¤Ñ疱½H¡]Pemphigus¡^ ½²§eªÚÂå®v www.DrSkin.com.tw ¤Ñ疱½H¬O«ü¤@¸s¦]¬°¦ÛÅé§ÜÅé§Î¦¨¡A¨H¿n¦b²ÓMªí±©Î¶¡½è¤W¡A¤Þ°_ªí¥Ö²ÓM¶¡ÃP¸Ñ¡A¥B§í¨î·sªº²ÓM¶¡Ápµ²©Ò»ÝªºM¾ô¤pÅ餧§Î¦¨¡A¦]¦Ó§Î¦¨¤ôþQªº¦ÛÅé§K¬Ì©Ê¯e¯f¡C¦b¬ü°êµo¥Í²v¬°¬ü10¸U¤H¦³0.1¨ì0.5¡A¦bµS¤Ó¤H¸û°ª¡A¤é¥»ªº¬ã¨s¤¤»PHLA-B1507¦³Ãö¡C³oºØ§ÜÅé´NºÙ¬°¤Ñ疱½H§ÜÅé©Î²ÓM¶¡½è§ÜÅé¡]intercellular substance antibody, ICS antibody¡^¡A¥Dn§ðÀ»ªí¥Ö²ÓM¶¡ªºM¾ô¤pÅé²Ä¤@¤Î²Ä¤T§Üì(desmoglein 1 & 3)¡A¥i¥H¥«°âELISA¸Õ¾¯ÀË´ú(0282261889)¡A¨ä¥L§Üì¥]¬ADsg4¤Îacetylcholine receptor-alpha9 ¡C¨Ì¾Ú§ÜÅ骺¤£¦P¡A·|¯}Ãa¤£¦P²`«×ªºªí¥Ö¡A¤Þ°_¤£¦PªºÁ{§É¤Î¯f²zªí²{¡C¦p¥u¦³¹ï§ÜM¾ô¤pÅé²Ä¤@§ÜìªÌ§Î¦¨¸¨¸©Ê¤Ñ疱½H¡A¥u¦³¹ï§ÜM¾ô¤pÅé²Ä¤T§ÜìªÌ§Î¦¨¤fµÄÂH½¤©Ê¤ÑþQ½H¡A¦p¦P®É¹ï§ÜM¾ô¤pÅé²Ä¤@¤Î²Ä¤T§Üì¡A«h¤Þ°_´M±`©Ê¤Ñ疱½H¡CM¾ô¤pÅé²Ä¤@¤Î²Ä¤T§Üì¦b¥¿±`¥Ö½§¤À¥¬¿@«×¤£¦P¡A«eªÌ¥H¤WI³Ì°ª¡AÁy³¡¦¸¤§¡A¦Ó¤UI¡B¤fµÄ¡BÀY¥Ö¤Î¤U¸¡¸û§C¡A¦Ó«áªÌ«h¥H¤fµÄ³Ì°ª¡AÀY¥Ö¦¸¤§¡A¤UI³Ì§C¡A³o¨Ç®t²§¤]¥i¥H³¡¥÷¸ÑÄÀ¨äÁ{§Éªº¤£¦P(JID 1991:96:159)¡C¦bP¯f¾÷Âà¤W¡A´M±`©Ê¤Ñ疱½H¸û¯S§Oªº¬O¦å²G¤¤·|¦³½Õ¸`©ÊT²ÓM(Treg = CD4+CD25+)¼Æ¥Ø§C¤U±¡ªp¡A¦Ó¸¨¸©Ê¤Ñ疱½H«h¥¿±`¡C¤Ñ疱½H·|¦X¨Ö¨ä¥¦§K¬Ì©Ê¯e¯f¡A¦p¬õ´³©Ê¯T½H¡A°®Å~¡A¦b°ê¤ººaÁ`32¦ì´M±`©Ê¤Ñ疱½H±wªÌ¤¤´N¦³¨â¦ì¦³°®Å~¡A¦bªø©°ªº114¦ì±wªÌ¤¤«h¦³3¦ì¦³°®Å~¡C¤Ñ疱½H¥i¤À¬°¥H¤U´XºØ¡G 1. ´M±`¤Ñ疱½H¡]Pemphigus vulgaris¡^ ´M±`¤Ñ疱½H¬O¤@ºØ³Ì±`¨£ªº¤Ñ疱½H¡A¥Dn¦b45-60·³µo¯f¡A¦ý¤p«Ä¤]·|¦³¡A¨È¬w³ø§i¨k©Êµy¦h¡A¥Dn¦ÛÅé§Ü쬰DSG3ªºcis-adhesive interface on EC1 ¡C¯f¨_·|¦b¥Ö½§¤ÎÂH½¤¤W§Î¦¨¤ô疱¡C¤@¥b¥H¤Wªº¯f¤H¡A³Ì¦ªº¯f¨_¥X²{©ó¤fµÄÂH½¤¡A´XÓ¤ë«á¤~¦b¥Ö½§¥X²{¤ô疱¡AÂH½¤¯f¨_ªì¬OÁSÄê¤Î·|µh¡A¤£©ö¡¦XÁÙ·|¦V©P³ò½¯©µ¡A¬Æ¦Ü«I¥Ç¨ì¹¹D¡A¦³®É±wªÌ·|¥ýµo²{¶î¤f¬õ®É®e©ö¯}¥Ö¡C¦Ü©ó¥Ö½§ªº¯f¨_«h¥i¥H¬OÃP¦¢©Ê¤ô疱©ÎÁSÄê¡CY±N«üÀY«ö¦b¥¿±`¥~Æ[ªº¥Ö±¤W¡A¦V¤ô¥±¤è¦V¾A·í¥[À£¡A¤U¤èªºªí¥Ö¼h§Y·|·Æ°Ê¡BéÂ÷¡AÁ{§É¤WºÙ¬°Nikolsky¤ó²{¶H¡C ´M±`¤Ñ疱½Hªº¶EÂ_°£Á{§É¥~¡A»Ýn§@¥Ö½§ªº¤Á¤ù¤Î©â¦å¡C¤Á¤ù¥i¥H¬Ý¨ì¤ôþQ·|¦b°ò©³²ÓM¼h¤§¤W¤è§Î¦¨¡A¤]¥i¥H°»´ú¨ìªí¥Ö²Õ´¤º§K¬Ì²y³J¥Õ¡]¥Dn¬OIgG¡^¤Î¸ÉÅé¨Ò¦pC3¨H¿n¡C©â¦å«h·|µo²{¹ï§Üªí¥Ö²ÓM¶¡½èªº¤ÑþQ½H§ÜÅé¡C¤ÑþQ½H§ÜÅ骺¼ÆÈ°ª§C¡AÁÙ¥i§@¬°ªvÀøªº°Ñ¦Ò¡C¯e¯fÄY««×¥i¨Ì«I¥Ç½d³ò¤Î¯f¼éÀã¡B°®Àê¡Bªí¥Ö¦A¥Í»P§_§P©w¡AºÙ¬°ABSIS (Eur J Derm 17/1, 4-11, 2007)¡C ´M±`¤ÑþQ½HY¤£¸gªvÀø¡A¥i¯à¦³P©Rªº¦MÀI¡A¦b»OÆWºaÁ`²Îp¤W¦º¤`²v¬ù¬°1/4¡A¥Dn¦º¦]¬O·P¬V¡A¤×¨ä¬OºñÁw±ìµß¤Îª÷¶À¦â¸²µå²yµß¡A¥t¥~¤]·|¦³¥Õ¦â©À¯]µß·P¬V¡A¬ù¦bªvÀø«e3¨ì18¤ë¥X²{¡C¨ä¥¦¨Öµo¯g¥]¬A®wªY¤ó¼xÔ¸s¡B¯á´ÕÀ£¢©Ê°©§é¡B¤W®ø¤Æ¹D¥X¦å¡BµÇ°IºÜ¡B¨x¬r©Ê¡B§C³J¥Õ¦å¡B§C¦å¹[¡B°ª¦å¿}(¬Æ¦Ü¤Þµo¿}§¿¯f©ü°g)¡B°ª¦åÀ£¡B¸zG¯gª¬(®ø¤Æ¤£¨}¡Bäú¤ß¡B¸¡Âm)¡Bºë¯«¯gª¬(ļ°Ê¡B¥¢¯v¡B¿³¾Ä)¡BÁy¼é¬õ¡BÃö¸`¿n¤ô¡B¬õ¯l¡B¤ß±ª¡BÀYµh¡B¥X¦½¡B¨ýı²§±`¡B¦Ù¦×°©Àf»Äµh¡B¤ë¸g¥¢½Õµ¥¡A¡BÃþ©T¾J©Ê¨xª¢¡BÃþ©T¾J©Ê¦Ùª¢¡Bºë¯«¯f¡CªvÀø¥H°ª¾¯¶qÃþ©T¾JªvÀø¬°¥D¡A¦³¤TºØ¤è¦¡¡A¦Ü©ó¹w«á¡A²Îp¤WIndo-Asian¤ñ¥ÕºØ¤H¨Î¡Aªìµo®Éintercellular antibody titre°ª¡Aanti-Dsg 3°ª¡A¯e¯f½wÂà¸ûªø. ¡]1)«ùÄòÀøªk¡A1-1.5mg/kg/d, ¤@¯ë«Øij»Ý¤j©ó100mg¡A¯S§O¬O¦bªì´Á¡A¨C4¨ì7¤Ñ¥[¶q50%¡A¦pªG¶W¹L120mg«hÀ³¤À¨â¦¸§ë¤©¡AY¨â©P¤º¤£¦A¦³·s¤ôþQ§Î¦¨¡A´Nºâ¬O¤w¸g¦³®Ä±±¨î¡A¤§«á³vº¥´î¶q¦Üºû«ù¶q¡A±`»ÝªvÀø¦Ü¤Ö6¤ë¡A±`n¨ì1¦~¡A¨ä¤¤°ª¾¯¶q´Á¬ù¬°2¨ì3¤ë¡A³o¤]¬O±wªÌ³Ì¤£µÎªAªº®É´Á¡A´î¶q®É¨C14¤Ñ´î50%¡C¦pªG¥Î¶q°ª¹L1.5mg/kg/d¤´µL®Ä«hºÙsteroid recalcitrant¡C (2)¨Ï¥Î¯ß½Ä¦¡ªvÀø¡A¦pmethylprednisolone¨C¤Ñ500mg~1gmX 3~5¤Ñ¡A©Î2¨ì4¶g¤@¦¸cyclophosphamide(0.5-1.0 g/m2¡AY¥Õ¦å²y©ó2©P®É¤j©ó2000«h¨C¦¸¥[¶q100-250 mg¡A¤¤¶¡¨C¤Ñµ¹¤©50mg)ªºÀø®Ä»P°ª¾¯¶q¤fªAÃþ©T¾J¤jP¬Û¦P¡C ¡]3¡^¤¤¾¯¶qÃþ©T¾J¡A¤SºÙ¬°mini Lever regimen(Arch Dermatol. 1984 Jan;120(1):44-7)¡Aµ¹¤©¹j¤Ñ40mg¡Aªø´Á¼Æ¦~¨Ï¥Î¡C¥Dn¥Î©óÂH½¤¬°¥Dªº¯f¨_¡A©¹©¹¼Æ¤ë¤~ÂH½¤ªø¦n¡C ¨Ï¥ÎÃþ©T¾J®É¡A¦h·|¦X¨Ö¨ä¥¦§K¬Ì§í¨î¾¯¨Ò¦pazathioprine(1.5-2.5 mg/kg¡A©Î50 - 250 mg/day)¡]1994 IJD)¡BCellcept(2-3 gm/d)(JID 2010, ´_µo²v¤U°)¡Bmethotrexate(7.5 - 17.5 mg/week)¡Bcyclosporin(Arch Derm 2000)¡Bchlorambucil (4 increase to 10 mg/day)¡C¤@¥÷¤ñ¸û¬ã¨s¡A¤´¥Hazathioprine³Ì«Øij¡]J Am Acad Dermatol. 2007 Oct;57(4):622-8¡^¡C»PÃþ©T¾J¨Ö¥Î¥i¥H°§CÃþ©T¾Jªº»Ýn¾¯¶q¡A¦ý¥²¶·ª`·N·P¬Vªº¦MÀI©Ê·|¼W¥[¡A¤@¯ë¦bªì´Á¤w±±¨î¶}©l´îÃĮɵ¹¤©¡CÄY«Ó®×©Î¤w¨Ï¥Î¨ì2mg/kg/day©Î¶W¹L240mg¤´µLªk¦³®Ä±±¨î®É¡A°£§K¬Ì§í¨î¾¯¥~¤]¥i¥H¦Ò¼{plasmaphoresis²¾°£§ÜÅé¡B§K¬Ì²y³J¥Õ©Î¬OB²ÓM³æ®è§ÜÅérituximab¡A©Î¬O¦¹¨âºØÃĪ«¦X¨Öµ¹¤©¡Cplasmaphoresis¨C©P3¦¸¦@2©P¡A¨C¦¸¦Û¶O¬ù1¸U¤¸¡C§K¬Ì²y³J¥Õ¶O¥Î°ª©ù¡A¨C¤ë¬ù»Ý30¸U¤¸¡AÁöµM¤£¹³¨ä¥¦ªvÀø¤Þ°_·P¬V¡A¦ý¤´¥i¯à¤Þ°_¨u¨£ªº¬Æ¦ÜP¦ºªº¦åºÞ¤º·»¦å(¦å¤pªO§C¤U)¡A¦]¦¹¦pªGªvÀø«á¦³Iµh¡Bµo§NŸ§Ý¡Bµo¿N¡B§¿¦â§ïÅÜ¡B§¿¶qÅܤ֡BÅ髬ðµM¤W¤É¡B¤ô¥÷º¢¯d/¤ô¸~¤Î©I§lµu«P¡An¯S§O¤p¤ß¡C¦Ü©óRituximab¥Îªk¬°¨C©P¤@¦¸375mg/m2¦@¥|©P¡A(500mg/50ml/vial, IF once in NS 500ml for 240min, 2.0833 mg/min + Vena 30mg before)¡A©Î¬O(1 g intravenously on days 1 and 15, followed by 500mg intravenously if clinically warranted at 6¡Vmonth intervals or repeated full dosing). ¨C500mg¬ù»Ý4¸U¦]¦¹¨C¤ën16¸U¡A¥Ø«e¥¿¦¡®Ö·Ç¾AÀ³¯g¬°²O¤Ú½F¤ÎÃþ·Àã©ÊÃö¸`ª¢¡A¥Dn°Æ§@¥Î¬°·P¬V¡C³Ìªñ¦b°ê¥~µo²{¥Î©ó¬õ´³©Ê¯T½H®É¡A¦³¤Þ°_¤@ºØºÙ¬°¶i¦æ©Ê¦hµo¸£¥Õ½è¯fÅܪºP¦º·P¬V¡A©Ò¥H¦pªGªvÀø¤¤²£¥Í µøı²§±`¡B¥¿Å·P²§±`¡B¨ó½Õ»Ùê®É¡A¤@©wn¥ß§Y³B²z¡C°ê¥~¬ã¨s¤W¡A¤Ñ疱½HéwªvÀø«á2¡B5¡B10¦~«á½wÂà²v¤À§O¬°25%¡B50%¡B75%¡A¦pªG¤£¿n·¥Ãþ©T¾JªvÀø¡A¦º¤`²v¬ù¦³9¦¨¡C¦bªø´Á°lÂܤU¡A¬ù50%¥i§¹¥þ°±ÃÄ¡A¥t¥~1/3«h¥i¨Ï¥Î¤£¶W¹L10mg¨Óºû«ù¡A¦Ü©ó¥t¥~1¨ì2¦¨«h»Ýn¸û°ª¾¯¶q¡C¹ï©ó¨Ï¥ÎÃþ©T¾J¶W¹L3¤ëªÌ¡A¸É¥Rforsamax¨C¤Ñ10mg¥i´î¤Ö°©½è¬y¥¢¡A¤ñalfacalcidol¦³®Ä¡C¦å¤¤§ÜÅé¿@«×¶V°ª¡AªvÀø¶V§xÃø¡A¤]¦]¦¹¦í°|´Á¤]·|©µªø¡A¥§¡³£»Ýn¤@Ó¤ë¥H¤W¡C¥t¥~ÁקK§½³¡¥~'¶Ë¡A¦p¿iÀ¿¡B·P¬V¡A¤]¥i¥H´î¤Ö§½³¡¤ôþQ§Î¦¨¡C¦b¥~¥ÎªvÀø¤W¡BÃþ©T¾J¡B´¶¯S¥Ö³£¦³³¡¥÷®ÄªG¡A³Ìªñ¤]¦³³ø§iÁxÆP«P¶i¾¯pyridostigmine bromide (Mestinon𦲷, Valeant Pharmaceuticals)¤Î4% Pilocarpine¾®½¦ªº®ÄªG¡A¤]¥i±Ä¥Î§½³¡Ãþ©T¾Jª`®g¡A¿@«×¬°¥Ö½§5-10mg/mL¡A¤fµÄ«h¬°10-20 mg/mL¡A¨C©P©Î¹j©P¤@¦¸¡C¸û»´·L¯f¤H¤]¥i¹Á¸ÕDapsone¡Btetracycline¡B4%nicotinamide gel¡C¥t¥~ªvÀø°®Å~ªº¥Íª«»s¾¯Enbrel°ê¤º¥~¤]¦³¹s¬PªºÀø®Ä³ø§i¡A¨C¤ë¶O¥Î¬°4¸U¤¸¡C¥t¥~©â·Ï(¨C¤Ñ15®Ú)¤]´¿³Q³ø§i¥i§ïµ½¤fµÄ¯f¨_¡A¥i¯à¬O¦]¬°¨ä¤¤ªº¥§¥j¤B¡A¦P¼Ëªº§@¥Î¤]³Qµo²{¥i¥Î©ó¨ä¥¦¥Ö½§¯fªvÀø¡A¦p¼ìºÅ©Ê¤j¸zª¢(Ulcertive colotis)¡BÃa¯j©ÊÁw¥Ö¯f(pyoderma gangrenosum)¤Î¤fµÄ¼ìºÅ(aphthous ulcer)¡C ¦b¥xÆWªº¤Ñ¯p½H¦º¦]²Îp¡A¨Ì§Ç¬°ªÍª¢ (3.64; 95% CI, 1.30-10.21), ±Ñ¦å¯g (11.57; 95% CI, 2.95-45.34), ¤ß¦åºÞ¯e¯f(2.69; 95% CI, 1.18-6.12), ®ø¤Æ©Ê¼ìºÅ (8.44; 95% CI, 1.22-58.21). 2. ¼W´Þ©Ê¤Ñ疱½H¡]Pemphigus vegetans¡^ ¼W´Þ©Ê¤Ñ疱½H¬O´M±`¤Ñ疱½Hªº¤@ºØ¯S®í«¬ºA¡A¥i¦P®É¦X¦s¦b¡C¯f¨_§e²{ªÎ«p©Ê¼W´Þ©Î¦×ªÞ¸~¼Ë¼W¥Í¡C¦nµo©óªí¥ÖÂH½¤¥æ¬É³B¡A¹ï¿i³¡¤Î¤jÃö¸`¤§©}°¼¤ÎÀY¥Ö¡AªvÀø¤W»P´M±`¤Ñ疱½H¬Û¦P¡C 3. ¸ª¬¤Ñ疱½H¡]Pemphigus foliaceus¡^ ¸ª¬¤Ñ疱½H¬O¤@ºØªí²L©Êªº¤Ñ疱½H¡AӮפ@¯ë¤ñ´M±`¤Ñ疱½Hµy¤Ö¡A¦ý¦b¤Ú¦è¶m¤U¡B¬ð¥§¦è¨È¦~»´¤k©Ê¡B«n«D¶Â¤H«h¬O¨Ò¥~¡A^°ê¤û¬zªº²Îp¤]¬O¨âºØ¤Ñ疱½H¤H¼Æ¬Û¦ü¡C¤ô疱§Î¦¨ªº¦ì¸m¬O¦b¨¤½è¼h¤U¤è¡A¦]¬°¤ôþQªº³»±¬Û·íÁ¡¡A¤ô疱¤£©úÅã¥B©ö¯}¡A·|§Î¦¨¸¨®h©Îµ²¯mªºªí±¡A³q±`¥ÑÀY¥Ö¡BÁy³¡©MÂß·F¤§¤W¥b³¡¶}©lµo¥Í¡AµM«á½wºC¦a¶i¦æ¡A¦³®É¬Æ¦Ü¥i½¯©µ¥þ¨µo®i¦¨¬õ¥Ö¯g¡CÂH½¤ªº«I¥Ç¦b¸ª¬¤ÑþQ½H¤£±`¨£¡Cµo§@¦~ÄÖ¤ñ´M±`¤ÑþQ½Hµyªø¡A¬ù¦b60-70·³¡C¬ð¥§¦è¨È¬ã¨sµo²{¡ADRB1*03, DQB1*0302 ¤Î DRB1*04 ¹ï°¸°ò¦]¡A®e©ö²£¥Í¦¹¯f¡A¦ÓDRB1*11 ¤Î DQB1*0301 «h¨ã¦³«OÅ@¤O¡C ªvÀø¤W´M±`¤Ñ疱½H¬Û¦ü¡A¦ýÃþ©T¾J¤§¾¯¶q±`¸û§C¡C¶Ç²Î¤Wı±o¡«á¸û¨Î¡A¤£¿n·¥ªvÀøªÌ¦º¤`²v¦b2¨ì3¦¨¡A¦ý¦bºaÁ`ªº²Îp¤W¦º¤`²v»P´M±`¤Ñ疱½H¬Ûªñ(28% vs 23%)¡C 4. ¬õ´³©Ê¤Ñ疱½H¡]Pemphigus erythematosus¡^ ¬õ´³©Ê¤Ñ疱½H¥iµø¬°¬õ´³©Ê¤Ñ疱½Hªº¤@ºØ§½©Ê¨È«¬¡A°£¤F¤Ñ疱½H§ÜÅé¥~¡A¯f¤H¦å²MÁÙ¥i°»´ú¨ì§Ü®Ö§ÜÅé¡]antinuclear antibody¡^¡A¬G¦¹¯f¥iµø¬°¸ª¬¤Ñ疱½H¤Î¬õ´³©Ê¯T½Hºî¦Xªºªí²{¡CÁ{§É¯fÅܤ]¬O¦X¨Ö¦³¸ª¬¤Ñ疱½H¯f¨_¡A¦ý¸û§½¡A¥D©óÀY¡BÁy¤Î¤W¥b¨¤§¯Ý°©³¡¤ÎªÓK¶¡³¡¡CÁy³¡«h¦³Ãþ¦ü¯×º|©Ê¥Ö½§ª¢©Î¬õ´³©Ê¯T½H¤§¥~Æ[¡C¦b¯f²z¤W¤]¬O¦P®É¦³¸ª¬¤ÑþQ½HÅܤƤΰò©³½¤¼W«p¤Î¯u¥Ö²O¤Ú²y®û¼íµ¥Ãþ¦ü¬õ´³©Ê¯T½H¤§ÅܤơCªvÀø¤W«hÃþ¦ü¸ª¬¤ÑþQ½H¡C 5. ¦ñ¸~½F©Ê¤Ñ疱½H¡]Paraneoplastic pemphigus¡^ ³o¬O¤@ºØ¬Û·í¯S®íªº¤Ñ疱½H¡A¤SºÙ¬°¦ñ¸~½F©Ê¦ÛÅé§K¬Ì¦h¾¹©x¯gÔ¸sParaneoplastic autoimmune multiorgan syndrome¡A°£¤F¶Ç²Îªº¨âºØ¤Ñ疱½H§ÜÅé¥~¡AÁÙ·|¦³¦h¼Ë©Êªº¨ä¥Lªí¥Ö¦ÛÅé§ÜÅé¡A¤Î¦h¼Ë©Ê¥Ö½§ÅܤơA¦]¦¹Á{§É¤W¦³¤£¦Pªí²{¦p¦h§Î©Ê¬õ´³¡B¤ô疱¡B«ó¥aÄö¡B«üºÝÃûÄê¡A¦Ó¹êÅç«ÇÀˬd¤W¦¹¯e¯fªº§Ü줣©ódesmoglein 1&3¡A¦]¦¹DIF¤ÎIIF¤]¥i¥X²{¤£¦Pµ²ªG¡A¥]¬ADIF+/IIF-, DIF-/IIF-, DIF-/IIF+, DIF+/IIF-(JCP 2009;36:34)¡C¦¹¯f¹ïÖß½¤ªº«I¥Ç¤]§ó¬°©úÅã¡A¦pªì´Á±`³Q¶EÂ_¬°¤fµÄÃûÄê©Ê«ó¥aÄö¡AÁö·|¼vÅT¶i¹¡A¦ý§ó¤j°ÝÃD¬OªÍ³¡¤p¤ä®ðºÞ¡A¬ù¦³1/3·|¤Þ°_ªý¶ë¦ÓP©R¡A¦ý§ÜÅ饻¨«h¦ü¥G¨Ã«D¤Þ°_ªÍ³¡«I¥Ç¥D¦]¡C¨ä¾l¦º¦]¬°¸~½F¥»¨©Î·P¬V¡C¦h¼Æªº±wªÌ·|¦X¨Ö´c©Ê¸~½F¡A¤×¨ä¯Ý¸¢½F¡A¦ý¤Ö¼Æ«h¬O¥uµo²{¨}©Ê¸~½F¡A¯S§O¬OCastleman¤ó¯f¡A®e©ö¥H¸¡µÄ¸~½Fªí²{¡C¦ñ¸~½F©Ê¤Ñ疱½H¥H«e³Q»{¬°¬O¨u¨£¯e¯f¡A¦ýÀHµÛ¹ï¦¹¯e¯fªº¤F¸Ñ¡A¤Î´c©Ê¸~½Fªº¼W¥[¡AÁ{§ÉӮר䣨u¨£¡CªvÀø¤W¥Dn¬On±N¸~½F¤Á°£¡C 6. IgA«¬¤Ñ疱½H (IgA pemphigus) ³o¬O¤@ºØ¨u¨£ªº¤Ñ疱½H¡AÁ{§É¤W¥H¸s»Eªí²L©Ê¤pÁwþQ¨Óªí²{¡A¥Dn¤À¥¬¦b¹«Áѳ¡¡BµÅ¤U¤ÎÂß·F³¡¦ì¡A»PÁw¯p©Ê°®Å~¦³³¡¥÷¬Û¦ü¡CY¦P®É¦³IgA/IgG«hºÙ¬°IgA/IgG pustular pemphigus 7. 疱¯l¼Ë¤Ñ疱½H (Pemphigus herpetiformis) ³o¬O¤@ºØ§ó¬°¨u¨£ªº¤Ñ疱½H¡A1975¦~¤~³Q½T¶E¡AÁ{§É¤W¥H¸s»E¤p¤ôªw¨Óªí²{¡A¦³®É±Æ¦C¦¨Àô§Î¡A¥Dn¬°Äo·P¡C¦b¯f²z¤W¥H¶Ý¥ì¬õ²y©Ê²ÓM¶¡¤ô¸~ªí²{¡A±`±`¤£·|¦³¨å«¬ªí¥ÖÃP¸Ñ¡C¥Dn§Üì¬Odesmoglein 1¡A¤Ö¼Æ¬°desmoglein 3¡A§ó¤Ö¼Æ¬°¨ä¥L§Üì(DIF +/IIF+¦ýELISA for Dsg1&3-, JD 2010, 37:264)¡C¦³¨Çªí²{Á{§É¤W¥¼§Î¦¨¤ôªw¡A¦pEAC-like (erythema-annulare-like acantholytic dermatosis) ¦h¼Æ¤Ñ疱½Hì¦]¤£©ú¡A¦ý³¡¤À¹ª«¤¤ªº¤Æ¦Xª«¦b¾Ç²z¤W¥i¯à·|¤Þ°_¯e¯f´c¤Æ¡A³o¨Ç¹ª«¥]¬A:
- ½µæÃþ: onion, mustard, turnip, broccoli, radish, cabbage, cauliflower, potato, leek, shallots, chives, tomatoes, gingercassava, eggplant
- »®ÆÃþ¡Gginger, ginseng, garlic, red chillies, asofoetida, coriander, cumin, black pepper, ajwain
- ¤ôªG¤Î°íªGÃþ: mango, raspberry, pistachio, avocado, cherry, cashew, banana, cranberry, guarana, pear, blackberry, walnut, peach mango, cashew , guarana, raspberry, peach, grape. betel nut, kola nut, peanut, pistachio
- ²ÕÄZ¾¯¤Î¿³¾Ä¾¯: coffee, tea, betel nut leaf, katha, cassava
- ¶¼®Æ: beer, wine, soft drinks . cocoa,
- Âø¶µ: ice cream, candy, baked foods, spices (red chillies), aspartame, sodium benzoate, tartrazine, coloring agents, nutritional supplements
- ¶¼¥Î¤ô: high tannin content in Brazil river water may be the reason for endemicity of fogo selvagem. Tannins can be removed by chlorination, which would explain why the incidence of fogo selvagem has decreased with urbanization.
³o¨ó¹ª«©Ò¤Þµo¤Ñ疱½Hªº¥i¯àª«½è¤À§O¬°
- Thiols: Three thiol-containing compounds in garlic (allylmercaptan, allylmethylsulfide, allysulfide) have been found to induce acantholysis in vitro
- Isothiocyanates, present in mustard oil, are of two types - Immunologically reactive compounds (allyl and benzyl isothiocyanate) and irritant compounds (phenyl isothiocyanate)
- Phenols : Phenolic compounds such as urushiol are found in the Toxicodendron family of plants, which are known to cause allergic contact dermatitis and a similar sequence of events may be involved in the acantholysis of pemphigus. Mango, pistachio and cashew belong to the same family of plants. Other phenolic compounds include aspartame (an artificial sweetener), food additives (preservatives, coloring and flavoring agents) and cinnamic acid (present in cinnamon and apple, grape, orange, pineapple and tomato juices).
- Tannins: Tannins are naturally occurring plant polyphenolic compounds with considerable biologic activity. They have been shown to induce acantholysis in vitro, which can be blocked by anti IL-1£\ and anti TNF-£\ antibodies.
ÄY««×µû¦ôPDAI (pemphigus disease area index)Àu©ó ABSIS (autoimmune bullous skin disorder intensity score) Drug Type | Systemic Agent | Mode of Administration | Dose | Systemic Corticosteroids | Prednisone | Oral | 1-2mg/kg/d | Dexamethasone | Oral or IV pulse | 50-200mg/d for 3-5 d | Methylprednisolone | IV pulse | 500-1,000mg/d for 3-5 d | Immunosuppressive and Anti-inflammatory Agents | Azathioprine | Oral | 3-4mg/kg/d | Chlorambucil | Oral | 0.05-0.2mg/kg/d | Colchicine | Oral | 1.2-1.8mg/d | Cyclophosphamide | Oral | 2-3mg/kg/d | IV pulse | 0.5-1g/m2 monthly | Immunoablative high-dose (IV) | 50mg/kg/d for 4 d | Cyclosporine | Oral | 2-5mg/kg/d | Dapsone | Oral | 50-200mg/d | Erythromycin | Oral | 1,200mg/d | Gold | IM injection | 25-50mg/biweekly | Oral | 6-9mg/d | Leflunomide | Oral | 20mg/d | 20mg/d | Oral, SC, IM or IV | 5-30mg/wk | Minocycline | Oral | 100-200mg/d | Mycophenolate mofetil | Oral | 30-45mg/kg/d | Tetracycline +/- Nicotinamide | Oral | 1-2g/d 1,500-2,000mg/d | Biologic Agents | Etanercept | SC injection | 50mg weekly | Infliximab | IV infusion | 5mg/kg/cycle | Intravenous Immunoglobulin (IVIg) | IV infusion | 2g/kg/cycle | Rituximab | IV infusion | 375mg/m2 weekly for 4 weeks; OR 1,000mg on days 1 and 15† | Table 1: Therapeutic doses for immunomodulatory drugs used in the treatment of pemphigus.(Skin Therapy Letter) |
¦³¶i¤@¨B¬ÛÃö¸ê°T»Ý¨D¥i¥HÁpµ¸International Pemphigus Pemphigoid Foundation(IPPF) |