title: Fatal haemorrhage due to extensive fragility of medium- and large-sized arteries and veins in a young patient with neurofibromatosis 1.
abstract: BACKGROUND
  Neurofibromatosis type 1 (NF1) is one of the most frequently inherited diseases. Vascular manifestations have been reported, mostly concerning stenosis of the renal artery or spontaneous rupture of single arteries. Also, venous aneurysms have been reported.
  
  
  METHODS
  We present clinical and pathological findings of a young patient with NF1 with lethal bleeding due to spontaneous rupture of a lumbal and renal artery.
  
  
  RESULTS
  High fragility of several arteries as well as veins in the abdominal and pelvic arterial and venous system resulted in a destroyed vessel structure caused by invading neurogen fibres.
  
  
  CONCLUSIONS
  In rare cases, NF1 is associated with a severe systemic vasculopathy concerning the arterial and venous vascular system.
authors.affiliation: Institute of Pathology, Johann-Wolfgang-Goethe University Frankfurt, Frankfurt/Main, Germany. n.hinsch@em.uni-frankfurt.de
authors.forename: Nora
authors.initials: N
authors.lastname: Hinsch
authors.forename: Susanne
authors.initials: S
authors.lastname: Kriener
authors.forename: Ralf-Gerhard
authors.initials: RG
authors.lastname: Ritter
authors.forename: Katharina
authors.initials: K
authors.lastname: Holzer
mesh_terms: D000328:Adult; D001158:Arteries / Q000473:pathology*; D002198:Capillary Fragility; D017809:Fatal Outcome; D005260:Female; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D009456:Neurofibromatosis 1 / Q000150:complications*; D016491:Peripheral Vascular Diseases / Q000209:etiology*; D012422:Rupture, Spontaneous; D014680:Veins / Q000473:pathology*