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一例表现为肥厚性硬脑膜炎的滑膜炎、痤疮、脓疱病、骨肥厚和骨炎(SAPHO)综合征。

A case of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome presenting with hypertrophic pachymeningitis.

作者信息

Shiraishi Wataru, Hayashi Shintaro, Iwanaga Yasutaka, Murai Hiroyuki, Yamamoto Akifumi, Kira Jun-ichi

机构信息

Department of Neurology, JCHO Kyushu Hospital, 8-1 kishinoura, Yahatanishi-ku, Kitakyushu City, Fukuoka 806-8501, Japan; Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan.

Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan.

出版信息

J Neurol Sci. 2015 Feb 15;349(1-2):229-31. doi: 10.1016/j.jns.2014.12.020. Epub 2014 Dec 20.

Abstract

A 43-year-old woman with a 3-year history of headache, fever, and swelling of the forehead, presented to our hospital. A general examination revealed palmar and plantar pustules. Blood analyses showed an elevated white blood cell count, C-reactive protein level, and erythrocyte sedimentation rate. Brain MRI revealed a partially thickened cranial bone with gadolinium enhancement, and also abnormally enhanced dura mater. Bone scintigraphy showed involvement of the cranial bone and bilateral sternoclavicular joints. Palmar skin biopsy indicated palmoplantar pustulosis. From these results, SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome with associated hypertrophic pachymeningitis was diagnosed. After corticosteroid therapy and tonsillectomy, the clinical symptoms and radiological abnormalities were improved. Clinicians should be aware of SAPHO as a potential unusual cause of hypertrophic pachymeningitis.

摘要

一名43岁女性,有3年头痛、发热和前额肿胀病史,前来我院就诊。全身检查发现掌跖脓疱。血液分析显示白细胞计数、C反应蛋白水平和红细胞沉降率升高。脑部MRI显示颅骨部分增厚,钆增强,硬脑膜也异常增强。骨闪烁显像显示颅骨和双侧胸锁关节受累。掌部皮肤活检提示掌跖脓疱病。根据这些结果,诊断为伴有肥厚性硬脑膜炎的SAPHO(滑膜炎、痤疮、脓疱病、骨肥厚和骨炎)综合征。经过皮质类固醇治疗和扁桃体切除术后,临床症状和影像学异常得到改善。临床医生应意识到SAPHO是肥厚性硬脑膜炎的一种潜在不寻常病因。

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