F. Cherif et al., JUNCTIONAL EPIDERMOLYSIS-BULLOSA - SHOULD WE ALLOW CIRCUMCISION, Annales de dermatologie et de venereologie, 125(10), 1998, pp. 724-726
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.