A Practical Approach to the Diagnosis, Evaluation, and Management of Cutaneous Small-vessel Vasculitis

Megan R. Goeser; Valerie Laniosz; David A. Wetter


Am J Clin Dermatol. 2014;15(4):299-306. 

In This Article

Abstract and Introduction


Cutaneous small-vessel vasculitis (CSVV) is a disorder characterized by neutrophilic inflammation predominantly limited to the superficial cutaneous postcapillary venules. CSVV may be idiopathic or may have a defined cause such as infection, medication, connective tissue disease, or malignancy. CSVV may also be associated with extracutaneous disease or systemic vasculitis. The most common clinical presentation of CSVV consists of symmetrically distributed palpable purpura of the lower extremities. In general, lesional skin biopsy samples should be examined with light microscopy and direct immunofluorescence for adult patients with suspected CSVV. A complete history, review of systems, physical examination, and selected laboratory studies also should be performed to assess for inciting causes or extracutaneous involvement of CSVV. Treatment varies and depends on the chronicity of CSVV, the severity of cutaneous involvement, and the presence or absence of both an underlying cause and extracutaneous involvement of CSVV. An isolated episode of CSVV associated with a known inciting factor may be managed by removal or treatment of the trigger, along with symptomatic measures. First-line systemic treatments for chronic, idiopathic CSVV include colchicine or dapsone, used singly or in combination. Recurrent, chronic, or severely symptomatic CSVV that does not respond to the aforementioned therapies may require initiation of an immunosuppressive agent such as azathioprine, mycophenolate mofetil, methotrexate, cyclosporine, or rituximab.

1 Introduction

Classification of vasculitis remains challenging. Two commonly used schemes are the American College of Rheumatology (ACR) classification criteria[1] and the Chapel Hill Consensus Conference (CHCC) nomenclature system.[2] The ACR and CHCC systems were intended as clinical research tools and a nomenclature system, respectively, rather than as diagnostic criteria for clinicians.Amore useful classification scheme for the practicing dermatologist is one based on vessel-size predominance.[2,3]

Numerous terms have been used in the literature for vasculitides predominantly affecting the skin. The CHCC revised 2012 nomenclature system refers to this entity as cutaneous leukocytoclastic angiitis,[2] whereas the ACR criteria of 1990 refer to this entity as hypersensitivity vasculitis.[1] Our preferred terminology is cutaneous small-vessel vasculitis (CSVV).[3,4]

CSVV is primarily limited to the small blood vessels of the skin but may be associated with larger systemic vasculitis or extracutaneous involvement.[5,6] Histologically, CSVV is characterized by perivascular neutrophilic inflammation of the postcapillary venules, fibrinoid destruction of the vessel walls, swelling of endothelial cells, and extravasation of red blood cells (i.e., leukocytoclastic vasculitis).[4] When vasculitis is idiopathic (i.e., no evidence of infection, medication, or other identifiable cause), it is termed primary CSVV. Although idiopathic cases of CSVV do occur, they may be associated with drug hypersensitivities, systemic inflammatory conditions, connective tissue diseases, infections, and malignancies.[4–8] Various CSVV subtypes include immunoglobulin (Ig) A vasculitis (Henoch-Schönlein purpura [HSP]),[2] urticarial vasculitis, and cryoglobulinemic vasculitis. The purpose of this overview is to provide a practical guide to diagnosing, evaluating, and managing CSVV.