尿道狭窄をきたした先天性表皮水疱症

JUNCTIONAL EPIDERMOLYSIS BULLOSA WITH URETHRAL STENOSIS

　先天性表皮水疱症は比較的軽微な外力により皮膚あるいは粘膜に水疱を生じる遺伝性疾患である．先天性表皮水疱症に合併した尿道狭窄の治療経験の1例を報告する．先天性表皮水萢症患者の尿路病変に対しては，経尿道的手術や金属ブジーのような尿路上皮を損傷せしめるような治療は無効であるばかりでなく，さらに病変を悪化させる可能性が高いことを常に念頭に置かなければならない．現在われわれが施行している間歇的尿道カテーテル留置は効果のある1つの方法である．

Treatment of genitourinary complications associated with epidermolysis bullosa is not easy because surgical treatment often worsens the disease. A 35-year-old male with epidermolysis bullosa diagnosed at his birth was seen at our department. At the age of 8, he had urinary retention because of bullae at the meatus and had receive catheterization. After that he had difficulty of urination and received urethral dilatation every 2 or 3 years. At the age of 33, endoscopic urethrotomy was performed at another hospital, but urethral dilatation had not been easy. 2 years later he came to our department with dysuria and fever.