水疱性類天疱瘡の1例

A CASE OF BULLOUS PEMPHIGOID

Ⅰ．はじめに

　1943年Civatteは尋常性天疱瘡に見られるacantholysisはジューリング氏疱疹状皮膚炎には見られず，また水疱の発生する部位が異ることを発表し，その後Lever^21）（1953）がbullous Pem-phigoidの名称で，本症を天疱瘡とジューリング氏疱疹状皮膚炎との中間に位置する独立疾患としてはじめて記載した。以来本症は数多くの異名をもち，Rook and Waddington^31）はpemphi-goidとして38例を，Prakken and Woerder-man^28）はparapemphigusとして19例を報告し，Steigleder^34）は高令者に多いことによりAlterspemphigusと呼び20例を発表した。またSneddon and Churchは高令者22例を観察しその臨床像につき詳細に述べ，^33）Kim and Win-kelmann^17）の小児例の報告もあり，他にBre-nnen and Montogomery^1），Herzberg^15）16），Stevenson^35），Tappeinerら^39），Saundersら^32）の報告がある。

　本症は比較的稀な症患とみなされているが，本邦においては仁木^25）（1959）が7例を集め本症の臨床像，組織的所見などをもととして本症を独立疾患として報告し，橋本教授^14）（1963）も本邦発表例23例に自験例を加えて本症の臨床症状などにふれ，文献的考察を加え，本症の独立性について記載している。

A sake brewer, aged 71, developed several erythemas ranging in size of a thumb to a hen's egg on the ventral aspects of his wrists and dorsum of his left hand about three months ago. The erythemas spread later over the entire body surface except on the face, scalp, palms and soles. Numerous bulla formations varied in size were seen on the bases of well marginated erythemas. The lesions developed on the tongue and soft palate too. The eruption was not pruritic and no Nikolsky sign could be elicited. Laboratory findings were all within the normal limit except serum potassium content (5.06mEq/1) which was slightly high. Iodine patch test showed negative reaction. Biopsy of the skin showed subepidermal bullae and no acantholysis was noticde.

As to treatment, systemic administration of perestone-N, dexamethasone (5mg/day) and erythrimycin (1200mg/day) depressed the new formation of bullae to a certain extent. Dexamethasone and betamethasone were slowly tapered off until discontinued. Bulla formation has not been observed since then. Germanin and anabolic hormone were administered during the corticosteroid had been tapered off.