姉妹に見られた円板状ならびに全身性エリテマトーデス

CHRONIC DISCOID AND SYSTEMIC LUPUS ERYTHEMATOSUS IN SISTERS

I．はじめに

　紅斑性狼瘡（以下LE）が，その多様な病像から皮膚科ばかりでなく各科領域での興味をひくようになつてすでに久しいが，その家族発生例については，Weidenhammer^1）のlupus vulgarisとLEとを併有する兄の弟にLEをみた報告をはじめとして，稀なものとされながらも次第にその数を増し，Hauser^2）（1958）は，世界文献に39例をみるとしている。わが国でも，小林^3）は，LE患者の家族で血清補体値の低値をみるとして，家族発生の要因の一端を明らかにしているが，報告例の多くは，急性播種状紅斑性狼瘡（以下SLE）または慢性円板状紅斑性狼瘡（以下DLE）のいずれか1型の発生例で，SLEとDLEとの組み合わせはそれ程多くはなく，さらにそれを兄弟ないしは姉妹に限定してみると稀有なもののようである。最近われわれは姉にDLE，その妹にSLEの発症をみた例を経験したので報告する。

Case 1 : A 38-year-old woman mentioned erythema on the right cheek and nasal back at the age of 28. She had been treated with antimalarials for about one year under the diagnosis of discoid lupus erythematosus. It recurred on the left cheek at 37 years of age.

Slightly reversed A/G ratio in serum was discovered. Histologic picture was typical.

Case 2 : A 18-year-old woman, a sister of case 1, found erythema with whitish pityriatic scales on the both cheeks. One month later the rashes were developed with fever, arthralgia and Raynaud's phenomenon.

She showed remission after systemic administration of adrenocortical steroids. Histologic picture was typical SLE. Marked increase of serum r-globulin was found.

As a mechanism of familiar occurrence of lupus erythematosus, there is a theory of transplac-entar transfer of LE factor or of hereditary dysproteinemia. In our cases abnormality in serum protein was proved, but the mechanism of familiar occurrence of them was difficult to be explained.