￭用語 伴性遺伝性魚鱗癬

魚の鱗のように皮膚の表面が硬くなる鱗屑を生じる皮膚病
　伴性遺伝性魚鱗癬（ぎょりんせん）とは、魚の鱗（うろこ）のように皮膚の表面が硬くなる鱗屑（りんせつ）を生じる皮膚疾患。伴性劣性遺伝で伝わり、ほとんどが男児に発症します。
　皮膚の表面は表皮細胞が細胞核を失って死んで作られる角質層で覆われており、この角質層は皮膚のバリア機能に重要な役割を果たしています。角質層には、垢（あか）になって自然にはがれ落ちては作られるターンオーバーという一定のサイクルがあり、その際、皮膚には古い角質層がスルリと落ちる巧みなメカニズムが備わっています。
　ところが、伴性遺伝性魚鱗癬においては、その機能がおかしくなって角質層がうまくはがれ落ちないために異常な角質層、すなわち魚の鱗のようにカサカサした鱗屑がみられるようになります。
　性染色体上に存在する遺伝子が親から子に伝わることを伴性遺伝といいますが、そのうち伴性劣性遺伝は第二世代以降の子孫に、親と対立する性格が伝わることをいいます。伴性遺伝で伝わるものはすべてＸ染色体上にあるのですが、伴性劣性遺伝子の数は約30種類ほどあります。
　伴性遺伝性魚鱗癬も、X 染色体上にあるステロイドサルファターゼ（ステロイドスルファターゼ）遺伝子の異常により発症します。ステロイドサルファターゼは、角質層にあるコレステロール硫酸から硫酸基を外してコレステロールにする酵素を作る遺伝子であるため、コレステロールに比べてくっつきやすいコレステロール硫酸が角質層の細胞間にたまり、古い角質層が落ちにくくなって発症します。
　出生児6000人に１人の頻度で発症し、生まれた時には皮膚症状がありませんが、数カ月から四肢の伸側を中心に、腋（わき）や肘（ひじ）など関節の屈側にも、比較的大きく暗褐色を呈する鱗屑がみられるようになります。体幹では腹部に鱗屑がみられます。毛穴に一致して、角質層が硬くなる角化がみられることもあります。合併症では、角膜の混濁を生じやすくなっています。
　皮膚症状は冬の時期に目立ち、夏には軽くなります。頭にも鱗屑を認めることも多いですが、成長とともに少なくなります。四肢の伸側、関節の屈側、腹部の鱗屑は、年齢を重ねても改善傾向を示しません。
　出生の際、全身が半透明の薄膜で包まれているケースもあり、これをコロジオン児といいます。
伴性遺伝性魚鱗癬の検査と診断と治療
　皮膚科、皮膚泌尿器科の医師は、皮膚の症状から診断します。伴性遺伝性魚鱗癬の確定診断は、白血球のステロイドサルファターゼの活性を測定して、活性が極めて低値であることからなされます。
　遺伝子の研究によって、伴性遺伝性魚鱗癬はステロイドサルファターゼという蛋白に異常があることがわかってきましたので、医師による診断の確定、遺伝相談のためには、遺伝子検査も役立ちます。
　また、アトピー性皮膚炎、尋常性魚鱗癬、水疱（すいほう）性魚鱗癬性紅皮症、小児乾燥性湿疹（しっしん）などと区別します。
　伴性遺伝性魚鱗癬には特効的な治療法はなく、対症療法が行われます。軽症には、皮膚の表面を滑らかにする尿素含有軟こう、ビタミンＡ含有軟こう、ビタミンＤ３軟こう、サリチル酸ワセリンが効きます。重症の場合は、エトレチナート剤（ビタミンＡ誘導体）を内服します。各々特有の副作用に注意が必要です。

タグ：用語（は） 尋常性魚鱗癬 魚鱗癬

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