The need to distinguish true primary systemic vasculitis from its multiple potential mimickers is one of the most challenging diagnostic conundrums in clinical medicine. This article reviews 9 challenging vasculitis mimickers: fibromuscular dysplasia, calciphylaxis, segmental arterial mediolysis, antiphospholipid syndrome, hypereosinophilic syndrome, lymphomatoid granulomatosis, malignant atrophic papulosis, livedoid vasculopathy, and immunoglobulin G4-related disease.
Keywords: Degos disease; Fibromuscular dysplasia; IgG4-related disease; Livedoid vasculopathy; Lymphomatoid granulomatosis; Mimicker; Segmental arterial mediolysis; Vasculitis.