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  • Cutaneous vasculitis can be a presenting feature of a systemic disease affecting other organ systems. Thus, all patients with cutaneous vasculitis need to be evaluated for possible systemic vasculitis including all forms of small- and medium-vessel vasculitis.

  • Additional testing for vasculitis is guided by findings from medical history and physical examination, but should usually include routine laboratory testing of renal function, evaluating for hepatitis B and C viral infections, serologic testing for ANA and ANCA, selected radiographic imaging, and other tests as indicated by presentation.

  • Skin biopsy is nearly always essential to establish a diagnosis of vasculitis and biopsies can help determine the type of vasculitis.

  • Mimics of idiopathic vasculitis need to be considered, especially including infection, malignancy, thrombosis, or embolic disease.

  • Drug-induced vasculitis frequently presents with skin lesions. However, all patients with suspected drug-induced disease should still be evaluated for other possible causes or types of vasculitis.

  • Treatment of vasculitis that involves the skin is guided by the disease and the organ systems involved. For the systemic necrotizing vasculitides, glucocorticoids are always used, initially at high doses for severe disease. Additional immunosuppressive medications depend on the type of vasculitis, extent of disease, and comorbidities of the patient.

  • Treatment of suspected drug-induced vasculitis may include discontinuing the suspected etiologic agent and careful observation but could also involve treatment with glucocorticoids or other drugs.

  • Use of immunosuppressive medications should be overseen by physicians experienced with the use of this class of drugs.

  • Comprehensive clinical followup of all patients with vasculitis is an essential aspect of management.


The term vasculitis can be defined broadly to mean inflammation of blood vessels. Although vasculitis sometimes affects a single organ, particularly the skin, to most clinicians vasculitis connotes a group of diseases in which inflammation of the blood vessels is the major, but not the only, pathologic process. The systemic vasculitides are a wide-ranging set of diseases that are mostly idiopathic, rare, and multisystemic. These diseases involve such variety of clinical presentations and pathologies that all clinicians in every medical and surgical specialty will encounter such patients.

Vasculitis of the skin is fairly common, and skin disease is a frequent manifestation in many forms of vasculitis, especially small- and medium-vessel arteritis where skin lesions may be the presenting symptom of a systemic illness.

This chapter focuses on skin disease in the systemic vasculitides. Isolated forms of skin vasculitis and some of the systemic vasculitides are covered in Chap. 138. In addition to outlining the skin manifestations of vasculitis in general and for specific types of vasculitis, this chapter will provide an approach to patients with skin disease in which vasculitis is a diagnostic consideration.

Multiple systems for classifying vasculitis exist, a situation that reflects a lack of clear understanding of the underlying pathophysiology and the overlap of clinical features among many types of vasculitis.1-6 The ...

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