皮肤性病学-天疱疮

Pemphigus周英湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科PemphigusClinicalFeatures&PathogenesisEtiologyLaboratoryFindingsDiagnosisTreatmentDifferentialDiagnosis湘雅二医院皮肤性病科Structureofepidermis湘雅二医院皮肤性病科PhemphigusP.VulgarisP.VegetansP.FoliaceusP.ErythematosusP.HerpetiformisParaneoplasticPemphigus,PNP湘雅二医院皮肤性病科Middle-agedThin-walled,flaccid,easilyrupturedbullaeGeneralizedlesionBadconditionFatalPemphigusvulgaris湘雅二医院皮肤性病科Bullouslesionsarisingfromapparentlynormalskinsurface.BullaeErosionCrust湘雅二医院皮肤性病科Nikolsky’ssignTheupperlayersoftheepidermisareeasilymadetoslipbyslightpressureorrubbingbecauseoftheabsenceofcohesionintheskin.ItisalsoseeninStevens-Johnsonsyndrome,toxicepidermalnecrolysis,andepidermolysisbullosa.Phemphigus湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科Case132y/omanwithflaccidfluid-filledblisterfor2yearsandextensivepainfulerosionsfor2weeks.BeginwithOralerosion,progresstoinvolvethetrunkandextremities湘雅二医院皮肤性病科Case1PET38.3℃BP145／110mmHg，lesions湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科inthelowerepidermisIntraepidermalblisterandcleftformationAcantholyticcells湘雅二医院皮肤性病科acantholysisPhemphigus湘雅二医院皮肤性病科acantholyticorTzankcellsPhemphigus湘雅二医院皮肤性病科Pemphigusvegetans

Fungoidvegetationsorpapillomatousproliferations湘雅二医院皮肤性病科Sharplydelineatinglesions湘雅二医院皮肤性病科湘雅二医院皮肤性病科Groin,genitalia,perineum,axillaeareoftenaffected.湘雅二医院皮肤性病科perinealviewshowingcharacteristichypertrophicflexuralvegetations.Notetheintactvesicles.湘雅二医院皮肤性病科湘雅二医院皮肤性病科GeneralizedexfoliativeerythrodermaPemphigusFoliaceus

湘雅二医院皮肤性病科湘雅二医院皮肤性病科CircumscribedpatchesoferythemaandcrustingPemphigusErythmatosus

湘雅二医院皮肤性病科湘雅二医院皮肤性病科Thelesionsareusuallylocalizedoncheeks,foreheadandears.湘雅二医院皮肤性病科scarringanderythemaaffectingbothcheeks湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科inthegranularlayerorbeneaththestratumcorneum湘雅二医院皮肤性病科ETIOLOGYConceptofPemphigusIntraepidermalblistersAcantholysisNikolskysignAnautoimmunedermatoses??AntigenAntibodies湘雅二医院皮肤性病科tonofilament湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科AntigenDsg2:所有有桥粒的组织Dsg3:表皮深层，粘膜全层Dsg1:表皮或粘膜浅层湘雅二医院皮肤性病科AntigenPVAg:Dsg1,Dsg3;130KdaPFAg:Dsg1;160KdaCelladhesionmolecules湘雅二医院皮肤性病科AntibodiesDIF:IgG&C3IIF:CirculatingICantibodyAbtitersoftenparalleldiseaseactivity湘雅二医院皮肤性病科

DirectImmunofluorescentIgG&C3canbedetectedwithintheepidermaltissueintercellularantibodies湘雅二医院皮肤性病科Animalmodel湘雅二医院皮肤性病科Organculturemodel48hr72hr湘雅二医院皮肤性病科P模型中纤溶酶原激活剂活性尿激酶可引起棘层松解抗尿激酶抗体能抑制模型中的棘层松解能抑制纤溶酶活性的抑肽酶等可抑制棘层松解0.2万u/ml尿激酶48小时湘雅二医院皮肤性病科Pathogenesis天疱疮抗体角质形成细胞Dsg1/Dsg3纤溶蛋白酶原激活物纤维蛋白酶系统活化细胞间粘合物质降解棘刺松解湘雅二医院皮肤性病科临床表现组织病理免疫荧光诊断依据Diagnosis湘雅二医院皮肤性病科TreatmentCorticosteroidsImmunosuppressantsSupportivetherapyTopicaltreatment湘雅二医院皮肤性病科Treatment皮质类固醇激素用药原则早期使用足量控制酌情减量维持要长ImmunosuppressantsAZP(Azathioprine)CTX(Cyclophosphamide)MTX(Methotrexate)CsAMMF(Mycophenolatemofetil)FK506(Tacrolimus)Tripterygiumglycosides湘雅二医院皮肤性病科湘雅二医院皮肤性病科请思考：新的治疗方法？湘雅二医院皮肤性病科Treatment抑制纤溶酶活性止血芳酸0.5tid细胞水平针对抗原？针对产生抗体的B细胞克隆rituximab(Monoclonalanti-CD20antibody)；Infliximab造血干细胞移植湘雅二医院皮肤性病科针对抗体吸附特异性抗体？抑制抗体生成血浆置换、IVIg基因治疗关闭产生抗体的B细胞克隆的基因阻止DNA转录阻止mRNA翻译抗体湘雅二医院皮肤性病科Treatmentofcase1一般治疗及护理甲基强地松龙80mg/d罗氏芬、雷尼替丁、钙剂等局部处理：湿敷及搽药湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科湘雅二医院皮肤性病科家族性慢性良性天疱疮Benignfamilialchronicpemphigus湘雅二医院皮肤性病科家族性慢性良性天疱疮Benignfamilialchronicpemphigus湘雅二医院皮肤性病科家族性慢性良性天疱疮Benignfamilialchronicpemphigus湘雅二医院皮肤性病科疱疹样皮炎DermatitisHerpetiformis湘雅二医院皮肤性病科疱疹样皮炎DermatitisHerpetiformis湘雅二医院皮肤性病科疱疹样皮炎DermatitisHerpetiformis湘雅二医院皮肤性病科疱疹样皮炎DermatitisHerpetiformis湘雅二医院皮肤性病科妊娠疱疹Herpesgestationis湘雅二医院皮肤性病科DifferentialdiagnosisBullousPemphigoid湘雅二医院皮肤性病科TheelderlypeopleLarge,tensebullae;thedenudedareas:atendencytohealspontaneously.湘雅二医院皮肤性病科湘雅二医院皮肤性病科Groin,axillae,theflexorsurfacesoftheforearms湘雅二医院皮肤性病科湘雅二医院皮肤性病科Basementmembranezone,BMZ半桥粒透明层致密层致密下层湘雅二医院皮肤性病科BPAg1:230KDantigenassembledinmacromolecularaggregateswithinthehemidesmosomesBPAg2：180KDantigenAtransmembraneglycoproteinwithbothintracellularandextracellularrelationshipswiththehemidesmosomes.湘雅二医院皮肤性病科Subepidermalblisterwithinflammatoryinfiltrate(eosinophils)