Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Evaluation of adults with cutaneous lesions of vasculitis

Nicole Fett, MD
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD


Cutaneous vasculitis can occur as a consequence of multiple disorders and is characterized by a wide variety of clinical findings. Because other diseases may present with similar clinical features, histopathologic examination is essential for confirming the diagnosis.

Patients with vasculitis are at risk for vasculitis involving other organs. In addition, the cause of cutaneous vasculitis is not always immediately clear. The performance of a thorough patient history and physical examination will guide the selection of the appropriate laboratory and radiologic studies for patient evaluation.

The clinical, histopathologic, and laboratory assessment of adults with cutaneous lesions suspicious for vasculitis will be reviewed here. The assessment of childhood vasculitis, the assessment of patients with retiform purpura, and detailed information on specific types of cutaneous vasculitis are discussed elsewhere. (See "Vasculitis in children: Evaluation" and "Approach to the patient with retiform (angulated) purpura" and "Overview of and approach to the vasculitides in adults" and "Management of adults with idiopathic cutaneous small vessel vasculitis".)


Cutaneous vasculitis results from inflammation of the small or medium-sized blood vessels in the skin. Small blood vessels are capillaries, post-capillary venules, and non-muscular arterioles in the superficial and mid-dermis (<50 micrometers). Medium-sized vessels consist of 50 to 150 micrometer vessels with muscular walls in the deep dermis and subcutis [1-5].

Cutaneous vasculitis occurs in a wide variety of clinical settings (table 1). Examples of vasculitic disorders (vasculitides) that can present with cutaneous lesions include [6]:

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Apr 12, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37:187.
  2. Carlson JA. The histological assessment of cutaneous vasculitis. Histopathology 2010; 56:3.
  3. Gonzalez-Gay MA, Garcia-Porrua C, Pujol RM. Clinical approach to cutaneous vasculitis. Curr Opin Rheumatol 2005; 17:56.
  4. Jennette JC, Falk RJ. The role of pathology in the diagnosis of systemic vasculitis. Clin Exp Rheumatol 2007; 25:S52.
  5. Nagai Y, Hasegawa M, Igarashi N, et al. Cutaneous manifestations and histological features of microscopic polyangiitis. Eur J Dermatol 2009; 19:57.
  6. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
  7. Carlson JA, Ng BT, Chen KR. Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. Am J Dermatopathol 2005; 27:504.
  8. Gross RL, Brucker J, Bahce-Altuntas A, et al. A novel cutaneous vasculitis syndrome induced by levamisole-contaminated cocaine. Clin Rheumatol 2011; 30:1385.
  9. Chung C, Tumeh PC, Birnbaum R, et al. Characteristic purpura of the ears, vasculitis, and neutropenia--a potential public health epidemic associated with levamisole-adulterated cocaine. J Am Acad Dermatol 2011; 65:722.
  10. Ullrich K, Koval R, Koval E, et al. Five consecutive cases of a cutaneous vasculopathy in users of levamisole-adulterated cocaine. J Clin Rheumatol 2011; 17:193.
  11. McGrath MM, Isakova T, Rennke HG, et al. Contaminated cocaine and antineutrophil cytoplasmic antibody-associated disease. Clin J Am Soc Nephrol 2011; 6:2799.
  12. Xu LY, Esparza EM, Anadkat MJ, et al. Cutaneous manifestations of vasculitis. Semin Arthritis Rheum 2009; 38:348.
  13. Dhadly M, Dean SM, Eberhardt RT. Cutaneous changes in peripheral vascular arterial disease. In: Fitzpatrick's Dermatology in General Medicine, 7th ed, Wolff K, Goldsmith LA, Katz SI (Eds), McGraw-Hill, 2008. Vol 2, p.1667.
  14. Chen KR, Carlson JA. Clinical approach to cutaneous vasculitis. Am J Clin Dermatol 2008; 9:71.
  15. Chung L, Kea B, Fiorentino DF. Cutaneous vasculitis. In: Dermatology, 2nd ed., Bolognia JL, Jorizzo, JL, Rapini RP, et al. (Eds), Elsevier Limited, Spain 2008. Vol 1, p.347.
  16. Piette W. Purpura: mechanisms and differential diagnosis. In: Dermatology, 2nd ed, Bolognia JL, Jorizzo JL, Rapini RP, et al (Eds), Elsevier Limited, Spain 2008. Vol 1, p.321.
  17. Loricera J, Blanco R, Ortiz-Sanjuán F, et al. Single-organ cutaneous small-vessel vasculitis according to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides: a study of 60 patients from a series of 766 cutaneous vasculitis cases. Rheumatology (Oxford) 2015; 54:77.
  18. Minz RW, Chhabra S, Singh S, et al. Direct immunofluorescence of skin biopsy: perspective of an immunopathologist. Indian J Dermatol Venereol Leprol 2010; 76:150.
  19. Hung SP, Yang YH, Lin YT, et al. Clinical manifestations and outcomes of Henoch-Schönlein purpura: comparison between adults and children. Pediatr Neonatol 2009; 50:162.
  20. Savige J, Pollock W, Trevisin M. What do antineutrophil cytoplasmic antibodies (ANCA) tell us? Best Pract Res Clin Rheumatol 2005; 19:263.
  21. Grau RG. Churg-Strauss syndrome: 2005-2008 update. Curr Rheumatol Rep 2008; 10:453.
  22. Grotz W, Baba HA, Becker JU, Baumgärtel MW. Hypocomplementemic urticarial vasculitis syndrome: an interdisciplinary challenge. Dtsch Arztebl Int 2009; 106:756.
  23. Davis MD, Brewer JD. Urticarial vasculitis and hypocomplementemic urticarial vasculitis syndrome. Immunol Allergy Clin North Am 2004; 24:183.
  24. Solans-Laqué R, Bosch-Gil JA, Pérez-Bocanegra C, et al. Paraneoplastic vasculitis in patients with solid tumors: report of 15 cases. J Rheumatol 2008; 35:294.
  25. Fain O, Hamidou M, Cacoub P, et al. Vasculitides associated with malignancies: analysis of sixty patients. Arthritis Rheum 2007; 57:1473.
  26. Bachmeyer C, Wetterwald E, Aractingi S. Cutaneous vasculitis in the course of hematologic malignancies. Dermatology 2005; 210:8.