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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