31. Primary Cutaneous Vasculitis (Small Vessel Vasculitis)

  1. Gary S. Hoffman MD, MS2,
  2. Cornelia M. Weyand MD, PhD3,
  3. Carol A. Langford MD, MHS2 and
  4. Jörg J. Goronzy MD, PhD3
  1. Jeffrey P. Callen MD, FACP, FAAD

Published Online: 3 MAY 2012

DOI: 10.1002/9781118355244.ch31

Inflammatory Diseases of Blood Vessels, Second Edition

Inflammatory Diseases of Blood Vessels, Second Edition

How to Cite

Callen, J. P. (2012) Primary Cutaneous Vasculitis (Small Vessel Vasculitis), in Inflammatory Diseases of Blood Vessels, Second Edition (eds G. S. Hoffman, C. M. Weyand, C. A. Langford and J. J. Goronzy), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781118355244.ch31

Editor Information

  1. 2

    Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Cleveland Clinic, Lerner College of Medicine, Cleveland, OH, USA

  2. 3

    Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA

Author Information

  1. Division of Dermatology, University of Louisville School of Medicine, Louisville, KY, USA

Publication History

  1. Published Online: 3 MAY 2012
  2. Published Print: 8 JUN 2012

ISBN Information

Print ISBN: 9781444338225

Online ISBN: 9781118355244



  • Cutaneous vasculitis;
  • small vessel vasculitis;
  • hypersensitivity vasculitis;
  • Henoch–Schönlein purpura;
  • mixed cryoglobulinemia


Cutaneous vasculitis remains an enigmatic disorder. Acute disease is generally benign and relatively easily managed. The presence of cutaneous lesions even when there is no discernable systemic disease, represents a systemic process that likely is triggered by deposition of immune complexes, followed by a cascade of cytokine release, inflammatory mediators and inflammatory cells. Cutaneous disease can be isolated or be reflective of a systemic vasculitic syndrome. Half of the patients with chronic disease do not have a recognizable cause while in others there is an associated disease, particularly rheumatologic disorders, or an infection or an ingestant (primarily pharmacotherapy). Successful management of patients is generally possible.