Lancet:落叶型天疱疮——案例报道

2016-10-18 MedSci MedSci原创

患者男,70岁,2014年4月因瘙痒性皮疹就诊,皮疹涉及头皮、面部、胸部、背部。 既往史:结肠癌和深静脉血栓形成。 皮疹的活检样本被解释为湿疹,但局部和全身使用糖皮质激素和抗生素后,患者的症状改善不是那么明显。 查体:看起来很健康,有红斑丘疹和斑块,玉米片大小,脂溢性分布。 以往的血清学研究,包括抗核抗体(ANA)、补体及血清蛋白电泳,均正常。 我们对皮损进行常规

患者男,70岁,2014年4月因瘙痒性皮疹就诊,皮疹涉及头皮、面部、胸部、背部。

既往史:结肠癌和深静脉血栓形成。

皮疹的活检样本被解释为湿疹,但局部和全身使用糖皮质激素和抗生素后,患者的症状改善不是那么明显。

查体:看起来很健康,有红斑丘疹和斑块,玉米片大小,脂溢性分布。



以往的血清学研究,包括抗核抗体(ANA)、补体及血清蛋白电泳,均正常。

我们对皮损进行常规组织学活检。以及对病灶周围钻取活组织检查(直接免疫荧光法)。



临床病理诊断为:落叶型天疱疮

治疗方案:每天30 mg泼尼松龙;一周一次利妥昔单抗 375 mg/m2静脉注射,持续4周;曲安奈德0·1%乳膏每日两次。

利妥昔单抗治疗后2个月,患者的皮肤病变改善显著,减少泼尼松龙至每天20 mg。

每3个月对患者进行一次随访,直至停止泼尼松龙治疗。

天疱疮是一种慢性、复发性、严重性表皮内疱性平凡病,可能是一种自身免疫性疾病。临床上可分四型:寻常型、增殖型、落叶型、红斑型。

落叶型天疱疮的皮损好发于头面、躯干、起出为疱破渗出形成黄褐色,由腻性叶状结,有腥臭、口腔粘膜损害轻、少见。

临床上往往仅见一红色创面或糜烂面,若能用探针无阻力地伸入到上皮下方或邻近的黏膜表层下方,尼氏征阳性,或揭皮试验阳性则有助于诊断。

原始出处:

Elena Gonzalez,et al.A refractory rash in a seborrheic distribution.Lancet.August 2016

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  1. 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createdName=doctorJiangchao, createdTime=Wed Oct 19 09:55:05 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149670, encodeId=b77f1496e0f8, content=落叶型天疱疮的皮损好发于头面、躯干、起出为疱破渗出形成黄褐色,由腻性叶状结,有腥臭、口腔粘膜损害轻、少见。多多关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=57, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Pw2csslt7CukWiabqiblnQAXaDEiaxhu025c3P0XshccTrbvGWBjKvnHIxbK3vqa80P7WGIFuicCFet97TicrNKpgB3TdabxP1vMh/0, createdBy=28851960965, createdName=榄枷檬, createdTime=Wed Oct 19 08:49:31 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149669, encodeId=368514966904, content=天疱疮是一种慢性、复发性、严重性表皮内疱性平凡病,可能是一种自身免疫性疾病。临床上可分四型:寻常型、增殖型、落叶型、红斑型。好好学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], 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createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Tue Oct 18 22:35:55 CST 2016, time=2016-10-18, status=1, ipAttribution=)]
    2017-01-01 1e1cd78dm87(暂无匿称)

    学习了。了解更加多的疱疹。

    0

  2. 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attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Pw2csslt7CukWiabqiblnQAXaDEiaxhu025c3P0XshccTrbvGWBjKvnHIxbK3vqa80P7WGIFuicCFet97TicrNKpgB3TdabxP1vMh/0, createdBy=28851960965, createdName=榄枷檬, createdTime=Wed Oct 19 08:49:09 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149639, encodeId=8d2814963901, content=长见识了……, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2017/09/23/8c541a9831aec7026dffdf6bf34ab45d.jpg, createdBy=11371963624, createdName=兰焱雨, createdTime=Wed Oct 19 07:03:28 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149599, encodeId=7297149599b2, content=这个病例不错呀!, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Wed Oct 19 00:09:21 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149597, encodeId=26f714959eaf, content=刚刚看到一例, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e2291960840, createdName=蓬莱阁, createdTime=Tue Oct 18 23:55:03 CST 2016, time=2016-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149588, encodeId=f29714958881, content=这个不错哦, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, 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    2016-10-31 howi
  3. 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createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Tue Oct 18 22:35:55 CST 2016, time=2016-10-18, status=1, ipAttribution=)]
    2016-10-19 虈亣靌

    学习了,分享了!!!

    0

  4. 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attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Pw2csslt7CukWiabqiblnQAXaDEiaxhu025c3P0XshccTrbvGWBjKvnHIxbK3vqa80P7WGIFuicCFet97TicrNKpgB3TdabxP1vMh/0, createdBy=28851960965, createdName=榄枷檬, createdTime=Wed Oct 19 08:49:09 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149639, encodeId=8d2814963901, content=长见识了……, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2017/09/23/8c541a9831aec7026dffdf6bf34ab45d.jpg, createdBy=11371963624, createdName=兰焱雨, createdTime=Wed Oct 19 07:03:28 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149599, encodeId=7297149599b2, content=这个病例不错呀!, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Wed Oct 19 00:09:21 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149597, encodeId=26f714959eaf, content=刚刚看到一例, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e2291960840, createdName=蓬莱阁, createdTime=Tue Oct 18 23:55:03 CST 2016, time=2016-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149588, encodeId=f29714958881, content=这个不错哦, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Tue Oct 18 22:35:55 CST 2016, time=2016-10-18, status=1, ipAttribution=)]
    2016-10-19 doctorJiangchao

    少见的病例

    0

  5. 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topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Wed Oct 19 00:09:21 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149597, encodeId=26f714959eaf, content=刚刚看到一例, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e2291960840, createdName=蓬莱阁, createdTime=Tue Oct 18 23:55:03 CST 2016, time=2016-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149588, encodeId=f29714958881, content=这个不错哦, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, 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    2016-10-19 榄枷檬

    落叶型天疱疮的皮损好发于头面、躯干、起出为疱破渗出形成黄褐色,由腻性叶状结,有腥臭、口腔粘膜损害轻、少见。多多关注

    0

  6. 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topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Wed Oct 19 00:09:21 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149597, encodeId=26f714959eaf, content=刚刚看到一例, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e2291960840, createdName=蓬莱阁, createdTime=Tue Oct 18 23:55:03 CST 2016, time=2016-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149588, encodeId=f29714958881, content=这个不错哦, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Tue Oct 18 22:35:55 CST 2016, time=2016-10-18, status=1, ipAttribution=)]
    2016-10-19 榄枷檬

    天疱疮是一种慢性、复发性、严重性表皮内疱性平凡病,可能是一种自身免疫性疾病。临床上可分四型:寻常型、增殖型、落叶型、红斑型。好好学习

    0

  7. 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attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Pw2csslt7CukWiabqiblnQAXaDEiaxhu025c3P0XshccTrbvGWBjKvnHIxbK3vqa80P7WGIFuicCFet97TicrNKpgB3TdabxP1vMh/0, createdBy=28851960965, createdName=榄枷檬, createdTime=Wed Oct 19 08:49:09 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149639, encodeId=8d2814963901, content=长见识了……, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2017/09/23/8c541a9831aec7026dffdf6bf34ab45d.jpg, createdBy=11371963624, createdName=兰焱雨, createdTime=Wed Oct 19 07:03:28 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149599, encodeId=7297149599b2, content=这个病例不错呀!, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Wed Oct 19 00:09:21 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149597, encodeId=26f714959eaf, content=刚刚看到一例, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e2291960840, createdName=蓬莱阁, createdTime=Tue Oct 18 23:55:03 CST 2016, time=2016-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149588, encodeId=f29714958881, content=这个不错哦, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Tue Oct 18 22:35:55 CST 2016, time=2016-10-18, status=1, ipAttribution=)]
    2016-10-19 兰焱雨

    长见识了……

    0

  8. 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attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Pw2csslt7CukWiabqiblnQAXaDEiaxhu025c3P0XshccTrbvGWBjKvnHIxbK3vqa80P7WGIFuicCFet97TicrNKpgB3TdabxP1vMh/0, createdBy=28851960965, createdName=榄枷檬, createdTime=Wed Oct 19 08:49:09 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149639, encodeId=8d2814963901, content=长见识了……, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2017/09/23/8c541a9831aec7026dffdf6bf34ab45d.jpg, createdBy=11371963624, createdName=兰焱雨, createdTime=Wed Oct 19 07:03:28 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149599, encodeId=7297149599b2, content=这个病例不错呀!, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Wed Oct 19 00:09:21 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149597, encodeId=26f714959eaf, content=刚刚看到一例, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e2291960840, createdName=蓬莱阁, createdTime=Tue Oct 18 23:55:03 CST 2016, time=2016-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149588, encodeId=f29714958881, content=这个不错哦, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Tue Oct 18 22:35:55 CST 2016, time=2016-10-18, status=1, ipAttribution=)]
    2016-10-19 Carlos007

    这个病例不错呀!

    0

  9. 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topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Wed Oct 19 00:09:21 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149597, encodeId=26f714959eaf, content=刚刚看到一例, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e2291960840, createdName=蓬莱阁, createdTime=Tue Oct 18 23:55:03 CST 2016, time=2016-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149588, encodeId=f29714958881, content=这个不错哦, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Tue Oct 18 22:35:55 CST 2016, time=2016-10-18, status=1, ipAttribution=)]
    2016-10-18 蓬莱阁

    刚刚看到一例

    0

  10. 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createdName=doctorJiangchao, createdTime=Wed Oct 19 09:55:05 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149670, encodeId=b77f1496e0f8, content=落叶型天疱疮的皮损好发于头面、躯干、起出为疱破渗出形成黄褐色,由腻性叶状结,有腥臭、口腔粘膜损害轻、少见。多多关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=57, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Pw2csslt7CukWiabqiblnQAXaDEiaxhu025c3P0XshccTrbvGWBjKvnHIxbK3vqa80P7WGIFuicCFet97TicrNKpgB3TdabxP1vMh/0, createdBy=28851960965, createdName=榄枷檬, createdTime=Wed Oct 19 08:49:31 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149669, encodeId=368514966904, content=天疱疮是一种慢性、复发性、严重性表皮内疱性平凡病,可能是一种自身免疫性疾病。临床上可分四型:寻常型、增殖型、落叶型、红斑型。好好学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Pw2csslt7CukWiabqiblnQAXaDEiaxhu025c3P0XshccTrbvGWBjKvnHIxbK3vqa80P7WGIFuicCFet97TicrNKpgB3TdabxP1vMh/0, createdBy=28851960965, createdName=榄枷檬, createdTime=Wed Oct 19 08:49:09 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149639, encodeId=8d2814963901, content=长见识了……, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2017/09/23/8c541a9831aec7026dffdf6bf34ab45d.jpg, createdBy=11371963624, createdName=兰焱雨, createdTime=Wed Oct 19 07:03:28 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149599, encodeId=7297149599b2, content=这个病例不错呀!, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Wed Oct 19 00:09:21 CST 2016, time=2016-10-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149597, encodeId=26f714959eaf, content=刚刚看到一例, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e2291960840, createdName=蓬莱阁, createdTime=Tue Oct 18 23:55:03 CST 2016, time=2016-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=149588, encodeId=f29714958881, content=这个不错哦, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160923/IMG57E54D670B3C08868.jpg, createdBy=e90b1956497, createdName=Carlos007, createdTime=Tue Oct 18 22:35:55 CST 2016, time=2016-10-18, status=1, ipAttribution=)]
    2016-10-18 Carlos007

    这个不错哦

    0