Cutaneous Vasculitis

  • Harold Chen
Living reference work entry


Vasculitis, inflammation of the vessel wall, can result in mural destruction with hemorrhage, aneurysm formation, and infarction, or intimal-medial hyperplasia and subsequent stenosis leading to tissue ischemia and infarction. The skin, in part due to its large vascular bed, exposure to cold temperatures, and frequent presence of stasis, is involved in many distinct as well as unnamed vasculitic syndromes that vary from localized and self-limited to generalized and life-threatening with multi-organ disease. Cutaneous vasculitis comprises a wide spectrum of overlapping primary and secondary disease entities that are characterized by predominant skin involvement and varying degrees of systemic manifestations (Carlson et al. 2005).


Kawasaki Disease Giant Cell Arteritis Takayasu Arteritis Hairy Cell Leukemia Mixed Connective Tissue Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. Booth, A., Harper, L., Hammad, T., et al. (2004). Prospective study of TNFalpha blockade with infliximab in anti-neutrophil cytoplasmic antibody associated systemic vasculitis. Journal of the American Society of Nephrology, 15, 717–721.CrossRefPubMedGoogle Scholar
  2. Carlson, J. A., & Chen, K.-R. (2007). Cutaneous vasculitis update: Neutrophilic muscular vessel and eosinophilic, granulomatous, and lymphocytic vasculitis syndrome. The American Journal of Dermatopathology, 29, 32–43.CrossRefPubMedGoogle Scholar
  3. Carlson, J. A., Ng, B. T., & Chen, K.-R. (2005). Cutaneous vasculitis update: Diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. The American Journal of Dermatopathology, 27, 504–528.CrossRefPubMedGoogle Scholar
  4. Chen, K.-R., & Carlson, J. A. (2008). Clinical approach to cutaneous vasculitis. American Journal of Clinical Dermatology, 9(2), 71–92.CrossRefPubMedGoogle Scholar
  5. Garcia-Porrua, C., Llorca, J., Gonzalez-Louzao, C., et al. (2001). Hypersensitivity vasculitis in adults: A benign disease usually limited to skin. Clinical and Experimental Rheumatology, 19, 85–88.PubMedGoogle Scholar
  6. Guillevin, L., Lhote, F., Amouroux, J., et al. (1996). Antineutrophil cytoplasmic antibodies, abnormal angiograms and pathological findings in polyarteritis nodosa and Churg-Strauss syndrome: Indications for the classification of vasculitides of the polyarteritis Nodosa Group. British Journal of Reumatology, 35, 958–964.CrossRefGoogle Scholar
  7. Jennette, J. C., Falk, R. J., Andrassy, K., et al. (1994). Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis and Rheumatology, 37, 187–192.CrossRefGoogle Scholar
  8. Jennette, J. C., Falk, R. J., Bacon, P. A., et al. (2013). 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis and Rheumatism, 65, 1–11.CrossRefPubMedGoogle Scholar
  9. Lotti, T., Ghersetich, I., Comacchi, C., et al. (1998). Cutaneous small-vessel vasculitis. Journal of the American Academy of Dermatology, 39, 667–687. quiz 688–90.CrossRefPubMedGoogle Scholar
  10. Raza, K., Thambyrajah, J., Townend, J. N., et al. (2000). Suppression of inflammation in primary systemic vasculitis restores vascular endothelial function: Lessons for atherosclerotic disease? Circulation, 102, 1470–1472.CrossRefPubMedGoogle Scholar
  11. Sais, G., Vidaller, A., Jucgla, A., et al. (1998). Prognostic factors in leukocytoclastic vasculitis: A clinicopathologic study of 160 patients. Archives of Dermatology, 134, 309–315.CrossRefPubMedGoogle Scholar
  12. Sorensen, S. F., Slot, O., Tvede, N., et al. (2000). A prospective study of vasculitis patients collected in a five year period: Evaluation of the Chapel Hill nomenclature. Annals of the Rheumatic Diseases, 59, 478–482.PubMedCentralCrossRefPubMedGoogle Scholar
  13. Willcocks, L., Chelliah, G., Brown, R., et al. (2003). Cutaneous vasculitis – A case for laparotomy? Journal of Rheumatology, 30, 1621–1623.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Medical GeneticsShriners Hospitals for ChildrenShreveportUSA
  2. 2.Perinatal and Clinical Genetics, Department of PediatricsLSU Health Sciences CenterShreveportUSA

Personalised recommendations