Update on Cutaneous Small Vessel Vasculitis: Terminology, Morphology, Diagnostic Evaluation, and Management

Fraticelli P, Benfaremo D, Gabrielli A. Diagnosis and management of leukocytoclastic vasculitis. Intern Emerg Med. 2021;16(4):831–41.

Article  PubMed  PubMed Central  Google Scholar 

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

Article  PubMed  Google Scholar 

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

Article  CAS  PubMed  Google Scholar 

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

Article  CAS  PubMed  Google Scholar 

Caproni M, Verdelli A. An update on the nomenclature for cutaneous vasculitis. Curr Opin Rheumatol. 2019;31(1):46–52.

Article  PubMed  Google Scholar 

•• Sunderkötter CH, Zelger B, Chen KR, et al. Nomenclature of cutaneous vasculitis: dermatologic addendum to the 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheumatol. 2018;70(2):171–84. 2018 expert panel dermatologic addendum with updated classification and nomenclature of cutaneous small vessel vasculitis subtypes. Delineates systemic vs skin-limited classifications and clinical presentations of each.

Goeser MR, Laniosz V, Wetter DA. A practical approach to the diagnosis, evaluation, and management of cutaneous small-vessel vasculitis. Am J Clin Dermatol. 2014;15(4):299–306.

Article  PubMed  Google Scholar 

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

Article  PubMed  Google Scholar 

• Gehlhausen JR, Wetter DA, Nelson C, Ramachandran S, McNiff JM, Ko CJ. A detailed analysis of the distribution, morphology, and histopathology of complex purpura in hospitalized patients: a case series of 68 patients. J Am Acad Dermatol. 2021;84(4):1188–96. Analyzes the presentation and etiologies of purpura when presenting with branching vs. nonbranching cutaneous morphology.

Piram M, Mahr A. Epidemiology of immunoglobulin A vasculitis (Henoch-Schönlein): current state of knowledge. Curr Opin Rheumatol. 2013;25(2):171–8.

Article  CAS  PubMed  Google Scholar 

Podjasek JO, Wetter DA, Pittelkow MR, Wada DA. Henoch-Schönlein purpura associated with solid-organ malignancies: three case reports and a literature review. Acta Derm Venereol. 2012;92(4):388–92.

Article  PubMed  Google Scholar 

• Micheletti RG, Chiesa Fuxench Z, Craven A, Watts RA, Luqmani RA, Merkel PA. Cutaneous manifestations of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2020;72(10):1741–7. Details cutaneous and other clinical manifestations of ANCA vasculitides. Helps to understand in which clinical scenarios ANCAs would need to be ordered for patients presenting with small vessel vasculitis.

Wiesner O, Russell KA, Lee AS, Jenne DE, Trimarchi M, Gregorini G, et al. Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaine-induced midline destructive lesions but not autoimmune vasculitis. Arthritis Rheum. 2004;50:2954–65.

Article  CAS  PubMed  Google Scholar 

McGrath MM, Isakova T, Rennke HG, Mottola AM, Laliberte KA, Niles JL. Contaminated cocaine and antineutrophil cytoplasmic antibody-associated disease. Clin J Am Soc Nephrol. 2011;6(12):2799–805.

Article  PubMed  PubMed Central  Google Scholar 

Marzano AV, Raimondo MG, Berti E, Meroni PL, Ingegnoli F. Cutaneous manifestations of ANCA-associated small vessels vasculitis. Clin Rev Allergy Immunol. 2017;53(3):428–38.

Article  PubMed  Google Scholar 

Audemard-Verger A, Terrier B, Dechartres A, et al. Characteristics and management of IgA vasculitis (Henoch-Schönlein) in adults: data from 260 patients included in a French Multicenter Retrospective Survey. Arthritis Rheumatol. 2017;69(9):1862–70.

Article  CAS  PubMed  Google Scholar 

Jachiet M, Flageul B, Bouaziz JD, Bagot M, Terrier B. Hypocomplementemic urticarial vasculitis. Rev Med Interne. 2018;39(2):90–8.

Article  CAS  PubMed  Google Scholar 

Koch M, Khan Z, Karle EM, Patel TP. Immune complex vasculitis: a rash that cannot be missed. BMJ Case Rep. 2020;13(2).

Makol A, Matteson EL, Warrington KJ. Rheumatoid vasculitis: an update. Curr Opin Rheumatol. 2015;27(1):63–70.

Article  CAS  PubMed  Google Scholar 

Chango Azanza JJ, Calle Sarmiento PM, Lopetegui Lia N, Alexander SA, Modi V. Leukocytoclastic vasculitis: an early skin biopsy makes a difference. Cureus. 2020;12(5): e7912.

PubMed  PubMed Central  Google Scholar 

• Walls AC, Din RS, Okhovat JP, Chansky PB, Micheletti RG, Mostaghimi A. J Am Acad Dermatol. 2021;84(4):1197–9. Large retrospective review demonstrating low utility of utilizing radiological studies to determine etiology of small vessel vasculitis.

Bouiller K, Audia S, Devilliers H, et al. Etiologies and prognostic factors of leukocytoclastic vasculitis with skin involvement: a retrospective study in 112 patients. Medicine (Baltimore). 2016;95(28): e4238.

Article  CAS  PubMed  Google Scholar 

Sunderkötter C, Bonsmann G, Sindrilaru A, Luger T. Management of leukocytoclastic vasculitis. J Dermatolog Treat. 2005;16(4):193–206.

Article  PubMed  Google Scholar 

Sais G, Vidaller A, Jucglà A, Gallardo F, Peyrí J. Colchicine in the treatment of cutaneous leukocytoclastic vasculitis. Results of a prospective, randomized controlled trial. Arch Dermatol. 1995;131(12):1399–1402.

Fredenberg MF, Malkinson FD. Sulfone therapy in the treatment of leukocytoclastic vasculitis. Report of three cases. J Am Acad Dermatol. 1987;16(4):772–778.

Jachiet M, Flageul B, Deroux A, et al. The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients. Arthritis Rheumatol. 2015;67(2):527–34.

Article  CAS  PubMed  Google Scholar 

Callen JP, Spencer LV, Burruss JB, Holtman J. Azathioprine. An effective, corticosteroid-sparing therapy for patients with recalcitrant cutaneous lupus erythematosus or with recalcitrant cutaneous leukocytoclastic vasculitis. Arch Dermatol. 1991;127(4):515–522.

Jorizzo JL, White WL, Wise CM, Zanolli MD, Sherertz EF. Low-dose weekly methotrexate for unusual neutrophilic vascular reactions: cutaneous polyarteritis nodosa and Behçet’s disease. J Am Acad Dermatol. 1991;24(6 Pt 1):973–8.

Article  CAS  PubMed  Google Scholar 

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

Article  PubMed  Google Scholar 

Maritati F, Fenoglio R, Pillebout E, et al. Brief report: rituximab for the treatment of adult-onset IgA vasculitis (Henoch-Schönlein). Arthritis Rheumatol. 2018;70(1):109–14.

Article  CAS  PubMed  Google Scholar 

De Vita S, Quartuccio L, Isola M, et al. A randomized controlled trial of rituximab for the treatment of severe cryoglobulinemic vasculitis. Arthritis Rheum. 2012;64(3):843–53.

Article  PubMed  Google Scholar 

McClure M, Gopaluni S, Jayne D, Jones R. B cell therapy in ANCA-associated vasculitis: current and emerging treatment options. Nat Rev Rheumatol. 2018;14(10):580–91.

Article  PubMed  Google Scholar 

Comments (0)

No login
gif