Sections

Medication

Medication Summary

No effective therapy has been established in all patients with leukocytoclastic vasculitis (LCV). The authors most frequently use colchicine or dapsone as first-line options in patients with cutaneous-predominant LCV, who may or may not have associated arthralgia. Agents used for LCV affecting internal organs, and for life-threatening LCV, include corticosteroids, cytotoxic/immunosuppressive agents (eg, cyclophosphamide), and rituximab.^[51]Few therapies for LCV have been tested in controlled trials.

Class Summary

These agents inhibit key events involved in the inflammatory process.

Colchicine

View full drug information

Has effects against neutrophils, which are involved in the pathogenesis of cutaneous vasculitis. Has been demonstrated to be steroid sparing in open-label studies. The only double-blind, placebo-controlled trial failed to demonstrate efficacy; however, the study had several methodological errors.

Class Summary

Some types of antimicrobials (eg, dapsone) have anti-inflammatory properties.

Dapsone (Avlosulfon)

View full drug information

Small, open-label studies or single case reports have suggested that dapsone is effective in some patients with cutaneous vasculitis.

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

Prednisone (Deltasone, Orasone, Meticorten)

View full drug information

Indicated for vasculitis affecting internal organs such as kidneys, lungs, or CNS. Skin involvement with bullous lesions may require corticosteroid therapy in order to prevent cutaneous ulceration and scarring.

Class Summary

These agents inhibit cell growth and proliferation, decreasing immune reactions.

Cyclophosphamide (Cytoxan, Neosar)

View full drug information

Useful in life-threatening cases of vasculitis. Patients with only skin disease generally should not be treated with this agent. Useful in patients with polyarteritis nodosa, granulomatosis with polyangiitis (formerly known as Wegener granulomatosis), and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). Alkylating agent that depresses T-cell and B-cell function.

Azathioprine (Imuran)

View full drug information

Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.

Methotrexate (Rheumatrex, Folex PFS)

View full drug information

Unknown mechanism of action in treatment of inflammatory reactions; may affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness). Adjust dose gradually to attain satisfactory response.

Mycophenolate mofetil (CellCept)

View full drug information

Inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses de novo purine synthesis by lymphocytes, thereby inhibiting their proliferation. Inhibits antibody production.

Two formulations are available and are not interchangeable. The original formulation, mycophenolate mofetil (MMF, CellCept) is a prodrug that, once hydrolyzed in vivo, releases the active moiety mycophenolic acid. A newer formulation, mycophenolic acid (MPA, Myfortic) is an enteric-coated product that delivers the active moiety. The latter is often associated with less gastrointestinal disturbance.

Rituximab (Rituxan)

View full drug information

Antibody genetically engineered chimeric murine/human monoclonal antibody directed against the CD20 antigen found on the surface of normal and malignant B lymphocytes. Antibody is an IgG1-kappa immunoglobulin that contains murine light- and heavy-chain variable region sequences and human constant region sequences.

Questions

Baigrie D, Goyal A, Crane JS. Leukocytoclastic Vasculitis. 2022 Jan. [QxMD MEDLINE Link]. [Full Text].
Lie JT. Nomenclature and classification of vasculitis: plus ça change, plus c'est la même chose. Arthritis Rheum. 1994 Feb. 37(2):181-6. [QxMD MEDLINE Link].
Aounallah A, Arouss A, Ghariani N, Saidi W, Sriha B, Denguezli M, et al. [Cutaneous leukocytoclastic vasculitis: about 85 cases]. Pan Afr Med J. 2017. 26:138. [QxMD MEDLINE Link].
Ball-Burack MR, Kosowsky JM. A Case of Leukocytoclastic Vasculitis Following SARS-COV-2 Vaccination. J Emerg Med. 2021 Oct 23. [QxMD MEDLINE Link]. [Full Text].
Fiorillo G, Pancetti S, Cortese A, Toso F, Manara S, Costanzo A, et al. Leukocytoclastic vasculitis (cutaneous small-vessel vasculitis) after COVID-19 vaccination. J Autoimmun. 2022 Feb. 127:102783. [QxMD MEDLINE Link]. [Full Text].
Lotti T, Ghersetich I, Comacchi C, Jorizzo JL. Cutaneous small-vessel vasculitis. J Am Acad Dermatol. 1998 Nov. 39(5 Pt 1):667-87; quiz 688-90. [QxMD MEDLINE Link].
Tai YJ, Chong AH, Williams RA, Cumming S, Kelly RI. Retrospective analysis of adult patients with cutaneous leukocytoclastic vasculitis. Australas J Dermatol. 2006 May. 47(2):92-6. [QxMD MEDLINE Link].
Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997 Nov 20. 337(21):1512-23. [QxMD MEDLINE Link].
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 Nov. 92(6):331-43. [QxMD MEDLINE Link].
Johnson EF, Wetter DA, Lehman JS, Hand JL, Davis DM, Tollefson MM. Leukocytoclastic vasculitis in children: clinical characteristics, subtypes, causes and direct immunofluorescence findings of 56 biopsy-confirmed cases. J Eur Acad Dermatol Venereol. 2017 Mar. 31 (3):544-549. [QxMD MEDLINE Link].
Tancrede-Bohin E, Ochonisky S, Vignon-Pennamen MD, et al. Schonlein-Henoch purpura in adult patients. Predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases. Arch Dermatol. 1997 Apr. 133(4):438-42. [QxMD MEDLINE Link].
Zurada JM, Ward KM, Grossman ME. Henoch-Schönlein purpura associated with malignancy in adults. J Am Acad Dermatol. 2006 Nov. 55(5 Suppl):S65-70. [QxMD MEDLINE Link].
Mackel SE, Jordon RE. Leukocytoclastic vasculitis. A cutaneous expression of immune complex disease. Arch Dermatol. 1982 May. 118(5):296-301. [QxMD MEDLINE Link].
Sams WM Jr. Hypersensitivity angiitis. J Invest Dermatol. 1989 Aug. 93(2 Suppl):78S-81S. [QxMD MEDLINE Link].
Kevil CG, Bullard DC. Roles of leukocyte/endothelial cell adhesion molecules in the pathogenesis of vasculitis. Am J Med. 1999 Jun. 106(6):677-87. [QxMD MEDLINE Link].
Watanabe T. Vasculitis following influenza vaccination: A review of the literature. Curr Rheumatol Rev. 2017 May 17. [QxMD MEDLINE Link].
Marquez J, Aguirre L, Muñoz C, Echeverri A, Restrepo M, Pinto LF. Cocaine-Levamisole-Induced Vasculitis/Vasculopathy Syndrome. Curr Rheumatol Rep. 2017 Jun. 19 (6):36. [QxMD MEDLINE Link].
Kunzler D, Lagrimas L, Vo T, DeCrescenzo A, Kaltwasser K, Wilson J. Antineutrophil cytoplasmic antibodies negative levamisole-induced leukocytoclastic vasculitis: a presumed case and literature review. Int J Dermatol. 2018 Sep 25. [QxMD MEDLINE Link].
Jose SK, Marfatia YS. Erythema elevatum diutinum in acquired immune deficiency syndrome: Can it be an immune reconstitution inflammatory syndrome?. Indian J Sex Transm Dis. 2016 Jan-Jun. 37 (1):81-4. [QxMD MEDLINE Link].
Cacoub P, Poynard T, Ghillani P, et al. Extrahepatic manifestations of chronic hepatitis C. MULTIVIRC Group. Multidepartment Virus C. Arthritis Rheum. 1999 Oct. 42(10):2204-12. [QxMD MEDLINE Link].
Ford V, Mooney C, Shah M, Jenkins E. Leukocytoclastic Vasculitis as the Presenting Symptom of Crohn's Disease in an Adolescent. J Investig Med High Impact Case Rep. 2020 Jan-Dec. 8:2324709620947608. [QxMD MEDLINE Link]. [Full Text].
Sanchez-Guerrero J, Gutierrez-Urena S, Vidaller A, et al. Vasculitis as a paraneoplastic syndrome. Report of 11 cases and review of the literature. J Rheumatol. 1990 Nov. 17(11):1458-62. [QxMD MEDLINE Link].
Umemura H, Yamasaki O, Iwatsuki K. Leukocytoclastic vasculitis associated with immunoglobulin A lambda monoclonal gammopathy of undetermined significance: A case report and review of previously reported cases. J Dermatol. 2018 Aug. 45 (8):1009-1012. [QxMD MEDLINE Link].
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 Jan. 65(1):1-11. [QxMD MEDLINE Link].
Garcia-Porrua C, Gonzalez-Gay MA, Lopez-Lazaro L. Drug associated cutaneous vasculitis in adults in northwestern Spain. J Rheumatol. 1999 Sep. 26(9):1942-4. [QxMD MEDLINE Link].
Gonzalez-Gay MA, Garcia-Porrua C. Systemic vasculitis in adults in northwestern Spain, 1988-1997. Clinical and epidemiologic aspects. Medicine (Baltimore). 1999 Sep. 78(5):292-308. [QxMD MEDLINE Link].
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 Feb. 102(2):186-91. [QxMD MEDLINE Link].
Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, et al. Cutaneous vasculitis in children and adults. Associated diseases and etiologic factors in 303 patients. Medicine (Baltimore). 1998 Nov. 77(6):403-18. [QxMD MEDLINE Link].
Ekenstam Eaf, Callen JP. Cutaneous leukocytoclastic vasculitis. Clinical and laboratory features of 82 patients seen in private practice. Arch Dermatol. 1984 Apr. 120(4):484-9. [QxMD MEDLINE Link].
Amores-Martín E, Iglesias-Sancho M, Fernández-Figueras MT. Leukocytoclastic vasculitis manifested as a Koebner phenomenon. Reumatol Clin. 2020 Jul - Aug. 16 (4):308-309. [QxMD MEDLINE Link].
Piette WW, Stone MS. A cutaneous sign of IgA-associated small dermal vessel leukocytoclastic vasculitis in adults (Henoch-Schonlein purpura). Arch Dermatol. 1989 Jan. 125(1):53-6. [QxMD MEDLINE Link].
Garcia-Porrua C, Gonzalez-Gay MA. Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch-Schonlein purpura in adults. Semin Arthritis Rheum. 1999 Jun. 28(6):404-12. [QxMD MEDLINE Link].
Davis MD, Daoud MS, Kirby B, et al. Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis. J Am Acad Dermatol. 1998 Jun. 38(6 Pt 1):899-905. [QxMD MEDLINE Link].
Wisnieski JJ, Baer AN, Christensen J, et al. Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Medicine (Baltimore). 1995 Jan. 74(1):24-41. [QxMD MEDLINE Link].
Gibson LE, Su WP. Cutaneous vasculitis. Rheum Dis Clin North Am. 1995 Nov. 21(4):1097-113. [QxMD MEDLINE Link].
Gonzalez-Gay MA, Garcia-Porrua C, Salvarani C, et al. Cutaneous vasculitis: a diagnostic approach. Clin Exp Rheumatol. 2003 Nov-Dec. 21(6 Suppl 32):S85-8. [QxMD MEDLINE Link].
Kawakami T, Yamazaki M, Mizoguchi M, Soma Y. High titer of serum antiphospholipid antibody levels in adult Henoch-Schönlein purpura and cutaneous leukocytoclastic angiitis. Arthritis Rheum. 2008 Apr 15. 59(4):561-7. [QxMD MEDLINE Link].
Jara LJ, Navarro C, Medina G, Vera-Lastra O, Saavedra MA. Hypocomplementemic urticarial vasculitis syndrome. Curr Rheumatol Rep. 2009 Dec. 11(6):410-5. [QxMD MEDLINE Link].
de Hollanda A, Beucher A, Henrion D, Ghali A, Lavigne C, Lévesque H, et al. Systemic and immune manifestations in myelodysplasia: a multicenter retrospective study. Arthritis Care Res (Hoboken). 2011 Aug. 63(8):1188-94. [QxMD MEDLINE Link].
Solans-Laqué R, Bosch-Gil JA, Pérez-Bocanegra C, Selva-O'Callaghan A, Simeón-Aznar CP, Vilardell-Tarres M. Paraneoplastic vasculitis in patients with solid tumors: report of 15 cases. J Rheumatol. 2008 Feb. 35(2):294-304. [QxMD MEDLINE Link].
Chango Azanza JJ, Calle Sarmiento PM, Lopetegui Lia N, Alexander SA, Modi V. Leukocytoclastic Vasculitis: An Early Skin Biopsy Makes a Difference. Cureus. 2020 May 1. 12 (5):e7912. [QxMD MEDLINE Link]. [Full Text].
Loricera J, González-Vela C, Blanco R, Hernández JL, Armesto S, González-López MA, et al. Histopathologic differences between cutaneous vasculitis associated with severe bacterial infection and cutaneous vasculitis secondary to other causes: study of 52 patients. Clin Exp Rheumatol. 2016 May-Jun. 34 (3 Suppl 97):93-7. [QxMD MEDLINE Link].
Carlson JA, Cavaliere LF, Grant-Kels JM. Cutaneous vasculitis: diagnosis and management. Clin Dermatol. 2006 Sep-Oct. 24(5):414-29. [QxMD MEDLINE Link].
Russell JP, Weenig RH. Primary Cutaneous Small Vessel Vasculitis. Curr Treat Options Cardiovasc Med. 2004 Apr. 6(2):139-149. [QxMD MEDLINE Link].
Fiorentino DF. Cutaneous vasculitis. J Am Acad Dermatol. 2003 Mar. 48(3):311-40. [QxMD MEDLINE Link].
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 Dec. 131(12):1399-402. [QxMD MEDLINE Link].
Callen JP. Colchicine is effective in controlling chronic cutaneous leukocytoclastic vasculitis. J Am Acad Dermatol. 1985 Aug. 13(2 Pt 1):193-200. [QxMD MEDLINE Link].
Callen JP. Cutaneous vasculitis: Relationship to systemic disease and therapy. Curr Probl Dermatol. 1993. 5:45-80.
Fraticelli P, Benfaremo D, Gabrielli A. Diagnosis and management of leukocytoclastic vasculitis. Intern Emerg Med. 2021 Jun. 16 (4):831-841. [QxMD MEDLINE Link]. [Full Text].
Lunardi C, Bambara LM, Biasi D, et al. Elimination diet in the treatment of selected patients with hypersensitivity vasculitis. Clin Exp Rheumatol. 1992 Mar-Apr. 10(2):131-5. [QxMD MEDLINE Link].
Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010 Jul 15. 363(3):221-32. [QxMD MEDLINE Link].

Media Gallery

Hypersensitivity vasculitis.
Henoch-Schönlein purpura.
Histopathology of leukocytoclastic vasculitis.
Urticarial vasculitis. Lesions differ from routine urticaria (hives) in that they last longer (often >24 h), are less pruritic, and often resolve with a bruise or residual pigmentation.
Erythema elevatum diutinum, a rare cutaneous vasculitis.

of 5

Author

A Brooke W Eastham, MD Board Certified Dermatologist, Nashville Skin and Cancer

A Brooke W Eastham, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Medical Dermatology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Ruth Ann Vleugels, MD, MPH Assistant Professor of Dermatology, Harvard Medical School; Associate Physician, Department of Dermatology, Brigham and Women's Hospital; Associate Physician, Department of Immunology and Allergy, Children's Hospital Boston

Ruth Ann Vleugels, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Rheumatology, American Medical Association, Society for Investigative Dermatology, Medical Dermatology Society, Dermatology Foundation

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Stock ownership for: Johnson & Johnson.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Bryan L Martin, DO Associate Dean for Graduate Medical Education, Designated Institutional Official, Associate Medical Director, Director, Allergy Immunology Program, Professor of Medicine and Pediatrics, Ohio State University College of Medicine

Bryan L Martin, DO is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Osteopathic Internists, American College of Physicians, American Medical Association, American Osteopathic Association

Disclosure: Nothing to disclose.

Leukocytoclastic Vasculitis Medication

Medication Summary

Anti-inflammatory Agents

Class Summary

Colchicine

Colchicine

Antibiotics

Class Summary

Dapsone (Avlosulfon)

Dapsone (Avlosulfon)

Corticosteroids

Class Summary

Prednisone (Deltasone, Orasone, Meticorten)

Prednisone (Deltasone, Orasone, Meticorten)

Cytotoxic/Immunosuppressive Agents

Class Summary

Cyclophosphamide (Cytoxan, Neosar)

Cyclophosphamide (Cytoxan, Neosar)

Azathioprine (Imuran)

Azathioprine (Imuran)

Methotrexate (Rheumatrex, Folex PFS)

Methotrexate (Rheumatrex, Folex PFS)

Mycophenolate mofetil (CellCept)

Mycophenolate mofetil (CellCept)

Rituximab (Rituxan)

Rituximab (Rituxan)

Leukocytoclastic Vasculitis Medication

Medication Summary

Anti-inflammatory Agents

Class Summary

Colchicine

Antibiotics

Class Summary

Dapsone (Avlosulfon)

Corticosteroids

Class Summary

Prednisone (Deltasone, Orasone, Meticorten)

Cytotoxic/Immunosuppressive Agents

Class Summary

Cyclophosphamide (Cytoxan, Neosar)

Azathioprine (Imuran)

Methotrexate (Rheumatrex, Folex PFS)

Mycophenolate mofetil (CellCept)

Rituximab (Rituxan)

References

Tables

Contributor Information and Disclosures

What would you like to print?