天疱瘡群と類天疱瘡群
上皮の接着機構が自己抗体(IgG)によって阻害されることで水疱などが生じる自己免疫疾患である。
上皮が剥がれた場所に水が溜まるようなイメージで水疱が形成される。
[治療]
治療はステロイド内服(ステロイド全身投与)が主体となる。
難治性の場合に他の治療として血漿交換療法や免疫抑制剤投与や大量ガンマグロブリン静注(IVIG)療法が候補に挙がる。
天疱瘡群
上皮細胞間接着を司るデスモソームの構成タンパク質であるデスモグレインが自己抗体によって阻害されることで起こる疾患である。
デスモグレイン1は主に上層表皮細胞間に存在しており、デスモグレイン3は主に下層表皮細胞間と粘膜上皮細胞間に存在する。
デスモグレイン1とデスモグレイン3が阻害されると尋常性天疱瘡(口腔内病変が特徴)となり、デスモグレイン1のみが阻害されると落葉状天疱瘡になる。
尋常性天疱瘡
粘膜病変である口腔内病変が特徴的である。
表皮の下層に水泡が形成される。
落葉状天疱瘡
表皮の上層に水泡が形成される。
薄い鱗屑を伴った紅斑が特徴である。
類天疱瘡群
表皮基底膜部において基底層表皮細胞と真皮(細胞外基質)の接着を司るヘミデスモソームの構成タンパク質(BP180・BP230・Ⅶ型コラーゲン)のうち、基底層表皮細胞側に存在するBP180・BP230が自己抗体によって阻害されると水疱性類天疱瘡になり、真皮側に存在するⅦ型コラーゲンが阻害されると後天性表皮水疱症となる。
水泡性類天疱瘡
食塩水処理皮膚を用いた蛍光抗体間接法にて表皮側にIgG陽性反応を認める。
後天性表皮水泡症
食塩水処理皮膚を用いた蛍光抗体間接法にて真皮側にIgG陽性反応を認める。
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