是一种罕见的血液疾病,主要累及婴幼儿。LCH是由通常的抗原呈递细胞——朗格汉斯细胞(LCs)不受控制的刺激和增殖引起的,该疾病表现出涉及多个部位的广泛临床和影像学特征。由于发病率相对较低,关于LCH流行病学的可用数据有限,每年每百万人口中约有2 - 5例。LCH男性多见,10% - 20%的病例累及颌骨,仅占上颌骨病例的1%,常表现为牙周或根尖周病变。我们报告一例48岁女性LCH累及上颌后部的病例。这是一个在年龄、性别、部位和严重程度方面都很独特的表现。牙科临床医生应意识到这一点,并将其视为与未解决的牙周病变相关的鉴别诊断的一部分,因为它在临床和影像学上都有相似表现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/245f/10112703/69c92f5313cb/JOMFP-27-98-g001.jpg

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