黑色素斑胃肠息肉综合征1例

黑色素斑胃肠息肉综合征,又名Peutz-Jeghers综合征,简称PJS,是一种常染色体显性遗传疾病,主要表现粘膜皮肤黑色素沉着和胃肠道息肉。国内报导40例左右。现将我们诊治一例报告如下。患者,女性,21岁,学生。下唇变黑10年,近期影响美观要求诊治。专科检查:鼻、眶下缘、耳屏前和口周皮肤可见散在淡褐色斑点。下唇唇红、颊粘膜、磨牙后区和软腭可见淡褐色或黑色斑。大小1～5mm,形状呈点状、圆形、椭圆形和不规则形,界限清楚。手掌足底亦可见黑斑。颊粘膜黑斑活检见上皮细胞层黑色素颗粒增加,上消化道纤维内窥镜检查见慢性浅表性胃炎。结肠钡剂灌…     

黑色素斑-胃肠息肉综合征1例

黑色素斑-胃肠息肉综台征,又名Peutz-Jeghers氏综合征,简称PJS[1],由Peulz氏于1921年首先报道.     

以Laugier-Hunziker综合征为例谈口腔黏膜色素沉着异常类疾病的鉴别诊断

口腔黏膜色素沉着异常类疾病临床上较为常见,鉴别诊断对于正确诊断和治疗十分重要。文章以1例Laugier-Hunziker综合征（LHS）病例的诊断为例,从临床表现、皮肤镜、实验室检查和病理学检查等方面着手,探讨口腔黏膜色素沉着异常类疾病的鉴别诊断,以期为临床医生提供帮助。     

克罗恩病患者口腔黏膜损害研究

目的：探讨口腔黏膜病变在克罗恩病（CD）患者中的临床特征。方法：将诊断为CD的59例患者由消化内科医师分为口腔型及非口腔型,再经口腔科医师对全部CD患者的口腔黏膜情况进行专科检查,分析CD患者中的口腔黏膜病变特征,并对两组之间的肠道病变部位进行分析研究。结果：59例CD患者中口腔型CD（23／32）比非口腔型CD（7／27）有更多的病例表现为口腔方面症状,在32例口腔型CD患者中,最常见的口腔黏膜损害为卡他性牙龈炎（n＝19）,其次为复发性阿弗他溃疡（n＝8）,肉芽肿性病变（n＝4）,鹅卵石样改变（n＝3）,唇炎（n＝3）,增殖性化脓性口腔炎（n＝1）。口腔型CD患者合并有肛周疾病者占53％（17／32）,多于非口腔型CD患者的22％（6／27）。结论：口腔症状在CD患者的临床表现中占有较大比例,其中最为常见的口腔表现为卡他性牙龈炎,有口腔症状的CD患者更有可能合并有肛周病变。     

黑色素斑肠息肉综合征1例

黑色素斑肠息肉综合征又称普-杰氏综合征,是一种遗传性疾病,属于常染色体遗传性疾病.表现为黏膜皮肤色素沉着与肠息肉形成,表现为唇黏膜有大小不等的色素沉着斑,颊、舌、龈、腭亦可发病,色素斑呈暗褐色或灰黑色,形状为圆形、椭圆形或不规则形,数目不定.肠息肉可发生在整个肠道,以小肠最多见.我科收治1例,报告如下.     

Gardner综合征诊疗现状及进展

Gardner综合征是口腔颌面部疾病中比较罕见的一类疾病,以多发结直肠道息肉、多发性骨瘤、皮肤和软组织肿瘤三联征为特征.其早期治疗有更好的预后,因此早期诊断非常重要.Gardner综合征早期病变一般在口腔颌面部表现出来,因此,口腔医生在本病的早期诊断中扮演了重要角色.本文总结了Gardner综合征与基因突变相关的病因研究,并且归纳了Gardner综合征以口腔颌面部畸形、多发胃肠道息肉以及硬纤维瘤为特点的临床表现及其相关治疗的进展,综述如下.     

口腔黏膜恶性黑色素瘤1例病例报告及文献复习

目的探讨口腔黏膜恶性黑色素瘤的临床诊断及鉴别诊断要点。方法主诉拔牙后持续牙龈出血患者1例,其临床表现为口腔黏膜大面积黑色病损,经全面临床检查结合常规病理及免疫组织化学检查明确诊断。结果临床表现、病理检查和免疫组织化学检查证实为口腔黏膜恶性黑色素瘤。结论对发生于口腔黏膜的黑色损害特别是进展迅速且有破溃出血者应高度警惕口腔黏膜恶性黑色素瘤的可能,并结合临床表现、病理表现及免疫组织化学进行早期诊断和鉴别诊断。     

Peutz Jeher’s综合征2例报告

Ｐｅｕｔｚ　Ｊｅｈｅｒ’ｓ综合征２例报告天津市汉沽医院口腔科尹世桓天津市汉沽医院内科王桂兰ＰｅｕｔｚＪｅｈｅｒ’ｓ综合证又称黑色素斑—胃肠道多发性息肉综合征，较为少见，本文报告２例，为同一家族中母子两人患病，报告如下。例１：徐×，女，３８岁，因口腔及...     

深圳市口腔黏膜病患者1387例临床分析

目的：分析深圳市口腔黏膜病的发病特点和发病规律。方法：选择我院口腔门诊初诊的口腔黏膜病患者1387例,收集临床资料,并对病种、主诉及性别构成比等进行统计分析。结果：共统计出16种口腔黏膜病,前5位分别是复发性口疮（32.88%）、口腔扁平苔藓（14.42%）、念珠菌性口炎（10.24%）、慢性唇炎（9.23%）和灼口综合征（8.80%）。主诉依次为溃疡或溃烂（68.20%）、疼痛或肿胀（11.82%）、肿物（10.74%）、斑纹（6.85%）、健康检查（1.01%）,其他（1.37%）。复发性口疮、口腔扁平苔藓、慢性唇炎及灼口综合征,女性明显多于男性（P〈0.05）,白斑、白角化病男性明显多于女性（P〈0.05）。结论：念珠菌性口腔炎可能是深圳市老年患者最常见的口腔黏膜病。     

口周色素沉着—肠息肉综合征

目的：报道4例口周色素沉着－肠息肉综合征病例的临床表现，病理特征，及对家族史进行追踪观察。方法：通过临床观察，X线检查，病理诊断，家族史作出分析。结果：口周色素沉着－肠息肉综合征具有家族遗传现象，肠道病变部位与恶有相关性。结论：发现病症需作家族遗传学调查，病变发生在结肠者为高危区，需作长期定时监护。     

Peutz-Jeghers syndrome in a 14-year-old boy: case report and review of the literature

Peutz-Jeghers syndrome (PSJ) is a relatively rare but well-recognized condition, with a prevalence of approximately one in 120,000 births in the USA. It is generally inherited as an autosomal dominant trait, although 35% of cases are new mutations. This disorder is characterized by melanocytic macules on the hands, feet, peri-oral skin and oral mucosa, and multiple gastrointestinal hamartomatous polyps. People with PSJ have an increased risk for developing a variety of malignant tumours. The aim of the present study was to report one case of PSJ in a 14-year-old boy with mucocutaneous pigmentation associated with duodenal hamartomatous polyps.     

Peutz-Jeghers syndrome (mucocutaneous melanosis associated with gastrointestinal polyposis). Presentation of 5 cases presenting in the same family

Five brothers suffering from Peutz-Jeghers syndrome with freckles in the skin of the face and mucous membrane of the oral cavity, and intestinal polyps are reported. These patients have not been traditionally considered of being at risk. The current opinion is that patients with this disorder can develop malignancies in the gastrointestinal tract, and tumors in other organs.     

Oral pigmented lesions: Clinicopathologic features and review of the literature

Diagnosis of pigmented lesions of the oral cavity and perioral tissues is challenging. Even though epidemiology may be of some help in orientating the clinician and even though some lesions may confidently be diagnosed on clinical grounds alone, the definitive diagnosis usually requires histopathologic evaluation. Oral pigmentation can be physiological or pathological, and exogenous or endogenous. Color, location, distribution, and duration as well as drugs use, family history, and change in pattern are important for the differential diagnosis. Dark or black pigmented lesions can be focal, multifocal or diffuse macules, including entities such as racial pigmentation, melanotic macule, melanocytic nevus, blue nevus, smoker’s melanosis, oral melanoacanthoma, pigmentation by foreign bodies or induced by drugs, Peutz-Jeghers syndrome, Addison´s disease and oral melanoma. The aim of this review is to present the main oral black lesions contributing to better approach of the patients. Key words:Pigmentation, melanin, oral, diagnosis, management.     

Peutz-Jeghers syndrome and cancer

A 27-year-old white woman with Peutz-Jeghers syndrome (PJS) associated with a rare but distinctive ovarian tumor, the sex cord tumor with annular tubules (SCTAT), is discussed. Pertinent oral manifestations of PJS and chemotherapy complications are illustrated by the case report. Traditionally, patients exhibiting PJS have not been considered at increased risk of cancer development. This case, coupled with a review of the recent literature, suggests that these patients are, indeed, at increased risk of malignant lesions of several organ systems.     

以口腔表征为首发的血液系统疾病500例临床分析

目的： 探讨以口腔表征为首发症状的血液系统疾病的临床特点,为早期诊断,防止漏诊误诊提供依据。方法： 回顾近5年因口腔症状首诊于上海交通大学医学院附属第九人民医院口腔相关科室（口腔黏膜病科、牙周病科、牙体牙髓病科和口腔颌面-头颈肿瘤科）的500例血液系统疾病患者的临床资料,分析其口腔表征的多样性及分布情况。结果： 500例首诊于口腔各科的血液系统疾病患者的口腔表征有口腔黏膜和牙龈出血、牙龈增生、口腔黏膜溃疡、口腔黏膜感染及口腔颌面部肿块。其中以口腔颌面部肿块症状首诊者数量居首,占19.8%。血常规、凝血机制和口腔病理活检对血液系统疾病的初步筛查与确诊至关重要。结论： 多种血液系统疾病都可能在疾病初期就出现口腔表征,正确认识血液系统疾病多样性的口腔表征,结合血常规和凝血机制检查以初步筛选血液系统疾病;对表现为口腔颌面部肿块的患者进行肿块活检,可在临床上更早地对血液系统疾病进行诊断和鉴别诊断,从而避免误诊和漏诊。口腔表征的治疗以针对血液系统疾病为主,口腔局部对症治疗为辅。     

Gardner syndrome with maxillofacial manifestation: A case report

Gardner syndrome is a hereditary disease in which patients develop gastrointestinal polyps, osteomas, desmoid tumors, epidermoid cysts, fibromas, lipomas, and retinal lesions. Dental abnormalities such as supernumerary or impacted teeth, odontomas and dentigerous cysts are also reported. The most serious concern in this syndrome is the extremely high risk of gastrointestinal polyps undergoing malignant transformation. Since the maxillofacial findings usually precede gastrointestinal polyps, the dentist plays a crucial role in the diagnosis of Gardner syndrome, and panoramic radiography is an important tool in the diagnosis of the disease. We report here a case of Gardner syndrome in a patient showing mandibular osteomas and impacted teeth. Also, cases of Gardner syndrome with maxillofacial manifestations reported in the literature were reviewed and compared with ours. According to the findings, osteomas are important manifestations of this syndrome, and regardless of the absence of family history of intestinal polyposis, their occurrence should prompt diagnostic evaluation for this disease.     

Oral mucosal hyperpigmentation secondary to antimalarial drug therapy

A case of oral mucosal hyperpigmentation resulting from antimalarial drug therapy is presented. The patient reported a history of long-term quinacrine therapy and exhibited diffuse blue-gray pigmentation of the nail beds and the skin of the nasal ala. Microscopic examination of the involved mucosa showed macrophages, containing both melanin and ferric iron, scattered within the connective tissue adjacent to the epithelium. The clinical, historical, and microscopic features of antimalarial-induced pigmentation are discussed. Other causes of diffuse or multifocal oral pigmentation are also addressed.     

Facial and perioral molluscum contagiosum as a manifestation of HIV infection

Molluscum contagiosum (MC) is a self-limiting viral disease of the skin and the mucous membranes. Facial and perioral MC is seen with increasing frequency in human immunodeficiency (HIV) infection, particularly in HIV infected homosexual men. The purpose of this study was to describe clinical observations of facial and perioral MC in HIV infected patients. One hundred and eighty HIV-positive individuals (160 males and 20 females) were examined over a period of five years. Fifty-eight were homosexual men and 19 were bisexual men. Fifty-one of 180 patients at the time of the first examination had CD+4 count < 200 cells/mm3 and another 63 presented loss of CD+4 cells in this level, during this period. Three HIV infected patients (two homosexual and one bisexual) were affected with facial and perioral MC. At the time of MC diagnosis the CD+4 count was less than 200 cells/mm3 for all three patients. One patient died nine months after MC diagnosis and the other two are still alive. It is remarkable that in this study no clinical lesions were observed on other sites of the skin.