title: Vasculopathy in von Recklinghausen's neurofibromatosis--a diagnostic quandary.
abstract: Vasculopathy is a not well-described presentation of neurofibromatosis type 1 in the dermatologic literature. We report a fatal case of neurofibromatosis vasculopathy in a 28-year-old woman who presented with skin ulcers and livedo racemosa; extensive gastrointestinal ulcers; and infarction of the bowel, liver, and spleen. Being alert to this manifestation may avoid unnecessary delay in diagnosis and enable, whenever possible, appropriate therapy.
authors.affiliation: Department of Dermatology, University Hospital of Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria.
authors.forename: Gerlinde
authors.initials: G
authors.lastname: Obermoser
authors.forename: Bettina G
authors.initials: BG
authors.lastname: Zelger
authors.forename: Gunda
authors.initials: G
authors.lastname: Millonig
authors.forename: Norbert
authors.initials: N
authors.lastname: Sepp
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Vogel
authors.forename: Bernhard
authors.initials: B
authors.lastname: Zelger
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D009456:Neurofibromatosis 1 / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D012867:Skin / Q000473:pathology; D017445:Skin Diseases, Vascular / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D012883:Skin Ulcer / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology