皮肤性

订阅

MedSci主页 / 所有栏目 / 皮肤性

PAPASH综合征一例

2018-03-14 佚名 中华皮肤科杂志

PAPASH
关注

患者男，22岁。面颈部、背部、腋窝、腹股沟暗红色丘疹、结节、脓疱、囊肿6年，双下肢多发暗紫红斑块伴溃疡1年，2016年10月31日于郑州大学第一附属医院皮肤科就诊。

【一般资料】

患者男，22岁

【主诉】

面颈部、背部、腋窝、腹股沟暗红色丘疹、结节、脓疱、囊肿6年，双下肢多发暗紫红斑块伴溃疡1年，2016年10月31日于郑州大学第一附属医院皮肤科就诊。

【现病史】

6年前，患者于某院诊断为痤疮，未予治疗。1年前双下肢出现多发紫红色斑块，后破溃并融合成溃疡，直径1～12cm。2002年5月9日，患者因左膝关节肿痛半年就诊于郑州大学第一附属医院小儿内科，检查示类风湿因子阴性，左膝关节腔积液涂片：中性粒细胞、少量淋巴及单核细胞，未见异常细胞，

【初步诊断】

诊断为左膝化脓性关节炎。

【体格检查】

囊肿型痤疮和化脓性汗腺炎表现：患者颜面、腰背部炎性丘疹、脓疱、脓肿及囊肿，部分破溃，腋窝及腹股沟化脓性汗腺炎损害一般情况欠佳，行走困难，血压142/92mmHg。双下肢多发紫红色斑块，中央溃疡形成。

【辅助检查】

下肢溃疡边缘皮损组织病理：表皮破溃，细胞间水肿，可见中性粒细胞进入，真皮浅中层明显水肿，局灶红细胞外溢，中性粒细胞、淋巴细胞、组织细胞为主弥漫性浸润，临床及病理符合坏疽性脓皮病。面颈部、腰背部可见广泛炎性丘疹、脓疱、脓肿及囊肿，腋窝、腹股沟少量暗红色结节，呈囊肿痤疮及化脓性汗腺炎表现。基因检测：PSTPIP1基因测序：外显子区未见突变，内含子区发现c.36+68G>A、c.137+47G>C、c.562+114C>Ghet复合杂合突变，在100例正常人测序中有45例发现该位点突变，考虑为多态性位点，致病意义不能明确。最终诊断：PAPASH综合征。

【治疗】

给予甲泼尼龙、头孢米诺、异维A酸及沙利度胺治疗，2周后皮损明显好转。现患者仍在随访中。

版权声明：
本网站所有内容来源注明为“梅斯医学”或“MedSci原创”的文字、图片和音视频资料，版权均属于梅斯医学所有。非经授权，任何媒体、网站或个人不得转载，授权转载时须注明来源为“梅斯医学”。其它来源的文章系转载文章，或“梅斯号”自媒体发布的文章，仅系出于传递更多信息之目的，本站仅负责审核内容合规，其内容不代表本站立场，本站不负责内容的准确性和版权。如果存在侵权、或不希望被转载的媒体或个人可与我们联系，我们将立即进行删除处理。
在此留言
评论区 (5)
#插入话题
  1. [GetPortalCommentsPageByObjectIdResponse(id=2030160, encodeId=2c872030160c6, content=<a href='/topic/show?id=d88913e2636' target=_blank style='color:#2F92EE;'>#PAP#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=13726, encryptionId=d88913e2636, topicName=PAP)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=82ba2500166, createdName=ms1467340776200723, createdTime=Tue May 08 19:37:00 CST 2018, time=2018-05-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=297149, encodeId=6a1c29e1493b, content=学习谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zMoyicyo6ia9f4IuLQAwZoxD6Hx4ibd5CMcOCYhStY6oDibbKK6O2X8iaicldO5ib8j1iapOIobIKCGiczU2A/0, createdBy=56251941490, createdName=虈亣靌, createdTime=Sat Mar 17 18:09:19 CST 2018, time=2018-03-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1301236, encodeId=83d9130123687, content=<a href='/topic/show?id=38e32852fe' target=_blank style='color:#2F92EE;'>#ASH#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2852, encryptionId=38e32852fe, topicName=ASH)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=39a0295, createdName=kksonne, createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1338179, encodeId=580a13381e957, content=<a href='/topic/show?id=cd2a262068' target=_blank style='color:#2F92EE;'>#APA#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2620, encryptionId=cd2a262068, topicName=APA)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ce072500140, createdName=12498b3em08(暂无昵称), createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431148, encodeId=f1e1143114850, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=)]
    2018-05-08 ms1467340776200723

    #PAP#

    0

  2. [GetPortalCommentsPageByObjectIdResponse(id=2030160, encodeId=2c872030160c6, content=<a href='/topic/show?id=d88913e2636' target=_blank style='color:#2F92EE;'>#PAP#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=13726, encryptionId=d88913e2636, topicName=PAP)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=82ba2500166, createdName=ms1467340776200723, createdTime=Tue May 08 19:37:00 CST 2018, time=2018-05-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=297149, encodeId=6a1c29e1493b, content=学习谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zMoyicyo6ia9f4IuLQAwZoxD6Hx4ibd5CMcOCYhStY6oDibbKK6O2X8iaicldO5ib8j1iapOIobIKCGiczU2A/0, createdBy=56251941490, createdName=虈亣靌, createdTime=Sat Mar 17 18:09:19 CST 2018, time=2018-03-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1301236, encodeId=83d9130123687, content=<a href='/topic/show?id=38e32852fe' target=_blank style='color:#2F92EE;'>#ASH#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2852, encryptionId=38e32852fe, topicName=ASH)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=39a0295, createdName=kksonne, createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1338179, encodeId=580a13381e957, content=<a href='/topic/show?id=cd2a262068' target=_blank style='color:#2F92EE;'>#APA#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2620, encryptionId=cd2a262068, topicName=APA)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ce072500140, createdName=12498b3em08(暂无昵称), createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431148, encodeId=f1e1143114850, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=)]
    2018-03-17 虈亣靌

    学习谢谢分享!

    0

  3. [GetPortalCommentsPageByObjectIdResponse(id=2030160, encodeId=2c872030160c6, content=<a href='/topic/show?id=d88913e2636' target=_blank style='color:#2F92EE;'>#PAP#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=13726, encryptionId=d88913e2636, topicName=PAP)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=82ba2500166, createdName=ms1467340776200723, createdTime=Tue May 08 19:37:00 CST 2018, time=2018-05-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=297149, encodeId=6a1c29e1493b, content=学习谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zMoyicyo6ia9f4IuLQAwZoxD6Hx4ibd5CMcOCYhStY6oDibbKK6O2X8iaicldO5ib8j1iapOIobIKCGiczU2A/0, createdBy=56251941490, createdName=虈亣靌, createdTime=Sat Mar 17 18:09:19 CST 2018, time=2018-03-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1301236, encodeId=83d9130123687, content=<a href='/topic/show?id=38e32852fe' target=_blank style='color:#2F92EE;'>#ASH#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2852, encryptionId=38e32852fe, topicName=ASH)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=39a0295, createdName=kksonne, createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1338179, encodeId=580a13381e957, content=<a href='/topic/show?id=cd2a262068' target=_blank style='color:#2F92EE;'>#APA#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2620, encryptionId=cd2a262068, topicName=APA)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ce072500140, createdName=12498b3em08(暂无昵称), createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431148, encodeId=f1e1143114850, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=)]
    2018-03-16 kksonne

    #ASH#

    0

  4. [GetPortalCommentsPageByObjectIdResponse(id=2030160, encodeId=2c872030160c6, content=<a href='/topic/show?id=d88913e2636' target=_blank style='color:#2F92EE;'>#PAP#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=13726, encryptionId=d88913e2636, topicName=PAP)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=82ba2500166, createdName=ms1467340776200723, createdTime=Tue May 08 19:37:00 CST 2018, time=2018-05-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=297149, encodeId=6a1c29e1493b, content=学习谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zMoyicyo6ia9f4IuLQAwZoxD6Hx4ibd5CMcOCYhStY6oDibbKK6O2X8iaicldO5ib8j1iapOIobIKCGiczU2A/0, createdBy=56251941490, createdName=虈亣靌, createdTime=Sat Mar 17 18:09:19 CST 2018, time=2018-03-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1301236, encodeId=83d9130123687, content=<a href='/topic/show?id=38e32852fe' target=_blank style='color:#2F92EE;'>#ASH#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2852, encryptionId=38e32852fe, topicName=ASH)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=39a0295, createdName=kksonne, createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1338179, encodeId=580a13381e957, content=<a href='/topic/show?id=cd2a262068' target=_blank style='color:#2F92EE;'>#APA#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2620, encryptionId=cd2a262068, topicName=APA)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ce072500140, createdName=12498b3em08(暂无昵称), createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431148, encodeId=f1e1143114850, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=)]
    2018-03-16 12498b3em08(暂无昵称)

    #APA#

    0

  5. [GetPortalCommentsPageByObjectIdResponse(id=2030160, encodeId=2c872030160c6, content=<a href='/topic/show?id=d88913e2636' target=_blank style='color:#2F92EE;'>#PAP#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=13726, encryptionId=d88913e2636, topicName=PAP)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=82ba2500166, createdName=ms1467340776200723, createdTime=Tue May 08 19:37:00 CST 2018, time=2018-05-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=297149, encodeId=6a1c29e1493b, content=学习谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zMoyicyo6ia9f4IuLQAwZoxD6Hx4ibd5CMcOCYhStY6oDibbKK6O2X8iaicldO5ib8j1iapOIobIKCGiczU2A/0, createdBy=56251941490, createdName=虈亣靌, createdTime=Sat Mar 17 18:09:19 CST 2018, time=2018-03-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1301236, encodeId=83d9130123687, content=<a href='/topic/show?id=38e32852fe' target=_blank style='color:#2F92EE;'>#ASH#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2852, encryptionId=38e32852fe, topicName=ASH)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=39a0295, createdName=kksonne, createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1338179, encodeId=580a13381e957, content=<a href='/topic/show?id=cd2a262068' target=_blank style='color:#2F92EE;'>#APA#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2620, encryptionId=cd2a262068, topicName=APA)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ce072500140, createdName=12498b3em08(暂无昵称), createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431148, encodeId=f1e1143114850, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Fri Mar 16 03:37:00 CST 2018, time=2018-03-16, status=1, ipAttribution=)]
    2018-03-16 zhouqu_8

    #综合征#

    0