简介和重要性:朗格汉斯细胞组织细胞增生症是一种罕见的疾病,其特征是朗格汉斯细胞在各种组织中的增殖。虽然它通常会影响骨骼,皮肤,和其他器官,口腔表现较不常见,但可能具有诊断挑战性.
方法:我们介绍了一例在14岁青少年中出现口腔表现的LCH的临床病例。患者出现进行性颌骨疼痛约两个月。
方法:口内检查显示下颌角前庭肿胀,前庭触诊疼痛。然而,病变的相邻牙齿没有移动,活力测试是积极的。放射学检查显示左下颌角存在不均匀的低密度溶骨性病变。组织病理学分析证实了LCH的诊断,牙龈组织中朗格汉斯细胞弥漫性浸润。患者被转诊到马赛医院,选择了治疗性弃权,每6个月进行一次临床和放射学随访,为期5年.在6周,观察到病变的显着减少和骨膜重建现象。一年后,病变已自发消退。临床讨论:疾病活动性评分允许定义疾病的严重程度和活动性并确定治疗。朗格汉斯细胞组织细胞增生症的临床表现是高度可变的。该疾病的严重形式发生在2岁之前,其特征是多系统受累和神经退行性受累。
结论:活检后病灶自发消退。在这种情况下,及时诊断和多学科管理对于成功的治疗结果至关重要。该病例强调了在牙科实践中识别LCH的口腔表现对于早期诊断和适当管理的重要性。
Introduction and importance: Langerhans cell histiocytosis is a rare disorder characterized by the proliferation of Langerhans cells in various tissues. While it commonly affects bones, skin, and other organs, oral manifestations are less frequent but can be diagnostically challenging.
METHODS: we present a clinical
case of LCH with an oral manifestation in a 14-year-old adolescent. The patient presented with progressive jaw pain for approximately two months.
METHODS: Intraoral examination revealed vestibular swelling at the mandibular angle, with painful vestibular palpation. However, the adjacent teeth to the lesion were not mobile, and the vitality test was positive. Radiographic examination showed an inhomogeneous hypodense osteolytic lesion at the left mandibular angle. Histopathological analysis confirmed the diagnosis of LCH with diffuse infiltration of Langerhans cells in the gingival tissue. The patient was referred to Hospital in Marseille and therapeutic abstention is chosen, clinical and radiological follow-up is established every 6 months for 5 years. At 6 weeks, a significant decrease in the lesion and an osteoperiosteal reconstruction phenomenon are observed. After one year, the lesion has spontaneously resolved. Clinical discussion: The disease activity score allows defining the severity and activity of the disease and determine the treatment. The clinical presentation of Langerhans cell histiocytosis is highly variable. The severe form of the disease occurs before the age of 2 and is characterized by multi-system involvement and neurodegenerative involvement.
CONCLUSIONS: The lesion resolved spontaneously after the biopsy was performed. Prompt diagnosis and multidisciplinary management are crucial for successful treatment outcomes in such cases. This
case highlights the importance of recognizing oral manifestations of LCH in dental practice for early diagnosis and appropriate management.