Vasculopathy in von Recklinghausen's neurofibromatosis--a diagnostic quandary.
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.
Obermoser
Gerlinde
G
Department of Dermatology, University Hospital of Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria.
Zelger
Bettina G
BG
Millonig
Gunda
G
Sepp
Norbert
N
Vogel
Wolfgang
W
Zelger
Bernhard
B
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