关键词: Langerhans cells adult diabetes insipidus eosinophilic granuloma histiocytosis osteonecrosis

来  源:   DOI:10.1002/ccr3.6927   PDF(Pubmed)

Abstract:
Langerhans cell histiocytosis (LCH) is an uncommon proliferative disease with an unknown cause. Its clinical manifestations vary and can involve a variety of organs. To diagnose LCH, radiographs, histopathological and immunohistochemical findings are essential. The gold standard for a definite diagnosis is positive CD1a/CD207 and S100 in the immunohistochemical results. Different treatment plans are available for patients struggling with LCH. To our knowledge, the LCH incidence rate is about 8.9 in one million children and 1-2 cases in one million adults. Our case shows the importance of early diagnosis of this rare condition for the prevention of any further spreading. This case report is about a 35-year-old male patient struggling with diabetes insipidus with a chief complaint of tooth mobility. Based on his X-ray radiographs, osteolytic lesions were found. A biopsy was performed, and due to histopathological and immunohistochemical findings, it was diagnosed as a case of LCH in adults. This paper shows that although the incidence rate of LCH is rare in adults, dealing with a patient struggling with diabetes insipidus and osteolytic bone lesions with an unknown cause, LCH must be taken into consideration. Due to the fact that LCH\'s first manifestations are mostly first oral, its good prognosis in the early stages, and if it progresses, it can turn fatal, it is important that dentists are aware of this disease, its clinical manifestations, and patient management. In case of suspicion, X-ray examination, biopsy, histopathological, and immunohistochemical exams must be performed.
摘要:
朗格汉斯细胞组织细胞增生症(LCH)是一种罕见的增殖性疾病,原因不明。其临床表现各不相同,可涉及多种器官。诊断LCH,射线照片,组织病理学和免疫组织化学结果是必不可少的。明确诊断的金标准是免疫组织化学结果中CD1a/CD207和S100阳性。不同的治疗计划可用于患有LCH的患者。据我们所知,100万儿童的LCH发病率约为8.9例,100万成人的LCH发病率约为1-2例。我们的案例表明,早期诊断这种罕见疾病对于防止任何进一步传播的重要性。该病例报告是关于一名35岁的男性患者患有尿崩症,主要主诉为牙齿活动。根据他的X光片,发现溶骨性病变。做了活检,由于组织病理学和免疫组织化学结果,它被诊断为成人LCH病例。本文表明,虽然LCH的发病率在成年人中很少见,处理患有尿崩症和溶骨性骨病变的患者,原因不明,必须考虑LCH。由于LCH的最初表现大多是口头的,早期预后良好,如果进展,它可以变成致命的,牙科医生意识到这种疾病是很重要的,其临床表现,和病人管理。如果有人怀疑,X光检查,活检,组织病理学,必须进行免疫组织化学检查。
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