Abstract
Cutaneous vasculitis may be a cutaneous manifestation of systemic vasculitis, a skin-limited variant of systemic vasculitis, or a type of single-organ vasculitis limited to the skin. Careful evaluation is necessary to distinguish these possibilities, differentiate skin-limited from systemic disease, and identify important underlying conditions, if present. Appropriate management depends on disease type and severity. In the case of cutaneous small vessel vasculitis (CSVV), treatment is primarily based on case reports and expert opinion, using a range of immunomodulatory or immunosuppressive therapies. This manuscript presents a systematic and stepwise approach to the evaluation of patients presenting with cutaneous vasculitis, as well as the management of those diagnosed with CSVV, using best available evidence. Studies relevant to the classification, evaluation, and management of skin vasculitis are ongoing.
Similar content being viewed by others
References
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;70:171–84.
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11.
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.
Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, et al., DCVAS Study Group. 2022 American College of Rheumatology/European Alliance of Associations for rheumatology classification criteria for granulomatosis with polyangiitis. Arthritis Rheumatol. 2022;74(3):393–9.
Xu LY, Esparza EM, Anadkat MJ, et al. Cutaneous manifestations of vasculitis. Semin Arthritis Rheum. 2009;38(5):348–60.
Micheletti RG, Chiesa Fuxench Z, Craven A, et al. Cutaneous manifestations of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2020;72:1741–7.
Mann S, Hussain A, Dua A, et al. Quality-of-life impact of cutaneous vasculitis: findings from the VascSkin study and vasculitis patient-powered research network [abstract]. In: 20th international vasculitis and ANCA workshop; Dublin; 2022.
Carlson JA. The histological assessment of cutaneous vasculitis. Histopathology. 2010;56(1):3.
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.
Linskey KR, Kroshinsky D, Mihm MC Jr, Hoang MP. Immunoglobulin A-associated small-vessel vasculitis: a 10-year experience at the Massachusetts General Hospital. J Am Acad Dermatol. 2012;66:813–22.
Micheletti RG, Werth VP. Small vessel vasculitis of the skin. Rheum Dis Clin N Am. 2015;41(1):21–32 (vii).
Micheletti RG. Cutaneous vasculitis in rheumatologic disease: current concepts of skin and systemic manifestations. Clin Dermatol. 2018;36(4):561–6.
Loricera J, Calvo-Rio V, Ortiz-Sanjuan F, et al. The spectrum of paraneoplastic cutaneous vasculitis in a defined population: incidence and clinical features. Medicine (Baltimore). 2013;92(6):331–43.
Loricera J, Blanco R, Ortiz-Sanjuan 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 case. Rheumatology (Oxford). 2015;54(1):77–82.
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;102:186–91.
Carlson JA, Chen KR. Cutaneous vasculitis update: small vessel neutrophilic vasculitis syndromes. Am J Dermatopathol. 2006;28:486–506.
Sais G, Vidaller A, Jucgla A, et al. Prognostic factors in leukocytoclastic vasculitis: a clinicopathologic study of 160 patients. Arch Dermatol. 1998;134:309–15.
Walls AC, Din RS, Okhovat JP, et al. Retrospective analysis of symptomatology, comorbidities and diagnostic evaluation of adults presenting with initial episode of leukocytoclastic vasculitis [abstract]. Medical Dermatology Society Annual Meeting; Washington, DC; 2016.
Walls AC, Din RS, Okhovat JP, Chansky PB, Micheletti RG, Mostaghimi A. Low utility of radiologic imaging in evaluating cutaneous small-vessel vasculitis: a multi-institutional retrospective study. J Am Acad Dermatol. 2021;84(4):1197–9.
Gonzalez-Gay MA, Garcia-Porrua C, Salvarani C, Lo Scocco G, Pujol RM. Cutaneous vasculitis: a diagnostic approach. Clin Exp Rheumatol. 2003;21(6 Suppl 32):S85–8.
Russell JP, Gibson LE. Primary cutaneous small vessel vasculitis: approach to diagnosis and treatment. Int J Dermatol. 2006;45(1):3–13.
Micheletti RG, Pagnoux C. Management of cutaneous vasculitis. Presse Med. 2020;49(3): 104033.
Callen JP. Colchicine is effective in controlling chronic cutaneous leukocytoclastic vasculitis. J Am Acad Dermatol. 1985;13(2 Pt 1):193–200.
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.
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.
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):515–22.
Tamura RN, Krischer JP, Pagnoux C, Micheletti R, et al. A small n sequential multiple assignment randomized trial design for use in rare disease research. Contemp Clin Trials. 2016;46:48–51.
Haeberle MT, Adams WB, Callen JP. Treatment of severe cutaneous small-vessel vasculitis with mycophenolate mofetil. Arch Dermatol. 2012;148(8):887–8.
Lopez LR, Davis KC, Kohler PF, Schocket AL. The hypocomplementemic urticarial-vasculitis syndrome: therapeutic response to hydroxychloroquine. J Allergy Clin Immunol. 1984;73:600–3.
Nurnberg W, Grabbe J, Czametzki BM. Synergistic effects of pentoxifylline and dapsone in leucocytoclastic vasculitis. Lancet. 1994;343(8895):491.
Chung L, Funke AA, Chakravarty EF, et al. Successful use of rituximab for cutaneous vasculitis. Arch Dermatol. 2006;142(11):1407–10.
Mang R, Ruzicka T, Stege H. Therapy for severe necrotizing vasculitis with infliximab. J Am Acad Dermatol. 2004;51(2):321–2.
Ong CS, Benson EM. Successful treatment of chronic leucocytoclastic vasculitis and persistent ulceration with intravenous immunoglobulin. Br J Dermatol. 2000;143(2):447.
Tosca A, Ioannidou DJ, Katsantonis JC, Kyriakis KP. Cyclosporin A in the treatment of cutaneous vasculitis. Clinical and cellular effects. J Eur Acad Dermatol Venereol. 1996;6:135–41.
Caplan A, Fett N, Rosenbach M, Werth VP, Micheletti RG. Prevention and management of glucocorticoid-induced side effects: a comprehensive review: a review of glucocorticoid pharmacology and bone health. J Am Acad Dermatol. 2017;76(1):1–9.
Caplan A, Fett N, Rosenbach M, Werth VP, Micheletti RG. Prevention and management of glucocorticoid-induced side effects: a comprehensive review: gastrointestinal and endocrinologic side effects. J Am Acad Dermatol. 2017;76(1):11–6.
Caplan A, Fett N, Rosenbach M, Werth VP, Micheletti RG. Prevention and management of glucocorticoid-induced side effects: a comprehensive review: infectious complications and vaccination recommendations. J Am Acad Dermatol. 2017;76(2):191–8.
Caplan A, Fett N, Rosenbach M, Werth VP, Micheletti RG. Prevention and management of glucocorticoid-induced side effects: a comprehensive review: ocular, cardiovascular, muscular, and psychiatric side effects and issues unique to pediatric patients. J Am Acad Dermatol. 2017;76(2):201–7.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Funding
No sources of funding were used to assist in the preparation of this article.
Conflict of interest
Robert G. Micheletti has no conflicts of interest to declare.
Ethics approval
Not applicable.
Consent to participate/publish
Not applicable.
Availability of data and material
Not applicable.
Code availability
Not applicable.
Author contributions
Acquisition of data, composition of the manuscript.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Micheletti, R.G. Cutaneous Small Vessel Vasculitis: A Practical Guide to Diagnosis and Management. Am J Clin Dermatol 24, 89–95 (2023). https://doi.org/10.1007/s40257-022-00736-6
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s40257-022-00736-6