bullous skin diseases - shanyar：大疱性皮肤病shanyar

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1、BullousSkinDisorders(BSD)Assistprof.Dr.AlielethawiSpecialistdermatologistC.A.B.D,F.I.C.M.SINTRODUCTIONBSDareskinconditionscharacterisedbyblisterformation.Ablisterisanaccumulationoffluidbetweencellsoftheepidermisorupperdermis.Causesofblistercouldbegenetic,physical,infla

2、mmatory,immunologicandasareactiontodrugs.BSDsaremostlyautoimmune.PATHOPHYSIOLOGYThekeratinocytesoftheepidermisaretightlyboundtogetherbydesmosomesandintercellularsubstoformabarrierofhightensilestrengthandstability.Beneaththeepidermisliesthebasementmembranezone(BMZ),whic

3、hisaspecialisedareaofcell-extracellularmatrixadhesion.Specialisedstructurestraversingthiszoneanchortheepidermistothedermis.TheBMZisparticularlyvulnerabletodamageormalformationandisacommonsiteofblisterformationTypes:GeneticBlisteringDiseases:A.EpidermolysisBullosaB.Hail

4、ey-Haileydisease(Benignfamilialpemphigus)2.ImmunobullousDiseases:A.IntraepidermalImmunobullousDiseases:1.PemphigusVulgaris(PV)2.Pemphigusvegetans.3.Pemphigusfoliaceus4.Pemphiguserythematosus5.ParaneoplasticPB.SubepidermalImmunobullousDiseases:1.BullousPemphigoid4.Pemph

5、igoidGestations2.LinearIgAdisease5.EpidermolysisBullosaAcquisita3.DermatitisHerpitiformsIMMUNOLOGICBULLOUSSKINDis.Theseincludes:PemphigusPemphigoidDermatitisHerpetiformis(DH)Chronicdermatosesofchildhood(linearIgAdis.)PEMPHIGUSisderivedfromtheGreekwordpemphixmeaningbubb

6、leorblister.Aserious,acuteorchronic,bullousautoimmunediseaseofskinandmucousmembranesbasedonacantholysis.Itisasevereandpotentiallylifethreateningdiseases.Typesincludes;P.vulgaris,vegetans,foliaceus,erythematosus,andparaneoplasticaEpidemiologyoccurworldwide.PVincidenceva

7、riesfrom0.5-3.2casesper100,000.morecommoninJewishandpeopleofMediterraneandescentorIndianoriginCommoninthemiddleagegroups(40-60yrsoflife)menandwomenequallyaffectedAETIOLOGYItisanautoimmunedis.inwhichpathogenicIgGantibodiesbindstoantigenswithintheepidermisThemainAgsarede

8、smoglein1and3(3inPV&1inPF).BothareadhesionmoleculesfoundinthedesmosomesTheAg-Abreactioninterfereswithadhesion,causing