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朗格汉斯细胞组织细胞增多症的口腔表现:病例系列

Oral manifestations of Langerhans cell histiocytosis: A case series.

作者信息

Neves-Silva Rodrigo, Fernandes Diego Tetzner, Fonseca Felipe Paiva, Rebelo Pontes Helder Antonio, Brasileiro Bernardo Ferreira, Santos-Silva Alan Roger, Vargas Pablo Agustin, Lopes Marcio Ajudarte

机构信息

Oral Diagnosis Department, Piracicaba Dental School, University of Campinas (UNICAMP), Piracicaba, São Paulo, Brazil.

Oral Pathology, Federal University of Pará, Belém, Brazil.

出版信息

Spec Care Dentist. 2018 Nov;38(6):426-433. doi: 10.1111/scd.12330. Epub 2018 Sep 12.

Abstract

AIM

Langerhans cell histiocytosis (LCH) is an unusual proliferative disorder of bone marrow-derived histiocytes (Langerhans cells) that can produce focal or systemic manifestations. Oral manifestations of LCH can present as single or multiple lesions and can be a challenge in clinical practice. The aim of this paper is to present the clinicopathological features of a series of nine patients with oral involvement.

METHODS AND RESULTS

The patient's age ranged from 2 to 63 years being five males and four females. The most common oral site involvement was the hard-palate mucosa. Ulceration was the main clinical feature. Only two patients showed clearly jawbone involvement. In eight out of nine patients, the diagnosis of LCH was established because of the oral manifestations.

CONCLUSION

The recognition of the clinical features of LCH oral manifestation is important to avoid misdiagnosis and to the establishment of the correct treatment. Thus, dentists can play a vital role in the diagnosis of LCH since oral lesions may be the earliest manifestation and sometimes the only sign of the disease. Furthermore, oral lesions may be the early signs of disease reactivation or a multisystem disease indication.

摘要

目的

朗格汉斯细胞组织细胞增多症(LCH)是一种源于骨髓的组织细胞(朗格汉斯细胞)的罕见增殖性疾病,可产生局部或全身表现。LCH的口腔表现可呈现为单个或多个病变,在临床实践中可能具有挑战性。本文旨在呈现一系列9例口腔受累患者的临床病理特征。

方法与结果

患者年龄从2岁至63岁不等,男性5例,女性4例。最常见的口腔受累部位是硬腭黏膜。溃疡是主要临床特征。仅有2例患者显示明显的颌骨受累。9例患者中有8例因口腔表现而确诊为LCH。

结论

认识LCH口腔表现的临床特征对于避免误诊和确立正确治疗很重要。因此,牙医在LCH的诊断中可发挥至关重要的作用,因为口腔病变可能是该疾病的最早表现,有时甚至是唯一症状。此外,口腔病变可能是疾病复发或多系统疾病的早期迹象。

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