Management of urinary tract in children with epidermolysis bullosa.
Epidermolysis bullosa is a group of rare genetic disorders characterized by noninflammatory blistering lesions of the skin occurring after minor mechanical trauma. In association with junctional epidermolysis bullosa, a syndrome of pyloric atresia has occasionally been noted in the literature. Several infants who had this combined disorder have been reported to have severe genitourinary tract involvement. Most of these patients have died at an early age because of severe urinary tract involvement. We describe a rare survivor who was initially treated with urinary diversion. Subsequent attempts at undiversion of this patient were unsuccessful. He is presently stable following rediversion. The entities of e. bullosa and e. bullosa/pyloric atresia are reviewed with emphasis on urologic associations.
Donatucci
C F
CF
Department of Surgery, Letterman Army Medical Center, Presidio, San Francisco, California.
Berger
T G
TG
Deshon
G E
GE
D002648:Child; D016109:Epidermolysis Bullosa, Junctional / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D011183:Postoperative Complications / Q000601:surgery; D011708:Pylorus / Q000002:abnormalities; D012086:Reoperation; D001743:Urinary Bladder / Q000601:surgery; D014547:Urinary Diversion; D014570:Urologic Diseases / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery