Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Overview of cutaneous small vessel vasculitis

Carmen Gota, MD
Section Editors
Eric L Matteson, MD, MPH
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Monica Ramirez Curtis, MD, MPH


The nomenclature of cutaneous small vessel vasculitis (CSVV) is diverse and often confusing. Names often used interchangeably but not always accurately, have included hypersensitivity angiitis, drug-induced vasculitis, leukocytoclastic vasculitis, cutaneous leukocytoclastic angiitis, serum sickness, serum sickness-like reactions, and allergic vasculitis. (See 'Definitions' below.)

This topic will present an overview of CSVV as a single-organ vasculitis limited to the skin. The evaluation and management of adults with cutaneous lesions of vasculitis are discussed in detail separately (see "Evaluation of adults with cutaneous lesions of vasculitis" and "Management of adults with idiopathic cutaneous small vessel vasculitis"). An overview of the approach to the vasculitides is also presented elsewhere. (See "Overview of and approach to the vasculitides in adults".)


Cutaneous small vessel vasculitis – Cutaneous small vessel vasculitis (CSVV) is defined as a single-organ, skin-isolated leukocytoclastic vasculitis (LCV) or angiitis without systemic vasculitis or glomerulonephritis [1].

It is important to note that the same cutaneous presentation can be seen in many other systemic small vessel vasculitides such as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (eg, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis), cryoglobulinemic vasculitis, immunoglobulin A vasculitis (IgAV; Henoch-Schönlein purpura [HSP]) as well as other conditions such as infective endocarditis. When a patient presents with palpable purpura that reveals LCV on biopsy, the patient must be evaluated for a variety of causes and associated conditions (see "Evaluation of adults with cutaneous lesions of vasculitis" and "Overview of cryoglobulins and cryoglobulinemia"). Further delineation of etiology can only be done by integrating the clinical presentation, and in some cases (such as IgAV [HSP]), the presence or absence of vascular immunoglobulin deposition on skin biopsy. Some patients who are diagnosed initially as having a single organ cutaneous LCV can later develop a systemic form of small vessel vasculitis.

Other terms that have been used interchangeably with CSVV, but should be considered separately are defined below:

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Oct 19, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
  2. ZEEK PM. Periarteritis nodosa and other forms of necrotizing angiitis. N Engl J Med 1953; 248:764.
  3. Mullick FG, McAllister HA Jr, Wagner BM, Fenoglio JJ Jr. Drug related vasculitis. Clinicopathologic correlations in 30 patients. Hum Pathol 1979; 10:313.
  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.
  5. Sais G, Vidaller A, Jucglà A, et al. Colchicine in the treatment of cutaneous leukocytoclastic vasculitis. Results of a prospective, randomized controlled trial. Arch Dermatol 1995; 131:1399.
  6. Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M. Cutaneous vasculitis in children and adults. Associated diseases and etiologic factors in 303 patients. Medicine (Baltimore) 1998; 77:403.
  7. Tosca N, Stratigos JD. Possible pathogenetic mechanisms in allergic cutaneous vasculitis. Int J Dermatol 1988; 27:291.
  8. van Rossum AP, Pas HH, Fazzini F, et al. Abundance of the long pentraxin PTX3 at sites of leukocytoclastic lesions in patients with small-vessel vasculitis. Arthritis Rheum 2006; 54:986.
  9. Leavitt RY, Fauci AS. Polyangiitis overlap syndrome. Classification and prospective clinical experience. Am J Med 1986; 81:79.
  10. Parker CW. Allergic reactions in man. Pharmacol Rev 1982; 34:85.
  11. Loricera J, Blanco R, Ortiz-Sanjuán 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 cases. Rheumatology (Oxford) 2015; 54:77.