儿科

订阅

MedSci主页 / 所有栏目 / 儿科

勒雪病1例

2018-03-27 佚名 临床皮肤科杂志

勒雪病
关注

患儿女，2岁。

【一般资料】

患儿女，2岁。

【主诉】

因头部、面部及躯干红斑、丘疹及结痂1年，于2016年4月来我科门诊就诊。

【现病史】

患儿1年前无明显诱因头、面部出现绿豆至黄豆大水疱，逐渐增多并累及躯干。大部分皮损散在分布，部分融合结痂后自行脱落，未予治疗。1年来皮损反复发作并进行性加重，伴明显瘙痒。患儿发病以来，精神及饮食尚可，二便均正常。患儿系足月顺产，发育正常

【既往史】

既往无特殊病史，家族中无类似疾病患者。

【体格检查】

全身浅表淋巴结未扪及增大，肝、脾肋下未及。皮肤科检查：头皮、发际、耳后、躯干可见粟粒至黄豆大黄红色或暗红色丘疹及斑丘疹，部分区域可见出血性丘疱疹，皮损散在或群集分布，其上可见针头至绿豆大瘀点、瘀斑及脓疱，部分上覆棕黄色或褐色鳞屑性痂（图1A）。背部可见皮损消退后遗留的点状萎缩斑（图1B），口腔黏膜未见异常。



【辅助检查】

血常规中红细胞计数5.14×1012/L[正常值（4.0～4.5）×1012/L，以下同）、血红蛋白（HGB）118g/L（120～140g/L）、血小板（PLT）354×109/L[（100～300×109/L]、降钙素原（PCT）0.3%（0.108%～0.282%）、红细胞平均体积（MCV）73.9fL（80～100fL）、平均血红蛋白量（MCH）23pg（27～34pg）及平均血红蛋白浓度（MCHC）311g/L（320～360g/L）。肝、肾功能:尿素2.0mmol/L（2.5～8.21mmol/L）、γ-谷氨酰转肽酶3.0U/L（7～50U/L）、总胆红素3.3μmol/L（5.1～18.8μmol/L）、直接胆红素1.3μmol/L（1.7～6.8μmol/L）及间接胆红素2.0μmol/L（3.4～10.3μmol/L）。红细胞沉降率（ESR）47mm/1h（≤18mm/1h）。外周血涂片示淋巴细胞比例增高。颅骨X射线检查:颅骨可见多发骨质缺损影，边界较清楚（图2A、B）；胸部X射线检查：双肺野清晰，左侧第一肋骨内缘形态欠规则（图2C）。腹部B超：肝脏、胆囊、脾脏及胰腺均正常。皮损组织病理检查：真皮乳头水肿，真皮浅层可见片状组织细胞样细胞浸润，细胞体积较大，核不规则（图3A）。免疫组化：CD1a(+)（图3B）、S-100蛋白（+）（图3C）、CD3散在（+）、CD20少（+）、CD117少量（+）及增殖核抗原（Ki-67）约10%（+）。





【初步诊断】

勒雪病（Letterer-Siwe）。

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 (4)
#插入话题
  1. [GetPortalCommentsPageByObjectIdResponse(id=304806, encodeId=7808304806e0, content=谢谢分享.学习了!, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=f8892279657, createdName=Joyrong, createdTime=Wed Apr 11 15:53:39 CST 2018, time=2018-04-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300688, encodeId=059530068846, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2018/03/18/9fba4e8a3782366829a5645367fdb658.jpg, createdBy=b5732293938, createdName=青山颖钰, createdTime=Thu Mar 29 06:05:24 CST 2018, time=2018-03-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300301, encodeId=c5c33003019a, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=44, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTI06MUzr7AI0nTlsgpDTRvVB51yAMdGfTKe8roX48ibEeqHD1dz3UibUa9SibU24UPLcEozJiaSB67WvQ/132, createdBy=fca0106828, createdName=Ramsey, createdTime=Tue Mar 27 22:38:54 CST 2018, time=2018-03-27, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300281, encodeId=37ac300281e7, content=了解一下.谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=40, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cdnapi.center.medsci.cn/medsci/head/2019/12/23/2f51002e1349ccda5be11c1eea1e1e60.jpg, createdBy=a22097948, createdName=天地飞扬, createdTime=Tue Mar 27 22:00:44 CST 2018, time=2018-03-27, status=1, ipAttribution=)]
    2018-04-11 Joyrong

    谢谢分享.学习了!

    0

  2. [GetPortalCommentsPageByObjectIdResponse(id=304806, encodeId=7808304806e0, content=谢谢分享.学习了!, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=f8892279657, createdName=Joyrong, createdTime=Wed Apr 11 15:53:39 CST 2018, time=2018-04-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300688, encodeId=059530068846, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2018/03/18/9fba4e8a3782366829a5645367fdb658.jpg, createdBy=b5732293938, createdName=青山颖钰, createdTime=Thu Mar 29 06:05:24 CST 2018, time=2018-03-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300301, encodeId=c5c33003019a, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=44, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTI06MUzr7AI0nTlsgpDTRvVB51yAMdGfTKe8roX48ibEeqHD1dz3UibUa9SibU24UPLcEozJiaSB67WvQ/132, createdBy=fca0106828, createdName=Ramsey, createdTime=Tue Mar 27 22:38:54 CST 2018, time=2018-03-27, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300281, encodeId=37ac300281e7, content=了解一下.谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=40, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cdnapi.center.medsci.cn/medsci/head/2019/12/23/2f51002e1349ccda5be11c1eea1e1e60.jpg, createdBy=a22097948, createdName=天地飞扬, createdTime=Tue Mar 27 22:00:44 CST 2018, time=2018-03-27, status=1, ipAttribution=)]
    2018-03-29 青山颖钰

    学习了

    0

  3. [GetPortalCommentsPageByObjectIdResponse(id=304806, encodeId=7808304806e0, content=谢谢分享.学习了!, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=f8892279657, createdName=Joyrong, createdTime=Wed Apr 11 15:53:39 CST 2018, time=2018-04-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300688, encodeId=059530068846, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2018/03/18/9fba4e8a3782366829a5645367fdb658.jpg, createdBy=b5732293938, createdName=青山颖钰, createdTime=Thu Mar 29 06:05:24 CST 2018, time=2018-03-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300301, encodeId=c5c33003019a, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=44, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTI06MUzr7AI0nTlsgpDTRvVB51yAMdGfTKe8roX48ibEeqHD1dz3UibUa9SibU24UPLcEozJiaSB67WvQ/132, createdBy=fca0106828, createdName=Ramsey, createdTime=Tue Mar 27 22:38:54 CST 2018, time=2018-03-27, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300281, encodeId=37ac300281e7, content=了解一下.谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=40, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cdnapi.center.medsci.cn/medsci/head/2019/12/23/2f51002e1349ccda5be11c1eea1e1e60.jpg, createdBy=a22097948, createdName=天地飞扬, createdTime=Tue Mar 27 22:00:44 CST 2018, time=2018-03-27, status=1, ipAttribution=)]
    2018-03-27 Ramsey

    学习了

    0

  4. [GetPortalCommentsPageByObjectIdResponse(id=304806, encodeId=7808304806e0, content=谢谢分享.学习了!, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=f8892279657, createdName=Joyrong, createdTime=Wed Apr 11 15:53:39 CST 2018, time=2018-04-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300688, encodeId=059530068846, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2018/03/18/9fba4e8a3782366829a5645367fdb658.jpg, createdBy=b5732293938, createdName=青山颖钰, createdTime=Thu Mar 29 06:05:24 CST 2018, time=2018-03-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300301, encodeId=c5c33003019a, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=44, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTI06MUzr7AI0nTlsgpDTRvVB51yAMdGfTKe8roX48ibEeqHD1dz3UibUa9SibU24UPLcEozJiaSB67WvQ/132, createdBy=fca0106828, createdName=Ramsey, createdTime=Tue Mar 27 22:38:54 CST 2018, time=2018-03-27, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300281, encodeId=37ac300281e7, content=了解一下.谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=40, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cdnapi.center.medsci.cn/medsci/head/2019/12/23/2f51002e1349ccda5be11c1eea1e1e60.jpg, createdBy=a22097948, createdName=天地飞扬, createdTime=Tue Mar 27 22:00:44 CST 2018, time=2018-03-27, status=1, ipAttribution=)]
    2018-03-27 天地飞扬

    了解一下.谢谢分享!

    0