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Annales de Dermatologie et de Vénéréologie
Vol 125, N° 10  - octobre 1998
p. 724
Doi : AD-10-1998-125-10-0151-9638-101019-ART60
Junctionnal epidermolysis bullosa: should weallow circoncision?
 

F. CHERIF (2), B. FAZAA (1), I. MOKHTAR (1), M. R. KAMOUN (1)
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Cas clinique

ÉPIDERMOLYSES BULLEUSES JONCTIONNELLES : FAUT-IL PERMETTRE LA CIRCONCISION ?

Introduction.

Les épidermolyses bulleuses jonctionnelles sont un syndromehétérogène rare, transmis sur un mode autosomique récessif. A côté de l'atteintecutanée classiquement observée, des atteintes muqueuses peuvent se voir, augmentant lesdifficultés de prise en charge. L'atteinte du tractus urinaire a été rapportée dansquelques cas, dominant alors le pronostic à long terme.

Observation.

Nous rapportons l'observation d'un enfant né en 1987 atteint d'uneépidermolyse bulleuse jonctionnelle associée à une sténose urétrale. Les lésionscutanées étaient similaires à celles observées au cours des épidermolyses bulleusesjonctionnelles généralisées atrophiques bénignes. L'atteinte urinaire est apparue audécours d'une circoncision rituelle, compliquée d'une sténose du méat urétral. Lesdilatations endo-urétrales ont abouti à une sténose de tout l'urètre, imposant la miseen place d'un cathéter suspubien à vie.

Discussion.

Etant donné le risque d'atteinte urinaire, nous discutons le risqued'une circoncision dans certaines formes d'épidermolyses bulleuses.

Abstract

Background.

Junctional epidermolysis bullosa is an uncommon heterogeneoussyndrome transmitted by recessive autosomal inheritance. In addition to the classical skinlesions, the syndrome may include mucosal involvement 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 bullosaassociated with urethral stenosis. Skin lesions typical of generalized atrophic benignjunctional epidermolysis bullosa were observed. Urinary tract involvement developed afterritual circumcision and led to stenosis of the urethral meatus. Endourethral dilatationswere unsuccessful and led to complete stenosis of the urethra, requiring life-longindwelling suprapubic catheter.

Discussion.

Due to the risk of urinary tract involvement, we discuss the risksinvolved with circumcision in certain forms of junctional epidermolysis bullosa.

C

HERIF

F., F

AZAA

B., M

OKHTAR

I., K

AMOUN

M. R.




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