ÀàÌìðå´¯Ñù±âƽ̦޺¼°¸±Ö×ÁöÐÔÌìðå´¯_ҩѧרҵ±ÏÒµÂÛÎÄ·¶ÎÄ
·¢²¼Ê±¼ä£º
2014-11-16
À´Ô´£º
ÈË´ó¾¼ÃÂÛ̳
ÀàÌìðå´¯Ñù±âƽ̦޺¼°¸±Ö×ÁöÐÔÌìðå´¯_ҩѧרҵ±ÏÒµÂÛÎÄ·¶ÎÄ
Ò»¡¢ÀàÌìðå´¯Ñù±âƽ̦޺£Û1-7£Ý
ÀàÌìðå´¯Ñù±âƽ̦޺(lichen planus pemphigoides£¬LPP)ÊÇÖ¸ËäÈ»ÔÚÁÙ´²±íÏÖ¡¢×éÖ¯²¡Àí¡¢ÃâÒßÓ«¹âµÈ¼ì²é£¬³ÊÏÖÓеäÐ͵ıâƽ̦޺(lichen planus£¬LP)ºÍ´óðåÐÔÀàÌìðå´¯(bullous pemphigold,BP)µÄÌØÕ÷£¬µ«Ñ»·¿¹ÌåÈ´ÊÇÕë¶ÔÒ»ÖÖ¶ÀÌصĿ¹Ô·Ö×Ó£¬ÊÇÒ»ÖÖ¶ÀÁ¢µÄ×ÔÉíÃâÒßÐÔ´óðåÐÔƤ·ôճĤ¼²²¡¡£
1.ÁÙ´²±íÏÖ£º
(1)Ƥ·ô£ºÒÔË®ðåΪÖ÷ÒªË𺦡£¿ÉΪСð壬Ҳ¿ÉÓдóð壻¿É·¢ÉúÔÚ±âƽ̦޺µÄËðº¦Ö®ÉÏ£¬Ò²¿É·¢ÉúÔÚÕý³£Æ¤·ôÉÏ£»¿ÉÏÈÓÚµäÐ͵ıâƽ̦޺ƤËð֮ǰ·¢Éú£¬Ò²¿ÉÔÚÆäºó·¢Éú¡£µ«¶àÔÚ¼±ÐÔ·º·¢ÐÔ±âƽ̦޺֮ºóͻȻ³öÏÖ¡£Ë®ðå͸Ã÷£¬ðå±Ú½ôÕÅ£¬ÄáÊÏÕ÷³£³ÊÒõÐÔ¡£È«ÉíÈκβ¿Î»¾ù¿É·¢Éú£¬µ«ÒÔËÄÖ«×îΪÏÔÖø£¬°éÓÐðþÑ÷¡£
(2)¿ÚÇ»£º¿É³öÏÖճĤË𺦣¬±íÏÖΪ½ôÕÅÐÔ´óðåµÄͬʱ°éÓÐÍø״ϸС°×É«ÌõÎÆË𺦡£Ë®ðåÉ¢ÔÚ·Ö²¼£¬ÆÆÀ£ÐγÉÀ£ÑñÃæ¡£³£¼ûµÄËðº¦ÊǽôÕÅÐÔСˮðåΧÈÆÓÚ±âƽ̦޺Ñù°×É«ÌõÎƺͰ߿éÖÜΧ¡£ÓÐЩË𺦱íÏÖΪɫËسÁ×Å¡£
2.×éÖ¯²¡Àí£ºLPPµÄÇðÕî¡¢°ß¿éËðº¦Çø×éÖ¯²¡ÀíÏÔʾ£ºµäÐ͵ıâƽ̦޺ÑùÌØÕ÷£¬¼´ÉÏƤ½Ç»¯¹ý¶È£¬¿ÅÁ£²ãÔöºñ£¬¼¬Ï¸°û²ã²»¹æÔòÔöºñ»òήËõ£¬»ùµ×ϸ°û¿ÕÅݱäÐÔ»òÒº»¯±äÐÔ£¬½áµÞ×é֯dz²ãÁÜ°Íϸ°û´ø×´½þÈ󣬿ɼû½ºÑùСÌå¡£
LPPµÄË®ðåËðº¦Çø×éÖ¯²¡ÀíÏÔʾ£ºÉÏƤÏÂË®ð壬ðåÄڿɼûµ¥ºË¼°ÊÈËáϸ°û£¬½áµÞ×é֯dz²ãѪ¹ÜÖÜΧ¿É¼ûÖжÈÖÂÃÜÁÜ°Íϸ°û¡¢×é֯ϸ°ûºÍÊÈËáϸ°û½þÈó£¬ÆäÉÏ·½»ùµ×ϸ°ûÒòÎÞÒº»¯±äÐÔ£¬¶ø¶àÍêÕûÎÞËð¡£
Ö±½ÓÃâÒßÓ«¹â·¨(DIF)¼ì²éðåÐÔÓë·ÇðåÐÔËðº¦Çø¾ùÏÔʾ£º»ùµ×Ĥ´øÓÐIgG¡¢C3³ÊÏß×´³Á»ý¡£Íâ¹ÛÕý³£µÄճĤƤ·ô»ùµ×Ĥ´øÒ²ÓÐIgGºÍC3³ÊÏß×´³Á»ý¡£¼ä½ÓÃâÒßÓ«¹â·¨(IIF)¼ì²éÏÔʾ£¬Ô¼1/2µÄ»¼ÕßѪÇåÖпɲ⵽ѻ·×ÔÉí¿¹Ìå¡£
3.·¢²¡»úÖƼ°ÆäÓë´óðåÐÔÀàÌìðå´¯¡¢´óðåÐÔ±âƽ̦޺µÄ¹Øϵ£ºÄ¿Ç°Éв»ÍêÈ«Çå³þ¡£ÍƲâÆäÃâÒß·´Ó¦ÎïµÄ³Á»ý¿ÉÄÜÊǼ̷¢ÓÚÁÜ°Íϸ°û½éµ¼µÄÉÏƤϸ°ûËðÉË¡£×ÔÉí¿¹ÌåµÄ²úÉú£¬¶àÈÏΪÊÇÓÉ»ùµ×ϸ°ûÑÏÖØËðÉË£¬Ê¹ÔÀ´Òþ±ÎµÄ¿¹Ô±©Â¶³öÀ´»ò²úÉúеĿ¹ÔÒýÆðµÄ×ÔÉí¿¹ÌåÐγɡ£
Ä¿Ç°ÕùÂ۵ĹؼüÊÇLPPÊÇÕæÕýµÄBP£¬»¹ÊÇBPÑùË𺦡£ÓÐѧÕßÈÏΪ´æÔÚÒ»¸ö²¡±äÆ×£¬¼´´ÓÁÙ´²Ã»ÓÐË®ðåµÄLPµ½°éÓÐBPÁÙ´²¡¢×é֯ѧ¼°ÃâÒßѧÌØÕ÷µÄLP¡£Ò²ÓÐѧÕßÈÏΪLPPÊÇÒ»¸öµ¥¶ÀµÄ¼²²¡£¬Ë®ðåµÄ³öÏÖÊǼ̷¢ÏÖÏ󣬶ø²»ÊÇLPÓëBPµÄżȻÖصþ¡£½öÊÇLPPÓëBPÔÚÁÙ´²¡¢×éÖ¯²¡Àí¡¢ÃâÒß²¡ÀíµÈ±íÏÖÉϾßÓÐÖصþÐÔ£¬µ«ÓÖÓжÀ×ÔµÄÌص㣬Ö÷Òª±íÏÖÔÚ£º¢ÙLPPµÄѪÇå²»ÄÜÓëÒÆÐÐÉÏƤ(Öí°òë×)Æð·´Ó¦£¬¶øBPµÄѪÇå¿ÉÓëÆä·¢Éú·´Ó¦£»¢ÚBPµÄ»ùµ×Ĥ´ø¿¹ÔÏà¶Ô·Ö×ÓÖÊÁ¿¶àÊÇ230000£¬ÉÙÊýÊÇ180000£¬¶øLPP³ýÁËÓÐÏà¶Ô·Ö×ÓÖÊÁ¿180000¿¹Ô·Ö×ÓÍ⣬ÉÐÓÐÒ»ÖÖ200000¿¹Ô£¬ÓÐѧÕßÈÏΪÏà¶Ô·Ö×ÓÖÊÁ¿200000¿¹Ô¿ÉÄÜÊÇLPPµÄ¶ÀÌØ¿¹Ô£»¢ÛLPPѪÇåÑ»·¿¹ÌåÓëBP¿¹Ô»ìºÏ²»·¢ÉúÃâÒß³Áµí·´Ó¦¡£Ìáʾ¸Ã¿¹Ìå²»ÊÇÕë¶ÔBP»ùµ×Ĥ´ø¿¹Ô¶ø²úÉúµÄ¡£
4.Õï¶ÏÓë¼ø±ðÕï¶Ï£º
(1)Õï¶Ï±ê×¼£ºÄ¿Ç°ÉÐÎÞ¹«ÈϵÄÕï¶Ï±ê×¼£¬´ÓÒÔϼ¸·½Ãæ¿ÉÒÔÌáʾLPPµÄÕï¶Ï£º¢ÙÔÕï¶ÏΪLPµÄ»¼Õß³öÏÖË®ðå»ò´óð壬ÓÈÆäË®ðå·¢ÉúÔÚÔ¶Àë̦޺ÑùË𺦵ÄÕý³£Õ³Ä¤Æ¤·ôÉÏ£»¢Ú·ÇðåÐÔËðº¦Çø¾ßÓеäÐ͵ÄLP×é֯ѧÌØÕ÷£¬µ«Ë®ðåËðº¦ÇøΪÉÏƤÏÂðåÇÒ²»°éLP×é֯ѧÌØÕ÷£»¢Û»ùµ×Ĥ´øÓÐIgG¡¢C3Ïß×´³Á»ý£¬IIFѪÇåÖÐÓ뿹»ùµ×Ĥ´ø¿¹Ô½áºÏ·¢Éú³Áµí·´Ó¦¡£ÁíÍâÃâÒߵ羵¡¢ÃâÒßÓ¡¼£¼¼Êõ¾ù¿ÉÐÖúÕï¶Ï¡£
(2)¼ø±ðÕï¶Ï£º¢Ù´óðåÐÔ±âƽ̦޺ÓеäÐ͵ÄÌõÎÆËðº¦ÌØÕ÷£¬²¢ÔÚ´Ë»ù´¡ÉϳöÏÖË®ðå»ò´óð壬һ°ãË®ðå¶à·¢ÉúÓÚÔÓÐËðº¦ÇøÖ®ÉÏ¡£ÎÞ¿¹»ùµ×Ĥ´øµÄ×ÔÉí¿¹Ìå¡£×éÖ¯²¡ÀíÏÔʾ£¬Ë®ðåÊÇÓÉÓÚ»ùµ×ϸ°ûÑÏÖØÒº»¯±äÐÔÒýÆðµÄÉÏƤ-½áµÞ×éÖ¯·ÖÀë²úÉúµÄÁÑ϶£¬¶øLPPÒòÎÞÒº»¯±äÐÔ£¬ðåÇ»¶¥²¿»ùµ×ϸ°ûÍêÕû£¬´ËΪÖØÒª¼ø±ðÒªµã¡£¢Ú´óðåÐÔÀàÌìðå´¯µÄ·¢²¡ÄêÁä½Ï´ó£¬¶ùͯÉÙ¼û¡£Ë®ðå·¢ÉúÔÚÕý³£Æ¤·ô»òºì°ß»ù´¡ÉÏ£¬ÎÞ±âƽ̦޺ÑùË𺦡£×éÖ¯²¡ÀíÏÔʾÉÏƤÏÂË®ð壬ÎÞ±âƽ̦޺Ñù×é֯ѧÌØÕ÷¡£ÃâÒߵ羵¼ì²éÆäÃâÒßÇòµ°°×³Á»ýµÄ²¿Î»£¬»ùµ×Ĥ´ø¿¹Ô·Ö×ÓÁ¿£¬ÃâÒß³Áµí·´Ó¦µÈ¾ùÏÔʾÓëLPPÓв»Í¬¡£´ËÍâÉÐÐëÓë´Ë¼ø±ðµÄÓдóðåÐÔ±íƤËɽâÖ¢(epiderm olysis bullosa acquisita,EBA)¡¢´óðåÐÔºì°ßÀÇ´¯(bullous lupus erythematosus,BLE)µÈ¡£
5.ÖÎÁƺÍÔ¤ºó£ºÉöÉÏÏÙƤÖʼ¤ËØÖÎÁÆЧ¹ûÃ÷ÏÔ£¬²ÉÓÃÖеȼÁÁ¿µÄÇ¿µÄËÉ»òÇ¿µÄËÉÁú¼´¿É»ñÏÔÖøÁÆЧ¡£Ò»°ã¼ÁÁ¿Îª40mg/d(1.5mg¡¤kg-1.d-1)Ç¿µÄËÉ¡£Ò²ÓÐÒÔÇ¿µÄËÉÓëÁòßòàÑßʺÏÓÃÖÎÁƳɹ¦µÄ±¨µÀ¡£»¹ÓÐÓûһÆùËØ500mg/dÖÎÁÆ£¬Ëðº¦ÓÚ4ÖÜÄÚ¿ªÊ¼ÏûÍË£¬ÖÎÁÆȬÓú¡£¸Ã²¡È«ÉíÖ¢×´Ïà¶Ô½ÏÇᣬԤºó½ÏºÃ¡£
¶þ¡¢¸±Ö×ÁöÐÔÌìðå´¯£Û8-15£Ý
ÓÉÓÚÌåÄÚÖ×ÁöµÄÑ»·ÌåÒºÒò×Ó»òÆä´úлÐÔ²úÎïµÄ×÷ÓöøÓÕ·¢µÄƤ·ôճĤ¼²²¡£¬±»³ÆΪ¸±Ö×ÁöÐÔƤ·ôճĤ²¡¡£µ±Ö×Áö»¼Õß³öÏÖÌìðå´¯ÑùËðº¦Ê±£¬¼´³Æ֮Ϊ¸±Ö×ÁöÐÔÌìðå´¯(paraneoplastic pemphigus£¬PNP)¡£µ«¸±Ö×ÁöÐÔÌìðå´¯²¢²»ÊÇÌìðå´¯ºÍÖ×ÁöµÄµ¥´¿²¢·¢´æÔÚ£¬¶øÊÇÒ»ÀàѪÇåÖÐÓÐÒ»×éÌØÊâ×ÔÉí¿¹ÌåµÄ×ÔÉíÃâÒßÐÔ¼²²¡¡£¸Ã²¡ÖÖÊ×ÏÈÓÉAnhaltµÈÈËÓÚ1990Ä걨µÀ¡£
1.ÁÙ´²±íÏÖ£º
(1)ճĤ£º»¼Õß¾ùÓнÏÖصÄճĤË𺦣¬½áĤ¡¢¿Ú´½¡¢¼Õ¡¢³Ýö¸¡¢ÑÊ¡¢Ê³µÀ¡¢ÍâÒõճĤ¾ù¿ÉÊÜÀÛ¡£Æä±íÏÖÀàËÆÌìðå´¯Ñù¸Ä±ä£¬Èç´óÃæ»ýÃÓÀ㬽ÒƤÊÔÑéÑôÐÔ£¬Ì½ÕëÊÔÑéÑôÐÔ£¬ÄáÊÏÕ÷ÑôÐԵȡ£
(2)Ƥ·ô£ºÊÜÀÛÃæ»ý½Ï¹ã£¬±íÏÖΪºì°ß¡¢·çÍÅ¡¢Ë®ð塢Ѫðå¡¢ÃÓÀᢽáðè¡¢±íƤ°þÍÑ£¬²¢ÓÐÃ÷ÏÔÌÛÍ´»òðþÑ÷¡£¾±¡¢Ëø¹ÇÉÏ¡¢Ò¸ÎÑ¡¢¸¹¹É¹µµÈ´¦µÄ±ídzÁܰͽáÖ×´ó¡£
»¼ÕßÍÌÑÊÀ§ÄÑ£¬Æ£¾ëÎÞÁ¦£¬¼¡Í´£¬È«ÉíÇé¿ö²î¡£ÓÐʱÈÝÒ×ÓëÖØÖ¢¶àÐκì°ß(Stevens-Johnson×ÛºÏÕ÷)»òÖж¾ÐÔ±íƤ»µËÀËɽâÖ¢Ïà»ìÏý¡£Ò²Óл¼ÕߵĴóðåðå±Ú½ôÕÅ£¬ÄáÊÏÕ÷ÒõÐÔ£¬Óë´óðåÐÔÀàÌìðå´¯ÏàËÆ¡£
2.×éÖ¯²¡Àí£º¶Ô¸±Ö×ÁöÐÔÌìðå´¯»¼ÕߵĿÚǻճĤ±ê±¾¹Û²ì·¢ÏÖ£¬Æ䲡ÀíÌصãÖ÷ÒªÓУº¢ÙÉÏƤÄÚ·¢Éú¼¬²ãËɽ⣻¢ÚÁÑ϶»òË®ðå¾ùÔÚ½ô¿¿»ùµ×ϸ°û²ãµÄÉÏ·½£¬ðåµ×ÈÞëÐγɣ»¢ÛÔÚ¼¬²ãËɽ⴦µÄÉÏƤÄÚ£¬¾ù¿É¼û½Ç»¯²»Á¼Ï¸°û£¬ÆäÊýÁ¿¸öÌå¼äÓвîÒ죻¢Ü¾ù³öÏÖ»ùµ×²ãÒº»¯±äÐÔ£¬¿ÉÓ뼬²ãËɽⲢ´æ»òµ¥¶À·¢Éú£¬µ«²»³öÏÖÉÏƤÏÂË®ð壻¢Ý²»Í¬³Ì¶ÈµÄÑ×֢ϸ°ûÒÆÈëÉÏƤ²ã£¬ÒÔµ¥¸öºËϸ°ûΪÖ÷¡£
Ö±½ÓÃâÒßÓ«¹â¼ì²é¿É¼û£¬IgGºÍ²¹ÌåC3ÔÚ¼¬Ï¸°û¼ä³Á»ýºÍ(»ò)²¹ÌåÑØ»ùµ×Ĥ´ø³Á»ý¡£µ«½ÏÑ°³£ÐÍÌìð崯ɫµ»ò³ÊÔî×´¡£C3ÔÚϸ°û¼äºÍ»ùµ×Ĥ´øÇø³Á»ýÓÐÌØÊâµÄÒâÒå¡£ÒÔºïʳµÀ¡¢ÈËƤ·ô¡¢ÊóƤ·ôΪµ×Î¼ä½ÓÃâÒßÓ«¹â¼ì²é(IIF)£¬¿É¼ûIgGÀ࿹Ìå³Á»ýÓÚϸ°û¼ä¡£ÓëÑ°³£ÐÍÌìðå´¯²»Í¬µÄÊÇ£¬PNPµÄIgÀà×ÔÉí¿¹Ì廹ÄÜÓëÆäËüÉÏƤ×éÖ¯ÖеÄÇÅÁ£½áºÏ£¬°üÀ¨ÒÔСÊóµÄ°òëס¢ºôÎüµÀ¡¢Ð¡³¦¡¢½á³¦¡¢¼××´ÏÙµÈÉÏƤºÍÐļ¡£¬¹Ç÷À¼¡Îªµ×Îïʱ£¬IIFҲΪÑôÐÔ£¬µ«ÒÔ°òë×ÉÏƤӫ¹â×îÇ¿£¬ÑôÐÔÂÊΪ76%¡£¶øÑ°³£ÐÍÌìðå´¯»¼ÕßѪÇåÖеÄ×ÔÉí¿¹ÌåÈ´²»ÄÜÓë°òëס¢Ð¡³¦µÈÒÆÐÐÉÏƤÖеÄÇÅÁ£½áºÏ£¬IIF½á¹ûΪÒõÐÔ¡£µ¥´¿Ö×ÁöÕßµÄѪÇå½áºÏҲΪÒõÐÔ¡£Òò´Ë£¬ÒÔÊó°òë×Ϊµ×ÎïµÄIIFÊǼì²âPNPµÄ¼òµ¥·½·¨£¬¿É´úÌæÃâÒß³Áµí(immuno-precipitation)ÔÚÁÙ´²µÄÔËÓá£
3.·¢²¡»úÖƼ°ÓëÖ×Áö·¢ÉúµÄ¹Øϵ£ºËäÈ»¸±Ö×ÁöÐÔÌìðå´¯µÄ·¢²¡»úÖƲ»Ç壬µ«ÈÏΪϵ»úÌå¶ÔÖ×ÁöµÄÃâÒß·´Ó¦ËùÖ¡£»úÌå²úÉúµÄ¿¹Ö×Áö×éÖ¯¿¹Ì壬ºÍÌåÄÚÕý³£×éÖ¯ÖеÄijЩ¿¹Ô³É·ÖÓн»²æ·´Ó¦£¬½áºÏºó¸ÉÈÅÆÆ»µÁË°Ð×éÖ¯µÄÕý³£¹¦Äܶø·¢²¡¡£ÕâÒ»¼Ù˵Òѵõ½²¿·Ö֤ʵ£º¢ÙÊÔÑé±íÃ÷£¬PNPѪÇåÖеÄÃâÒßÇòµ°°×ÔÚ·¢²¡¹ý³ÌÖÐÆðÖØÒª×÷Ó㬷¢²¡»úÖÆÖдæÔÚ×ÔÉíÃâÒß»ù´¡¡£¢ÚÓÃÃâÒß³Áµí·¨¼ì²é·¢ÏÖ£¬PNP»¼ÕßѪÇåÖеÄ×ÔÉí¿¹ÌåΪһ×鿹Ì壬ÄÜÓë¶àÖÖÉÏƤ·¢Éú·´Ó¦£¬Æ俹ԳɷֵÄÏà¶Ô·Ö×ÓÖÊÁ¿·Ö±ðΪ250000¡¢230000¡¢210000¡¢190000¡£250000ºÍ210000µ°°×£¬·Ö±ðÊÇÇÅÁ£ÖÂÃÜ°ßµÄÖ÷Òª³É·ÖÇÅÁ£µ°°×(Desmophlakin)¢ñºÍ¢ò(DP¢ñ¡¢DP¢ò)¡£¶øDP¢ñ¡¢DP¢ò±»ÈÏΪÊÇijЩÖ×Áöϸ°ûµÄ±ê¼ÇÎï¡£ÈçDP¢ñ¡¢DP¢ò¿¹Ìå¿ÉÓÃÓÚ