大疱类疾病

口腔粘膜大疱类疾病OralVesiculobullousDiseasesBACKTOINDEXNEXT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HerpessimplexNEXT带状疱疹Herpeszoster水痘ChickenpoxNEXT多形红斑Erythemamultiform药物过敏性口炎AllergicmedicamentosusstomatitisNEXT病毒感染性变态反应性自身免疫性创伤性斑纹类单纯疱疹带状疱疹、水痘药疹接触性口炎多型红斑（斯—约综合征）天疱疮类天疱疮寻常型增殖型叶型红斑型瘢痕性大疱性扁平苔藓盘状红斑狼疮粘膜血疱NEXT疱性损害一.总论BACK2.疱的概念1.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WaterblisterNEXTBACK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NEXTNEXT•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pemphiguserythematousNEXT2.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)NEXTNEXT鼻、眼、生殖器、肛门早期少突发尼氏征(+)摩擦区多种病损并存自觉症状早常见口腔各部位水疱糜烂尼氏征(+)寻常型/面部蝶型红斑少见红斑型眼、生殖器鳞屑痂边缘翘起如叶正常或轻微红肿、糜烂叶型鼻、生殖器早期发生腋脐肛门乳头状增殖尼氏征(+)唇红缘增殖尼氏征(+)增殖型其他皮肤口腔分型4.病理(pathology)棘层松解上皮内疱棘层松解细胞NEXT5.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NEXT3）特殊检查a.活检b.细胞学：涂片寻找棘层松解细胞（天疱疮细胞）c.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NEXT6.鉴别诊断(differentialdiagnoses)天疱疮多形红斑剥脱性龈炎大疱性表皮松解症疾病归类临床表现好发部位全身情况NEXT7.治疗(treatment)1)支持疗法：高蛋白高维生素饮食水电解质平衡输血2)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NEXT3)局部：0.5％达克罗宁止痛皮质散消炎4)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)1.概述良性粘膜类天疱疮benignmucosamembranepemphigoid2.病因自身免疫性疾病抗基底膜抗体NEXT3.临床表现4.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NEXT5.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NEXT7.治疗1)局部：抗炎抗粘连2)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NEXT3.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NEXT4.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NEXT6.鉴别诊断大疱类天疱疮瘢痕类天疱疮寻常型天疱疮大疱性表皮松解症病因临床病理预后NEXT7.治疗1.激素2.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NEXT1.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(－)NEXT2.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NEXT3.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BACKSummaryLiketheskin,theoralmucosamanifestsavarietyofvesiculobullousdiseases（大疱类疾病）.Thesevaryinfrequency,severity,andsystemicconsequence.Themostimportantvesiculobullousdiseasesare“pemphigus”（天疱疮）and“pemphigoid”（类天疱疮）.NEXTPemphigusvulgaris（寻常型天疱疮）isaseriouschronicvesiculobullousdiseaseofmucosaandskin.Thediseaseisseenalmosttotallyinadultsovertheageof60years.Thespecificcauseofthediseaseispoorlyunderstood.However,autoimmunereaction（自身免疫）isthoughttobeimportantinthepathogenesisofit.NEXTAnautoimmunemechanismissuggestedbythefindingofantibodiesinthelesionsofskinandmucasalocalizedintheintercellular（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.NEXTOrallesionsarepresentinalmostallcasesofpemphigusvulgarisandoftenprecedeskininvolvement.Anyareaofthemouthmaybeinvolved,includingmucosalsurfacesandgingivae.Inseverecasesalmosttheentireoralmucasamaybeaffected,withvesiculobullouslesionsinvariousstagesofdevelopment,regression,andhealing.NEXTTheskinlesionsareinitiallyvesiculobullouseruptionsthatsubsequentlyulcerateandarethenpronetosecondaryinfectionandpoorhealing.Diagnosisofthediseaseisbasedonhistory,clinicalexamination,cytologicsmears（细胞学涂片）,biopsy,andimmunofluorescent（免疫荧光）techniques.NEXTAlthoughthereisnocureforpemphigusvulgaris,thediseasecanusuallybesuccessfullycontrolledwithimmunosuppressivedrugssuchasprednisone（强的松）.Butthesedrugsmayproducesignificantsideeffects.Localoralsymptomscanbecontrolledwithpalliativerinsesorointment,suchasdyclonine（达克罗宁）andbenzocaine（苯佐卡因）inorabase.NEXTPemphigoid(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pemphigusNEXT天疱疮pemphigusNEXT天疱疮pemphigus揭皮试验(＋)BACK天疱疮pemphigusBACKBACK增殖性天疱疮PemphigusvegetansBACK棘层松解（×400）acantholysisBACK上皮内疱（×120）IntraepithelialbullaBACK棘层松解细胞（×2000）AcantholyticcellBACK上皮下疱（×100）SubepithelialbullaBACK天疱疮免疫荧光ImmunofluorescenceofspecimenfromthelesionsofpemphigusvulgarisBACK类天疱疮免疫荧光Immunofluorescenceofspecimenfromthelesionsofpemphigoid1)口腔龈：剥脱性龈炎尼氏征(－)悬雍垂、软腭、扁桃体、舌腭弓等瘢痕性粘连畸形2)眼单纯性结膜炎睑球粘连泪管阻塞3)皮肤好发于面部头皮水疱疱壁厚不易破尼氏征(－)4)其他咽气管尿道生殖器肛门瘢痕粘连BACK瘢痕性类天疱疮BACK尼氏征，即尼科尔斯基（Nikolsky）征由于天疱疮是棘层松解的结果，因此用手指轻推外表正常的皮肤与粘膜，即可迅速形成水疱，或使原有的水疱在皮肤上扩展。这种现象称为尼氏征阳性。BACK激素禁忌证：高血压糖尿病骨质疏松