Vasculopathy in von Recklinghausen's neurofibromatosis--a diagnostic quandary.

Obermoser, Gerlinde; Zelger, Bettina G; Millonig, Gunda; Sepp, Norbert; Vogel, Wolfgang; Zelger, Bernhard · J Am Acad Dermatol · 2004

case_report · Level V

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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.

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