Challenging mimickers of primary systemic vasculitis

Rheum Dis Clin North Am. 2015;41(1):141-60, ix. doi: 10.1016/j.rdc.2014.09.011.


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.

Publication types

  • Review

MeSH terms

  • Antiphospholipid Syndrome / diagnosis*
  • Arterial Occlusive Diseases / diagnosis*
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / immunology
  • Calciphylaxis / diagnosis*
  • Diagnosis, Differential
  • Fibromuscular Dysplasia / diagnosis
  • Humans
  • Hypereosinophilic Syndrome / diagnosis*
  • Immunoglobulin G / immunology
  • Lymphomatoid Granulomatosis / diagnosis*
  • Malignant Atrophic Papulosis / diagnosis
  • Vascular Diseases / diagnosis
  • Vasculitis / diagnosis*


  • Immunoglobulin G