Skip to main content

Advertisement

SpringerLink
Log in

Primary cutaneous small vessel vasculitis

  • Published:
Current Treatment Options in Cardiovascular Medicine Aims and scope Submit manuscript

Opinion statement

Disorders associated with cutaneous vasculitis include numerous well-described etiologies. Primary cutaneous vasculitis limits discussion to primary leukocytoclastic vasculitis, essential mixed cryoglobulinemia, urticarial vasculitis, Henoch-Schönlein purpura, and erythema elevatum diutinum. Although the therapeutics for these disorders are based on limited data, we attempt to construct a consensus opinion on the management of primary cutaneous vasculitis. Therapy of primary cutaneous vasculitis is indicated for symptomatic or systemic involvement, because cutaneous small vessel vasculitis is frequently a self-limited, single episodic disease. Conservative, symptomatic treatment includes leg elevation, warming, antihistamines, and nonsteroidal anti-inflammatory drugs. For mild recurrent disease, colchicine, dapsone, and prednisone are first-choice agents. Systemic or severe cutaneous disease requires more potent immunosuppression (eg, prednisone, azathioprine, or mycophenolate mofetil). Plasmapheresis/plasma exchange and intravenous immunoglobulin are viable considerations for refractory disease, but are cumbersome and expensive modalities. There is insufficient evidence to advocate the use of new biological or monoclonal antibody therapies in primary cutaneous vasculitis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Fiorentino DF: Cutaneous vasculitis. J Am Acad Dermatol 2003, 48:311–340. Comprehensive review on the subject of cutaneous vasculitis with an excellent table summarizing therapeutic options for individual vasculitic entities.

    Article  PubMed  Google Scholar 

  2. Lotti T, Ghersetich I, Comacchi C, Jorizzo J: Cutaneous small vessel vasculitis. J Am Acad Dermatol 1998, 39:667–687.

    Article  PubMed  CAS  Google Scholar 

  3. Venzor J, Lee WL, Huston DP: Urticarial vasculitis. Clin Rev Allergy Immunol 2002, 23:201–216. Excellent review of UV.

    Article  PubMed  Google Scholar 

  4. 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, 102:186–191.

    Article  PubMed  CAS  Google Scholar 

  5. Callen JP Ekenstam E: Cutaneous leukocytoclastic vasculitis: clinical experience in 44 patients. Southern Med J 1987, 80:848–851.

    Article  CAS  PubMed  Google Scholar 

  6. Mehregan DR, Hall MJ, Gibson LE: Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol 1992, 26:441–448.

    PubMed  CAS  Google Scholar 

  7. Atzori L, Ferreli C, Biggio P: Less common treatment in cutaneous vasculitis. Clin Dermatol 1999, 17:641–647. Solid review of traditional medications used for very limited, mild cutaneous vasculitis.

    Article  PubMed  CAS  Google Scholar 

  8. Callen JP: Leukocytoclastic vasculitis. In Treatment of Skin Disease—Comprehensive Therapeutic Strategies. Edited by Lebwohl M, et al. London: Mosby/Elsevier Science Limited; 2002:370–343. Concise, although limited review of LCV treatment options and the evidence in the literature.

    Google Scholar 

  9. Vena G, Cassano N: Immunosuppressive therapy in cutaneous vasculitis. Clin Dermatol 1999, 17:633–640.

    Article  PubMed  CAS  Google Scholar 

  10. Lunardi C, Bambara LM, Biasi D, et al.: Elimination diet in the treatment of selected patients with hypersensitivity vasculitis. Clin Exp Rheumatol 1992, 10:131–135.

    PubMed  CAS  Google Scholar 

  11. Ferri C, Pietrogrande M, Cecchetti R, et al.: Low-antigen diet in the treatment of patients with mixed cryoglobulinemia. Am J Med 1989, 87:519–524.

    Article  PubMed  CAS  Google Scholar 

  12. Callen JP: Colchicine is effective in chronic cutaneous leukocytoclastic vasculitis. J Am Acad Dermatol 1985, 13:193–200.

    PubMed  CAS  Google Scholar 

  13. Sais G, Vidaller A, Jucgla A, et al.: Colchicine in the treatment of cutaneous leukocytoclastic vasculitis. Results of a prospective, randomized controlled trial. Arch Dermatol 1995, 131:1399–1402.

    Article  PubMed  CAS  Google Scholar 

  14. Asherson RA, Buchanan N, Kenwright S, et al.: The normocomplementemic vasculitic syndrome—report of a case and response to colchicine. Clin Exp Dermatol 1991, 16:424–427.

    Article  PubMed  CAS  Google Scholar 

  15. Pyne D, Mootoo R, Bhanji A: Colchicine for the treatment of recurrent Henoch-Schönlein purpura in an adult. Rheumatology 2001, 40:1430–1431.

    Article  PubMed  CAS  Google Scholar 

  16. Muramatsu C, Tanabe E: Urticarial vasculitis: response to dapsone and colchicine. J Am Acad Dermatol 1985, 13:1055.

    PubMed  CAS  Google Scholar 

  17. Freedberg IM, Eisen AZ, Wolff K, eds: Fitzpatrick’s Dermatology in General Medicine, edn 6. New York, NY: McGraw-Hill; 2003.

    Google Scholar 

  18. Grabbe J, Haas N, Moller A, Henz BM: Erythema elevatum diutinum—evidence for disease dependent leukocyte alterations and response to dapsone. Br J Dermatol 2000, 143:415–420.

    Article  PubMed  CAS  Google Scholar 

  19. Fredenberg MF, Malkinson FD: Sulfone therapy in the treatment of leukocytoclastic vasculitis. Dermatology 1987, 16:772–778.

    CAS  Google Scholar 

  20. Forston JS, Zone J, Hammond ME, Groggel GC: Hypocomplementemic urticarial vasculitis syndrome responsive to dapsone. J Am Acad Dermatol 1986, 15:1137–1142.

    Article  Google Scholar 

  21. Eiser AR, Singh P, Shanies HM: Sustained dapsoneinduced remission of hypocomplementemic urticarial vasculitis—a case report. Angiology 1997, 48:1019–1022.

    PubMed  CAS  Google Scholar 

  22. Ramelli GP, Bianchetti MG: Dapsone in cutaneous Henoch-Schönlein syndrome—worth a trial. Acta Paediatr 1997, 86:337.

    PubMed  CAS  Google Scholar 

  23. Sarma PS: Dapsone in Henoch-Schönlein purpura. Postgrad Med J 1994, 70:464–465.

    PubMed  CAS  Google Scholar 

  24. Nürnberg W, Grabbe J, Czarnetzki M: Urticarial vasculitis syndrome effectively treated with dapsone and pentoxifylline. Acta Derm Venereol 1995, 75:54–56.

    PubMed  Google Scholar 

  25. Nürnberg W, Grabbe J, Czarnetzki M: Synergistic effects of pentoxifylline and dapsone in leukocytoclastic vasculitis. Lancet 1994, 343:491.

    Article  PubMed  Google Scholar 

  26. Rhodes LE, Tingle MD, Park BK, et al.: Cimetidine improves the therapeutic/toxic ratio of dapsone in patients on chronic therapy. Br J Dermatol 1995, 132:257–262.

    Article  PubMed  CAS  Google Scholar 

  27. Lopez LR, Davis KC, Kohler PF, Schocket AL: The hypocomplementemic urticarial-vasculitis syndrome: therapeutic response to hydroxychloroquine. J Allergy Clin Immunol 1984, 73:600–603.

    Article  PubMed  CAS  Google Scholar 

  28. Calderon MJ, Landa N, Aguirre A, Diaz-Perez JL: Successful treatment of cutaneous PAN with pentoxifylline. Br J Dermatol 1993, 128:706–708.

    Article  PubMed  CAS  Google Scholar 

  29. Wahba-Yahav AV: Chronic leukocytoclastic vasculitis associated with polycythemia vera: effective control with pentoxifylline. J Am Acad Dermatol 1992, 6:1006–1007.

    Article  Google Scholar 

  30. Callen JP, Spencer L, Burruss JB, Holtman J: Azathioprine: an effective corticoid-sparing therapy for patients with lupus erythematosus or with recalcitrant cutaneous leukocytoclastic vasculitis. Arch Dermatol 1991, 127:515–522.

    Article  PubMed  CAS  Google Scholar 

  31. Singh S, Devidayal L, Kumar L, et al.: Severe Henoch-Schönlein nephritis: resolutions with azathioprine and steroids. Rheumatol Int 2002, 22:133–137.

    Article  PubMed  CAS  Google Scholar 

  32. Renard M, Wouters C, Proesmans W: Rapidly progressive glomerulonephritis in a boy with hypocomplementemic urticarial vasculitis. Eur J Pediatr 1998, 157:243–245.

    Article  PubMed  CAS  Google Scholar 

  33. Wolverton SE, ed: Comprehensive Dermatologic Drug Therapy. Philadelphia: WB Saunders; 2001.

    Google Scholar 

  34. Worm M, Muche M, Schulze P, et al.: Hypocomplementemic urticarial vasculitis: successful treatment with cyclophosphamide-dexamethasone pulse therapy. Br J Dermatol 1998, 139:704–707.

    Article  PubMed  CAS  Google Scholar 

  35. Cupps TR, Springer RM, Fauci AS: Chronic, recurrent small-vessel cutaneous vasculitis. JAMA 1982, 247:1994–1998.

    Article  PubMed  CAS  Google Scholar 

  36. Georganas C, Ioakimidis D, Iatrou C, et al.: Relapsing Wegener’s granulomatosis: successful treatment with cyclosporin-A. Clin Rheumatol 1996, 15:189–192.

    Article  PubMed  CAS  Google Scholar 

  37. Tosca AD, Ioannidou DJ, Katsantonis JC, Kyriakis KP: Cyclosporin A in the treatment of cutaneous vasculitis. Clinical and cellular effects. J Eur Acad Dermatol Venereol 1996, 6:135–141.

    Article  Google Scholar 

  38. Stratigos JD, Katsambas A, Potouridou E: Cyclosporin—a therapy for allergic cutaneous vasculitis with multiple recurrences. J Dermatol Treat 1994, 5:93–95.

    Google Scholar 

  39. Jorizzo JL, White WL, Wise CM, et al.: Low-dose methotrexate for unusual neutrophilic vasculitic reactions: cutaneous polyarteritis nodosa and Behçet’s disease. J Am Acad Dermatol 1991, 24:973–978.

    PubMed  CAS  Google Scholar 

  40. Callen JP: A clinical approach to the vasculitic patient in the dermatologic office. Clin Dermatol 1999, 17:549–553.

    Article  PubMed  CAS  Google Scholar 

  41. Van der Woude FJ, Schmitt WH, Birck R, et al.: Immunosuppression in ANCA-associated vasculitis. Transplant Proc 2001, 33:2225–2226.

    Article  PubMed  Google Scholar 

  42. Worm M, Sterry W, Kolde G: Mycophenolate mofetil is effective for maintenance therapy of hypocomplementemic urticarial vasculitis. Br J Dermatol 2000, 142:1324.

    Article  Google Scholar 

  43. Ong CS, Benson EM: Successful treatment of chronic leukocytoclastic vasculitis and persistent ulceration with intravenous immunoglobulin. Br J Dermatol 2000, 143:447–449.

    Article  PubMed  CAS  Google Scholar 

  44. Sais G, Vidaller A, Servitje O, et al.: Leukocytoclastic vasculitis and common variable immunodeficiency: successful treatment with intravenous immune globulin. J Allergy Clin Immunol 1996, 98:232–233.

    Article  PubMed  CAS  Google Scholar 

  45. Gedalia A, Correa H, Kaiser M, Sorensen R: Case report: steroid sparing effect of intravenous gamma globulin in a child with necrotizing vasculitis. Am J Med Sci 1995, 309:226–228.

    Article  PubMed  CAS  Google Scholar 

  46. Gaskin G, Pusey CD: Plasmapheresis in antineutrophil cytoplasmic antibodies-associated systemic vasculitis. Ther Apher 2001, 5:176–181.

    Article  PubMed  CAS  Google Scholar 

  47. Turner AN, Whittaker S, Banks I, et al.: Plasma exchange in refractory cutaneous vasculitis. Br J Dermatol 1990, 122:411–415.

    Article  PubMed  CAS  Google Scholar 

  48. Wysenbeek AJ, Calabrese LH, Mandel DR, Clough JD: Limited plasmapheresis in fulminant leukocytoclastic vasculitis. J Rhematol 1982, 9:315–318.

    CAS  Google Scholar 

  49. Hattori M, Ito K, Konomoto T, et al.: Plasmapheresis as sole therapy for rapidly progressive Henoch-Schönlein purpura nephritis in children. Am J Kidney Dis 1999, 33:427–433.

    PubMed  CAS  Google Scholar 

  50. Levine SM, Stone JH: New approaches to treatment in systemic vasculitis: biologic therapies. Best Pract Res Clin Rheumatol 2001, 15:315–333.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Dermatology, Mayo Clinic, 200 First Street, SW, 55905, Rochester, MN, USA

    James P. Russell MD & Roger H. Weenig MD

Authors
  1. James P. Russell MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Roger H. Weenig MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Russell, J.P., Weenig, R.H. Primary cutaneous small vessel vasculitis. Curr Treat Options Cardio Med 6, 139–149 (2004). https://doi.org/10.1007/s11936-004-0042-3

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11936-004-0042-3

Keywords

Search

Navigation