口腔膜大类疾病.ppt

口腔粘膜大疱类疾病OralVesiculobullousDiseases,BACKTOINDEX,NEXT,总论,选择相应内容点击,BACK,天疱疮,瘢痕性类天疱疮,大疱性类天疱疮,其他大疱性疾病,本课小结,单纯疱疹Herpessimplex,NEXT,带状疱疹Herpeszoster,水痘Chickenpox,NEXT,多形红斑Erythemamultiform,药物过敏性口炎Allergicmedicamentosusstomatitis,NEXT,病毒感染性变态反应性自身免疫性创伤性斑纹类,NEXT,疱性损害,一.总论,BACK,疱的概念,疾病范畴,疱vesicle粘膜或皮肤内储存液体而成水疱waterblister疱内容物为浆液血疱bloodblister疱内容物为血液脓疱pustule疱内容物为脓液大疱bulla直径5mm,上皮内疱上皮下疱,NEXT,血疱Bloodblister,水疱Waterblister,NEXT,BACK,上皮下疱subepithelialbulla,上皮内疱intraepithelialbulla,二.天疱疮（pemphigus）,1.概述(preface),严重(severity)致死性疾病激素(corticosteroid)的使用降低了死亡率,NEXT,NEXT,慢性(chronicity)不易愈合反复发作皮肤粘膜联合疾病(skin-mucosaassociateddisease)寻常型口腔损害多见其他型口腔损害少见,分类classification,寻常型pemphigusvulgaris增殖型pemphigusvegetans叶型pemphigusfoliaceus红斑型pemphiguserythematous,NEXT,2.病因(pathogeny)不明自身免疫性疾病autoimmunedisease抗棘细胞层间粘合物质的自身抗体3.临床表现(clinicalmanifestation),NEXT,NEXT,4.病理(pathology)棘层松解上皮内疱棘层松解细胞,NEXT,5.诊断(diagnose)1）病史2）临床表现寻常型：口腔损害最早最重增殖型：疱底肉芽组织增殖叶型：皮损为主松弛大疱鳞屑痂边翘如叶红斑型：皮损为主面部对称红斑鳞屑痂,NEXT,3）特殊检查活检细胞学：涂片寻找棘层松解细胞（天疱疮细胞）免疫荧光法：直接法间接法显示棘细胞层间抗细胞粘合物质的抗体4）全身情况：恶病质,NEXT,6.鉴别诊断(differentialdiagnoses),NEXT,7.治疗(treatment),支持疗法：高蛋白高维生素饮食水电解质平衡输血皮质激素首选规则用药：起始控制减量维持加用免疫抑制剂可减少激素用量加用抗生素预防继发感染注意禁忌证和不良反应,NEXT,局部：0.5达克罗宁止痛皮质散消炎中医中药,NEXT,增殖性天疱疮治疗前后,BACK,三.瘢痕性类天疱疮（cicatricialpemphigoid),概述良性粘膜类天疱疮benignmucosamembranepemphigoid病因自身免疫性疾病抗基底膜抗体,NEXT,临床表现病理：上皮下疱无棘层松解免疫荧光直接法基底膜区连续细长荧光带,NEXT,5.诊断临床表现活检免疫荧光法6.鉴别诊断寻常型天疱疮多形渗出性红斑糜烂性扁平苔癣,NEXT,7.治疗,局部：抗炎抗粘连全身：激素红霉素,BACK,四.大疱性类天疱疮（bullouspemphigoid）,1.概述病程长，预后好2.病因自身免疫性疾病抗基底膜抗体,NEXT,3.临床表现口腔：少见疱小不易破尼氏征()颊部多见皮肤：外观正常皮肤或红斑的基础上起大疱尼氏征(),NEXT,大疱性类天疱疮,NEXT,大疱性类天疱疮,NEXT,4.病理上皮下疱无棘层松解连续细长荧光带（直接免疫荧光）5.诊断：皮肤大疱免疫荧光,NEXT,6.鉴别诊断,NEXT,7.治疗,激素氨苯砜四环素烟酰胺联合,BACK,五.其他大疱性疾病,（一）类天疱疮样扁平苔藓（lichenplanuspemphigoides，LPP）同时有典型的LP、BP特征又有独特的抗原结构分子,NEXT,1.临床表现口腔：紧张性大疱网状白条纹皮肤：与大疱性类天疱疮相似尼氏征(),NEXT,2.诊断与鉴别无公认诊断标准有典型LPBP特征。基底膜带有IgG、C3线状沉淀，IIF血清中与抗基底膜带抗原结合沉淀鉴别：大疱性扁平苔藓大疱性类天疱疮大疱性红斑狼疮大疱性表皮松解症,NEXT,3.治疗,强的松硫唑嘌呤灰黄霉素,NEXT,（二）副肿瘤性天疱疮(paraneoplasticpemphigus,PNP)体内肿瘤的循环体液因子或其代谢产物的作用诱发天疱疮副肿瘤性天疱疮（三）线性IgA大疱性皮肤病无密集的嗜酸性粒细胞浸润带有微脓肿和纤维蛋白沉淀,BACK,SummaryLiketheskin,theoralmucosamanifestsavarietyofvesiculobullousdiseases（大疱类疾病）.Thesevaryinfrequency,severity,andsystemicconsequence.Themostimportantvesiculobullousdiseasesare“pemphigus”（天疱疮）and“pemphigoid”（类天疱疮）.,NEXT,Pemphigusvulgaris（寻常型天疱疮）isaseriouschronicvesiculobullousdiseaseofmucosaandskin.Thediseaseisseenalmosttotallyinadultsovertheageof60years.Thespecificcauseofthediseaseispoorlyunderstood.However,autoimmunereaction（自身免疫）isthoughttobeimportantinthepathogenesisofit.,NEXT,Anautoimmunemechanismissuggestedbythefindingofantibodiesinthelesionsofskinandmucasalocalizedintheintercellular（细胞间）spacesbetweenepithelialcells.Thepresenceofantidesmosomalantibodies（抗桥粒抗体）resultsinthedissolutionofintercellularcementingsubstance（细胞间粘合物质）.Thisprocessresultsinintraepithelial（上皮内）separationofthestratumgerminativum（基底层）fromtheunderlyingstratumspinosum（棘层）,withsubsequentacantholysis（棘层松解）andlossofepithelialintegrity.,NEXT,Orallesionsarepresentinalmostallcasesofpemphigusvulgarisandoftenprecedeskininvolvement.Anyareaofthemouthmaybeinvolved,includingmucosalsurfacesandgingivae.Inseverecasesalmosttheentireoralmucasamaybeaffected,withvesiculobullouslesionsinvariousstagesofdevelopment,regression,andhealing.,NEXT,Theskinlesionsareinitiallyvesiculobullouseruptionsthatsubsequentlyulcerateandarethenpronetosecondaryinfectionandpoorhealing.Diagnosisofthediseaseisbasedonhistory,clinicalexamination,cytologicsmears（细胞学涂片）,biopsy,andimmunofluorescent（免疫荧光）techniques.,NEXT,Althoughthereisnocureforpemphigusvulgaris,thediseasecanusuallybesuccessfullycontrolledwithimmunosuppressivedrugssuchasprednisone（强的松）.Butthesedrugsmayproducesignificantsideeffects.Localoralsymptomscanbecontrolledwithpalliativerinsesorointment,suchasdyclonine（达克罗宁）andbenzocaine（苯佐卡因）inorabase.,NEXT,Pemphigoid(类天疱疮)includescicatricialpemphigoidandbullouspemphigoid.Theyarealsoautoimmunediseases.Unlikepemphigus,theantibodiesaredemonstratedintheareaoftheepithelialbasementmembrane.Consequently,subepithelialvesiculobullouslesionarise,inwhichseparationisnotedbetweentheepitheliumandtheunderlyingconnectivetissue.,NEXT,本课小结,天疱疮的发病机制天疱疮和类天疱疮的临床分型及其表现特征天疱疮的病理基础口腔粘膜大疱类疾病的皮肤损害，建立粘膜皮肤联发疾病概念鉴别诊断皮质激素在天疱疮和类天疱疮治疗中的应用方法、效果、注意事项,BACK,天疱疮pemphigus,NEXT,天疱疮pemphigus,NEXT,天疱疮pemphigus,揭皮试验(),BACK,天疱疮pemphigus,BACK,BACK,增殖性天疱疮Pemphigusvegetans,BACK,棘层松解（400）acantholysis,BACK,上皮内疱（120）Intraepithelialbulla,BACK,棘层松解细胞（2000）Acantholyticcell,BACK,上皮下疱（100）Subepithelialbulla,BACK,天疱疮免疫荧光Immunofluorescenceofspecimenfromthelesionsofpemphigusvulgaris,BACK,类天疱疮免疫荧光Immunofluorescenceofspecimenfromthelesionsofpemphigoid,口腔龈：剥脱性龈炎尼氏征()悬雍垂、软腭、扁桃体、舌腭弓