Skip to main content

A Practical Approach to the Diagnosis, Evaluation, and Management of Cutaneous Small-Vessel Vasculitis


Cutaneous small-vessel vasculitis (CSVV) is a disorder characterized by neutrophilic inflammation predominantly limited to the superficial cutaneous postcapillary venules. CSVV may be idiopathic or may have a defined cause such as infection, medication, connective tissue disease, or malignancy. CSVV may also be associated with extracutaneous disease or systemic vasculitis. The most common clinical presentation of CSVV consists of symmetrically distributed palpable purpura of the lower extremities. In general, lesional skin biopsy samples should be examined with light microscopy and direct immunofluorescence for adult patients with suspected CSVV. A complete history, review of systems, physical examination, and selected laboratory studies also should be performed to assess for inciting causes or extracutaneous involvement of CSVV. Treatment varies and depends on the chronicity of CSVV, the severity of cutaneous involvement, and the presence or absence of both an underlying cause and extracutaneous involvement of CSVV. An isolated episode of CSVV associated with a known inciting factor may be managed by removal or treatment of the trigger, along with symptomatic measures. First-line systemic treatments for chronic, idiopathic CSVV include colchicine or dapsone, used singly or in combination. Recurrent, chronic, or severely symptomatic CSVV that does not respond to the aforementioned therapies may require initiation of an immunosuppressive agent such as azathioprine, mycophenolate mofetil, methotrexate, cyclosporine, or rituximab.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3


  1. 1.

    Hunder GG, Arend WP, Bloch DA, Calabrese LH, Fauci AS, Fries JF, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis: introduction. Arthritis Rheum. 1990;33(8):1065–7.

    CAS  PubMed  Article  Google Scholar 

  2. 2.

    Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11.

    CAS  PubMed  Article  Google Scholar 

  3. 3.

    Fiorentino DF. Cutaneous vasculitis. J Am Acad Dermatol. 2003;48(3):311–40.

    PubMed  Article  Google Scholar 

  4. 4.

    Russell JP, Gibson LE. Primary cutaneous small vessel vasculitis: approach to diagnosis and treatment. Int J Dermatol. 2006;45(1):3–13.

    PubMed  Article  Google Scholar 

  5. 5.

    Marzano AV, Vezzoli P, Berti E. Skin involvement in cutaneous and systemic vasculitis. Autoimmun Rev. 2013;12(4):467–76 (Epub 2012 Aug 16).

    PubMed  Article  Google Scholar 

  6. 6.

    Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, Garcia-Fuentes M. Cutaneous vasculitis in children and adults: associated diseases and etiologic factors in 303 patients. Medicine (Baltimore). 1998;77(6):403–18.

    CAS  PubMed  Article  Google Scholar 

  7. 7.

    Martinez-Taboada VM, Blanco R, Garcia-Fuentes M, Rodriguez-Valverde V. Clinical features and outcome of 95 patients with hypersensitivity vasculitis. Am J Med. 1997;102(2):186–91.

    CAS  PubMed  Article  Google Scholar 

  8. 8.

    Loricera J, Calvo-Rio V, Ortiz-Sanjuan F, Gonzalez-Lopez MA, Fernandez-Llaca H, Rueda-Gotor J, et al. The spectrum of paraneoplastic cutaneous vasculitis in a defined population: incidence and clinical features. Medicine (Baltimore). 2013;92(6):331–43.

    PubMed  Article  Google Scholar 

  9. 9.

    Piette WW. The differential diagnosis of purpura from a morphologic perspective. Adv Dermatol. 1994;9:3–23.

    CAS  PubMed  Google Scholar 

  10. 10.

    Piette WW. Purpura. In Callen JP, Jorizzo JL, Bolognia JL, Piette WW, Zone JJ, editors. Dermatological signs of internal disease. 4th ed. London: Saunders/Elsevier; 2009. p. 85–92.

  11. 11.

    Mehregan DR, Hall MJ, Gibson LE. Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol. 1992;26(3 Pt 2):441–8.

    CAS  PubMed  Article  Google Scholar 

  12. 12.

    Carlson JA. The histological assessment of cutaneous vasculitis. Histopathology. 2010;56(1):3–23.

    PubMed  Article  Google Scholar 

  13. 13.

    Fett N. Evaluation of adults with cutaneous lesions of vasculitis [Internet]. Topic 13769 Version 11.0. UpToDate; 2014 [updated 2013 Oct 10; cited 2014 Jan 16].

  14. 14.

    Dhadly M, Dean SM, Eberhardt RT. Cutaneous changes in peripheral vascular arterial disease. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick’s dermatology in general medicine. 7th ed. Vol. 2. New York (NY): McGraw-Hill Medical; 2008. p. 1667–79.

  15. 15.

    Sokumbi O, Wetter DA. Clinical features, diagnosis, and treatment of erythema multiforme: a review for the practicing dermatologist. Int J Dermatol. 2012;51(8):889–902.

    PubMed  Article  Google Scholar 

  16. 16.

    Hughey LC. Approach to the hospitalized patient with targetoid lesions. Dermatol Ther. 2011;24(2):196–206.

    PubMed  Article  Google Scholar 

  17. 17.

    Xu LY, Esparza EM, Anadkat MJ, Crone KG, Brasington RD. Cutaneous manifestations of vasculitis. Semin Arthritis Rheum. 2009;38(5):348–60 Epub 2008 Mar 20.

    PubMed  Article  Google Scholar 

  18. 18.

    Chung L, Kea B, Fiorentino DF. Cutaneous vasculitis. In: Bolognia JL, Jorizzo JL, Rapini RD, editors. Dermatology. 2nd ed. Vol. 1. St. Louis (MO): Mosby Elsevier; 2008. p. 347–67.

  19. 19.

    Wetter DA. Purpura. In: Mattuci-Cerinic M, Furst D, Fiorentino DF, editors. Skin manifestations of rheumatic disease. New York (NY): Springer; 2013. p. 47–54.

  20. 20.

    Sanchez NP, Van Hale HM, Su WP. Clinical and histopathologic spectrum of necrotizing vasculitis: report of findings in 101 cases. Arch Dermatol. 1985;121(2):220–4.

    CAS  PubMed  Article  Google Scholar 

  21. 21.

    Hodge SJ, Callen JP, Ekenstam E. Cutaneous leukocytoclastic vasculitis: correlation of histopathological changes with clinical severity and course. J Cutan Pathol. 1987;14(5):279–84.

    CAS  PubMed  Article  Google Scholar 

  22. 22.

    Cribier B, Couilliet D, Meyer P, Grosshans E. The severity of histopathological changes of leukocytoclastic vasculitis is not predictive of extracutaneous involvement. Am J Dermatopathol. 1999;21(6):532–6.

    CAS  PubMed  Article  Google Scholar 

  23. 23.

    Gibson LE, Winkelmann RK. Cutaneous granulomatous vasculitis: its relationship to systemic disease. J Am Acad Dermatol. 1986;14(3):492–501.

    CAS  PubMed  Article  Google Scholar 

  24. 24.

    Massa MC, Su WP. Lymphocytic vasculitis: is it a specific clinicopathologic entity? J Cutan Pathol. 1984;11(2):132–9.

    CAS  PubMed  Article  Google Scholar 

  25. 25.

    Davis MD, Daoud MS, Kirby B, Gibson LE, Rogers RS 3rd. Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis. J Am Acad Dermatol. 1998;38(6 Pt 1):899–905.

    CAS  PubMed  Google Scholar 

  26. 26.

    Bahrami S, Malone JC, Webb KG, Callen JP. Tissue eosinophilia as an indicator of drug-induced cutaneous small-vessel vasculitis. Arch Dermatol. 2006;142(2):155–61.

    PubMed  Article  Google Scholar 

  27. 27.

    Poterucha TJ, Wetter DA, Gibson LE, Camilleri MJ, Lohse CM. Histopathology and correlates of systemic disease in adult Henoch-Schönlein purpura: a retrospective study of microscopic and clinical findings in 68 patients at Mayo Clinic. J Am Acad Dermatol. 2013;68(3):420–4.e3 (Epub 2012 Sep 6).

    Google Scholar 

  28. 28.

    Schroeter AL, Copeman PW, Jordon RE, Sams WM Jr, Winkelmann RK. Immunofluorescence of cutaneous vasculitis associated with systemic disease. Arch Dermatol. 1971;103(3):254–9.

    Article  Google Scholar 

  29. 29.

    Byun JW, Song HJ, Kim L, Shin JH, Choi GS. Predictive factors of relapse in adult with Henoch-Schönlein purpura. Am J Dermatopathol. 2012;34(2):139–44.

    PubMed  Article  Google Scholar 

  30. 30.

    Gibson LE. Cutaneous vasculitis update. Dermatol Clin. 2001;19(4):603–15.

    CAS  PubMed  Article  Google Scholar 

  31. 31.

    Callen JP. A clinical approach to the vasculitis patient in the dermatologic office. Clin Dermatol. 1999;17(5):549–53.

    CAS  PubMed  Article  Google Scholar 

  32. 32.

    Kinney MA, Jorizzo JL. Small-vessel vasculitis. Dermatol Ther. 2012;25(2):148–57.

    PubMed  Article  Google Scholar 

  33. 33.

    Lotti T, Ghersetich I, Comacchi C, Jorizzo JL. Cutaneous small-vessel vasculitis. J Am Acad Dermatol. 1998;39(5 Pt 1):667–87.

    CAS  PubMed  Article  Google Scholar 

  34. 34.

    Fett N. Management of adults with idiopathic cutaneous small vessel vasculitis [Internet]. Topic 13785 Version 5.0. UpToDate; 2014 [updated 2013 Sep 6; cited 2014 Jan 16].

  35. 35.

    Callen JP. Colchicine is effective in controlling chronic cutaneous leukocytoclastic vasculitis. J Am Acad Dermatol. 1985;13(2 Pt 1):193–200.

    CAS  PubMed  Article  Google Scholar 

  36. 36.

    Asherson RA, Buchanan N, Kenwright S, Fletcher CM, Hughes GR. The normocomplementemic urticarial vasculitis syndrome: report of a case and response to colchicine. Clin Exp Dermatol. 1991;16(6):424–7.

    CAS  PubMed  Article  Google Scholar 

  37. 37.

    Sais G, Vidaller A, Jucgla A, Gallardo F, Peyri J. Colchicine in the treatment of cutaneous leukocytoclastic vasculitis: results of a prospective, randomized controlled trial. Arch Dermatol. 1995;131(12):1399–402.

    CAS  PubMed  Article  Google Scholar 

  38. 38.

    Swerlick RA, Lawley TJ. Small-vessel vasculitis and cutaneous vasculitis. In: Churg A, Churg J, editors. Systemic vasculitides. New York (NY): Igaku-Shoin Medical Publishers, Inc; 1991. p. 193–201.

  39. 39.

    Ruiz Villaverde R, Blasco Melguizo J, Martin Sanchez MC, Naranjo Sintes R. Annular leucocytoclastic vasculitis: response to dapsone. J Eur Acad Dermatol Venereol. 2002;16(5):544–6.

    Google Scholar 

  40. 40.

    Fredenberg MF, Malkinson FD. Sulfone therapy in the treatment of leukocytoclastic vasculitis: report of three cases. J Am Acad Dermatol. 1987;16(4):772–8.

    CAS  PubMed  Article  Google Scholar 

  41. 41.

    Fortson JS, Zone JJ, Hammond ME, Groggel GC. Hypocomplementemic urticarial vasculitis syndrome responsive to dapsone. J Am Acad Dermatol. 1986;15(5 Pt 2):1137–42.

    CAS  PubMed  Article  Google Scholar 

  42. 42.

    Haeberle MT, Adams WB, Callen JP. Treatment of severe cutaneous small-vessel vasculitis with mycophenolate mofetil. Arch Dermatol. 2012;148(8):887–8.

    PubMed  Article  Google Scholar 

  43. 43.

    Worm M, Sterry W, Kolde G. Mycophenolate mofetil is effective for maintenance therapy of hypocomplementaemic urticarial vasculitis. Br J Dermatol. 2000;143(6):1324.

    CAS  PubMed  Article  Google Scholar 

  44. 44.

    Lunardi C, Bambara LM, Biasi D, Zagni P, Caramaschi P, Pacor ML. Elimination diet in the treatment of selected patients with hypersensitivity vasculitis. Clin Exp Rheumatol. 1992;10(2):131–5.

    CAS  PubMed  Google Scholar 

  45. 45.

    Ferri C, Pietrogrande M, Cecchetti R, Tavoni A, Cefalo A, Buzzetti G, et al. Low-antigen-content diet in the treatment of patients with mixed cryoglobulinemia. Am J Med. 1989;87(5):519–24.

    CAS  PubMed  Article  Google Scholar 

  46. 46.

    Lopez LR, Davis KC, Kohler PF, Schocket AL. The hypocomplementemic urticarial-vasculitis syndrome: therapeutic response to hydroxychloroquine. J Allergy Clin Immunol. 1984;73(5 Pt 1):600–3.

    CAS  PubMed  Article  Google Scholar 

  47. 47.

    Chung L, Funke AA, Chakravarty EF, Callen JP, Fiorentino DF. Successful use of rituximab for cutaneous vasculitis. Arch Dermatol. 2006;142(11):1407–10.

    PubMed  Article  Google Scholar 

  48. 48.

    Sokumbi O, Wetter DA, Makol A, Warrington KJ. Vasculitis associated with tumor necrosis factor-α inhibitors. Mayo Clin Proc. 2012;87(8):739–45 Epub 2012 Jul 13.

    CAS  PubMed Central  PubMed  Article  Google Scholar 

  49. 49.

    Marzano AV, Borghi A, Stadnicki A, Crosti C, Cugno M. Cutaneous manifestations in patients with inflammatory bowel diseases: pathophysiology, clinical features, and therapy. Inflamm Bowel Dis. 2014;20(1):213–27.

    PubMed  Article  Google Scholar 

  50. 50.

    Marzano AV, Borghi A, Meroni PL, Crosti C, Cugno M. Immune-mediated inflammatory reactions and tumors as skin side effects of inflammatory bowel disease therapy. Autoimmunity. 2014 Jan 20 [Epub ahead of print].

  51. 51.

    Ong CS, Benson EM. Successful treatment of chronic leukocytoclastic vasculitis and persistent ulceration with intravenous immunoglobulin. Br J Dermatol. 2000;143(2):447–9.

    CAS  PubMed  Article  Google Scholar 

  52. 52.

    Sais G, Vidaller A, Servitje O, Jucgla A, Peyrí J. Leukocytoclastic vasculitis and common variable immunodeficiency: successful treatment with intravenous immune globulin. J Allergy Clin Immunol. 1996;98(1):232–3.

    CAS  PubMed  Article  Google Scholar 

  53. 53.

    Turner AN, Whittaker S, Banks I, Jones RR, Pusey CD. Plasma exchange in refractory cutaneous vasculitis. Br J Dermatol. 1990;122(3):411–5.

    CAS  PubMed  Article  Google Scholar 

  54. 54.

    Nett N, Callen JP. Leukocytoclastic vasculitis. In: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson I, editors. Treatment of skin disease: comprehensive therapeutic strategies. 4th ed. Edinburgh: Elsevier/Saunders; 2014. p. 384–7.

Download references


No sources of funding were used to prepare this review. M. R. Goeser, V. Laniosz, and D. A. Wetter have no conflicts of interest that are directly relevant to the content of this review.

Author information



Corresponding author

Correspondence to David A. Wetter.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Goeser, M.R., Laniosz, V. & Wetter, D.A. A Practical Approach to the Diagnosis, Evaluation, and Management of Cutaneous Small-Vessel Vasculitis. Am J Clin Dermatol 15, 299–306 (2014).

Download citation


  • Vasculitis
  • Connective Tissue Disease
  • Mycophenolate Mofetil
  • Dapsone
  • Systemic Vasculitis