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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Linear scleroderma "en coup de sabre"の日本語訳

Patxi Zavala Gottau1, Francisco Caiza-Zambrano2, Luis Ariel Miquelini3

  • 1Neurology Department, Hospital Britanico de Buenos Aires, Buenos Aires, Argentina patxizavalagottau@gmail.com.

Practical neurology
|January 23, 2026
PubMed
まとめ
この要約は機械生成です。

線維性強皮症「en coup de sabre」は、成人発症の稀な疾患であり、頭皮病変やてんかんなどの神経学的問題として現れることがある。早期診断とミコフェノール酸モフェチルによる治療は、病状を安定させることができる。

キーワード:
抗けいれん薬臨床神経学てんかん免疫学MRI

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Last Updated: May 11, 2026

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科学分野:

  • 皮膚科学;神経学;病理学

背景:

  • 線維性強皮症「en coup de sabre」は、稀な炎症性および線維化性の皮膚疾患である。;成人では稀に発生し、主に神経学的である皮膚外症状を呈することは稀である。

研究 の 目的:

  • 神経学的関与を伴う成人発症の線維性強皮症「en coup de sabre」の症例を報告すること。;頭皮病変および痙攣を呈する成人において、この診断を考慮することの重要性を強調すること。

主な方法:

  • 前頭部の病変およびてんかんを有する43歳女性の症例提示。;神経学的変化を評価するための磁気共鳴（MR）脳画像検査。;強皮症の組織病理学的確認のための皮膚生検。

主要な成果:

  • 患者は、前頭部の傷跡様の病変とてんかんを呈した。;頭蓋内MRI検査では、同側の局所性皮質萎縮と白質変化が明らかになった。;皮膚生検では、強皮症に一致する硝子様硬化が確認された。;ミコフェノール酸モフェチルにより臨床的安定が得られた。

結論:

  • 成人発症の線維性強皮症「en coup de sabre」は、稀ではあるが、頭皮病変およびてんかんを有する成人において考慮されるべきである。;成人発症例では神経学的症状が重要である。;ミコフェノール酸モフェチルは有効な治療選択肢となる可能性がある。