Overview of cutaneous small vessel vasculitis
- Carmen Gota, MD
Carmen Gota, MD
- Orthopedic and Rheumatologic Institute at Cleveland Clinic
- Center for Vasculitis Care and Research at Cleveland clinic
- Section Editors
- Eric L Matteson, MD, MPH
Eric L Matteson, MD, MPH
- Section Editor — Treatment Issues in Rheumatology
- Chair, Division of Rheumatology
- Professor of Medicine
- Mayo Clinic College of Medicine
- Jeffrey Callen, MD, FACP, FAAD
Jeffrey Callen, MD, FACP, FAAD
- Editor-in-Chief — Dermatology
- Section Editor — Skin and Systemic Disease
- Professor of Medicine
- University of Louisville School of Medicine
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 .
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 (IgA) vasculitis (Henöch-Schönlein purpura) 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 IgA vasculitis), 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:
- Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
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- 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.