JUNCTIONAL EPIDERMOLYSIS-BULLOSA - SHOULD WE ALLOW CIRCUMCISION

Background. Junctional epidermolysis bullosa is an uncommon heterogene ous syndrome transmitted by recessive autosomal inheritance. In additi on to the classical skin lesions, the syndrome may include mucosal inv olvement which further complicates treatment. A few cases with urinary tract lesions dominating long-term prognosis have been reported. Case report. An child born in 1987 had junctional epidermolysis bullosa as sociated with urethral stenosis. Skin lesions typical of generalized a trophic benign junctional epidermolysis bullosa were observed. Urinary tract involvement developed after ritual circumcision and led to sten osis of the urethral meatus. Endourethral dilatations were unsuccessfu l and led to complete stenosis of the urethra, requiring life-long ind welling suprapubic catheter. Discussion. Due to the risk of urinary tr act involvement, we discuss the risks involved with circumcision in ce rtain forms of junctional epidermolysis bullosa.