С¶ùÒÅ´«ÐÔ´óðåÐÔ±íƤËɽâÖ¢ÔçÆÚÓÐÄÄЩ֢״
¡¡¡¡ÒÅ´«ÐÔ´óðåÐÔ±íƤËɽâÖ¢Ëä·ÖΪÈýÐͼ°Öî¶àÑÇÐÍ£¬µ«Óй²Í¬µÄÁÙ´²Ìص㣬¶àΪ³öÉúºó»ò2ËêÄÚ·¢²¡£¬Ä¦²Á²¿Î»ÈçÊÖ¡¢×㡢ϥ¡¢Öâ¡¢õס¢Íβ¿¡£Ä¦²Á¼´³öÏÖ´óС²»µÈµÄË®ðåѪðå(ͼ1)£¬ÃÓÀᢽáð衢ɫËسÁ×Å¡£¿É¼ûËÚÇðÕήËõ¡¢ñ£ºÛ¡¢¼×ÓªÑø²»Á¼¡¢Íº·¢ºÍ¼Ì·¢¸ÐȾµÈ¡£Ö¸Öºñ£ºÛºóÓ°Ï칦ÄÜÉõÖÁÖ²С£
¡¡¡¡ÈýÐÍÁÙ´²±íÏÖ¼û±í1¡£
¡¡¡¡ÏÖ½«ÈýÐͼ°ÑÇÐͼòÊöÈçÏ£º
¡¡¡¡1.µ¥´¿ÐÔ´óðåÐÔ±íƤËɽâÖ¢(epidermolysis bullosa simplex£¬EBS)
¡¡¡¡(1)¾ÖÏÞÐÔ(localized)£º
¡¡¡¡¢ÙÊÖ×ãµ¥´¿ÐÔ´óðåÐÔ±íƤËɽâÖ¢(EBS of hands and feet)£ºÒà³ÆWeber-CockayneÐÍ£¬ÊÇ×î³£¼ûµÄEBSÑÇÐÍ¡£Ë®ðåÖ÷Òª·¢ÉúÓÚ×ãõÅ£¬Æä´ÎΪÊÖÕÆ£¬»úеĦ²ÁÑÏÖØʱ£¬Ë®ðå¿É·¢ÉúÓÚÉíÌåÈκβ¿Î»£¬ÔÚÐÂÉú¶ù»òÓ¤¶ùÆÚ·¢²¡£¬ÉÙÊýÑÓ³ÙÖÁÇà´ºÆÚ»ò³ÉÄêÆÚ¡£¶¬ÇáÏÄÖØ£¬³¤Ê±¼äÐÐ×ß¼°»úеĦ²Á¾ù¿Éʹ²¡Çé¼ÓÖØ£¬³£ÐγɾÖÏÞ»ò¹ã·ºëÝëÕ¡£ËæÄêÁäÔö³¤ÓÐЩ²¡È˵IJ¡ÇéÓÐËù»º½â£¬ÉÙÊý»¼ÕßÓм×ÓªÑø²»Á¼¡¢ËÚÇðÕîºÍñ£ºÛÐγɣ¬ÎÞƤ·ôÍⲡ±ä¡£
¡¡¡¡¢ÚÑÀ»òÑÀ·¢Óý²»È«ÐÔµ¥´¿ÐÔ´óðåÐÔ±íƤËɽâÖ¢(EBS with anodontia/hypodontia)£ºÒà³ÆKallin×ÛºÏÕ÷¡£´ËÐÍÉÙ¼û£¬³ý¾ÖÏÞÐÔË®ðåÍ⣬°éÓд෢¡¢²¿·Öͺ·¢ºÍÑÀ·¢Óý²»È«¡£
¡¡¡¡(2)È«ÉíÐÔ(generalized)£º
¡¡¡¡¢ÙKoebnerÐ͵¥´¿ÐÔ´óðåÐÔ±íƤËɽâÖ¢(EBS£¬Koebner variant)£ºÎªEBSµÄµÚ¶þ³£¼ûÑÇÐÍ¡£³£ÔÚ³öÉúʱ»òÉúºó²»¾Ã·¢²¡¡£È«Éí·º·¢Ë®ð壬ÊÜѹ²¿Î»Ã÷ÏÔ£¬¿ÉÓм×ÓªÑø²»Á¼¡¢ËÚÇðÕîºÍ(»ò)ñ£ºÛ¡£µ«ñ£ºÛ±ídz¶ø¾ÖÏÞ¡£³ýÓÐЩ»¼ÕßÔÚÓ¤¶ùÔçÆÚ¿ÚÇ»ÄÚ»á³öÏÖÇá΢¶ø¾ÖÏÞµÄË®ðåÍ⣬ÎÞƤ·ôÍⲡ±ä¡£
¡¡¡¡¢ÚðåÕîÑùµ¥´¿ÐÔ´óðåÐÔ±íƤËɽâÖ¢(EBS herpetiformis)£ºÒà³ÆDowling-MearaÐÍ£¬ÎªEBSµÄµÚÈý³£¼ûÑÇÐÍ¡£³öÉúʱ¼´·¢²¡£¬¹ã·ºµÄƤ·ô°þÍÑ£¬³£µ¼Ö»¼¶ùÔÚÉúºóµÚ1ÄêËÀÍö£¬´ó¶àÊý»¼ÕßµÄƤËðÓëKoebnerÐÍÏàËÆ£¬¼×ÓªÑø²»Á¼¡¢ËÚÇðÕîºÍ(»ò)ñ£ºÛµÄ·¢ÉúÒ²ÓëKoebnerÐÍÏàËÆ¡£µ½ÇഺǰÆÚ£¬¶àÊý»¼Õ߶¼»áÐγɹ㷺»òÈںϵÄÕÆõŽǻ¯¡£
¡¡¡¡¢Û°éÓлò²»°éÓÐƤ·ô½Ç»¯²¡ºÍ»¨°ß×´É«ËسÁ×ŵĵ¥´¿ÐÔ´óðåÐÔ±íƤËɽâÖ¢(EBS with mottled pigmentation with/without keratoderma)£º´ËÐͺ±¼û¡£Ä³Ð©²¿Î»³öÏÖƤ·ôµÄ²»¹æÔòÉ«ËسÁ×Űߣ¬ÕâЩɫËسÁ×Ű߶àÊý²»ÊÇ·¢ÉúÔÚÔø¾·¢ÉúË®ðåµÄ²¿Î»¡£ËæÄêÁäÔö³¤£¬ÓÐЩ²¡Àý¿ÉÒÔ¼õÇá¡£ÕÆõŽǻ¯ÓëWeber-CockayneÐÍÏàËÆ£¬ÎÞƤ·ôÍⲡ±ä¡£
Ïà¹ØÎÄÕ£º | ¡óΪʲôº¢×ÓÉúÏÂÀ´¾Í¶úÁû | ¡óÒÅ´«²¡ÓÐÄÄЩÌصã |
¡óÒÅ´«ÐÔÉöÔಡµÄ·ÀÖÎÖصã | ¡óÔõÑù×Բ顰´óðåÐÔ±íƤËɽâÖ¢¡± |