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Craniofacial Manifestations Of Eosinophilic Granuloma

机译:嗜酸性肉芽肿的颅面表现

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Purpose : The aim of the study was to present the cranifacial manifestations of eosinophilic granuloma with the review of the features , the choice of the method of treatment and the outcome of the disease.Patients and Methods : We present 5 cases with the most common clinical presentations of intraoral swelling and loose teeth. The lesions were located at the maxilla and mandible . One patient had an ulcerative lesion at the temporal region of the scalp. The radiologic view showed lytic lesions at the scalp and maxilla-mandibular area. All of the patients had an incisional biopsy with the diagnosis of "Langerhans cell Histiocytosis".Results : The choice of treatment was curettage in all patients and radiotherapy was combinated in one patient. Postoperative follow-up of the patients showed no recurrenceConclusion : Intraoral lesions located at the maxilla and mandible associated with the loose teeth should be biopsied for the diagnosis of eosinophilic granuloma. Introduction Eosinophilic granuloma (EG) of bone may be defined as a benign , well-localized solitary or multiple osseous lesions characterized by large number of eosinophils and Langerhans type histiocytes(1,2,3). It is first described by Lichtenstein and Jaffe in 1940 (1,4,5). Independently of them , Otoni and Ehrlich described cases showing the same type of the lesion under the name of “solitary granuloma of bone”. It is now known that the disease process may be present not only in one bone but in several or even many bones , so the designation “solitary granuloma” is already inappropriate (5).Eosinophilic granuloma is classified as one of a triad of nonlipid reticuloendothelial disorders; Histiocytosis X disease which may be described as a nonneoplastic , proliferative disorder of histiocytes of unknown aetiology (6,7). EG differs from Hand-Schüller-Christian disease and Letterer-Siwe disease in that it is a less agressive disease process, confined to bone, occurs later in life and demonstrates an abundance of eosinophils (1,2,4,5,6,7,8). Hand-Schüller-Christian disease which is the chronic disseminated histiocytes includes bone lesions, Diabetes Insipidus and exophtalmia (1,7). Another dimension of the disease spectrum is an acute systemic illness named as Letterer-Siwe disease. It occurs mainly in infants and young children with a rapidly fatal course manifesting itself in multiple organs (1,7).EG is the least severe and most common form of Histiocytosis X. It can affect any bones with the exception of those of hands and feet (2,3). It is more common in the pelvis, ribs, skull, vertebrae, facial bones, femur, tibia and jaws (1,2,5). The jaws are affected in 10%-20% of all EG cases. The posterior mandibular region is the most frequently affected site (9). Materials And Methods We would like to present 5 cases whose ages range from 4 years to 44 years.The most common clinic presentation was intraoral swelling and mass.Some of them also complained of loose teeth, tenderness and fetor oris.One patient had an ulcerative lesion located at the temporal region of the scalp.The laboratory findings were normal.The panoramic radiograph showed lytic lesion in the mandible.The craniography of the patient with the temporal lesion showed cranial bone destruction. Four year old patient had nuclear bone scanning with Tc 99m which revealed increased uptake of the radionuclide in the mandible.All of the patients had an incisional biopsy with the diagnosis of “Langerhans cell Histiocytosis”. A visceral ultrasonography and radiological skeletal survey showed no evidence of multifocal soft tissue or skeletal involvement. The choice of treatment was curettage. Postoperative follow-up of them showed no recurrence.
机译:目的:本研究的目的是通过嗜酸性粒细胞肉芽肿的特征,治疗方法的选择和疾病的结果来介绍颅骨的表现。患者和方法:我们介绍了5例临床上最常见的病例口腔内肿胀和牙齿松动的表现。病变位于上颌骨和下颌骨。一名患者的头皮颞部有溃疡性病变。影像学检查显示头皮和上颌-下颌骨区域有溶解性病变。所有患者均经切开活检,诊断为“朗格汉斯细胞组织细胞增生症”。结果:所有患者均选择刮宫疗法,其中一名患者接受放疗。患者的术后随访未见复发。结论:应活检位于上颌骨和与松动牙齿有关的下颌骨的口腔内病变,以诊断嗜酸性肉芽肿。引言骨嗜酸性肉芽肿(EG)可以定义为良性,局限性的孤立性或多发性骨性病变,其特征是大量嗜酸性粒细胞和Langerhans型组织细胞(1,2,3)。最早由Lichtenstein和Jaffe在1940年(1,4,5)描述。 Otoni和Ehrlich分别以“骨孤立性肉芽肿”为名描述了显示相同类型病变的病例。现已知道,疾病过程不仅可能存在于一个骨骼中,而且可能存在于数个甚至什至许多骨骼中,因此称“孤立性肉芽肿”已经不合适(5)。嗜酸性肉芽肿被归类为非脂质网状内皮组织的三联征之一。疾病;组织细胞增生性X病,可描述为病因不明的组织细胞的非肿瘤性增生性疾病(6,7)。 EG与Hand-Schüller-Christian病和Letterer-Siwe病的不同之处在于,它是一种侵略性较小的疾病过程,仅限于骨骼,在生命的后期发生,并显示出大量的嗜酸性粒细胞(1,2,4,5,6,7 ,8)。慢性弥漫性组织细胞Hand-Schüller-Christian疾病包括骨病变,尿崩症和食管外溢(1,7)。疾病谱的另一个方面是一种急性全身性疾病,称为莱特-西威病。它主要发生在具有快速致命过程的婴幼儿中,并在多个器官中表现出来(1,7)。EG是组织细胞病X病最不严重,最常见的形式。除手和手的骨骼外,它还可以影响任何骨骼。英尺(2,3)。它更常见于骨盆,肋骨,头骨,椎骨,面骨,股骨,胫骨和颌骨(1,2,5)。在所有EG病例中,颌部受到影响的占10%-20%。下颌后区域是受影响最频繁的部位(9)。资料与方法我们想介绍5例年龄在4岁至44岁之间的病例,最常见的临床表现为口腔内肿胀和肿块,其中一些还主诉牙齿松动,压痛和胎儿or肌,其中1例患者有溃疡性病变位于头皮颞区,实验室检查结果正常,全景X线片显示下颌骨溶解性病变,颞部病变患者的颅骨造影显示颅骨破坏。四岁的患者进行了Tc 99m的核骨扫描,发现下颌骨中放射性核素的摄取增加。所有患者均进行了切开活检,诊断为“朗格汉斯细胞组织细胞增生症”。内脏超声检查和影像学骨骼检查未显示多灶性软组织或骨骼受累的证据。治疗的选择是刮宫。他们的术后随访未见复发。

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