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Cutaneous vasculitis refers to a number of syndromes. Leukocytoclastic vasculitis is a specific histopathologic entity. This pattern is observed in many of the vasculitic syndromes that affect the small vessels of the skin. Circulating immune complexes are involved in the pathogenesis of many of the vasculitic syndromes. Syndromes that can involve the small cutaneous vessels include hypersensitivity vasculitis, Henoch-Schonlein purpura, vasculitis associated with paraproteinemias, vasculitis as part of a collagen-vascular disorder, and hypocomplementemic (or urticarial) vasculitis. In addition, many patients with Wegener's Granulomatosis, microscopic polyarteritis, and other vasculitides.
Evaluation of the patient with cutaneous vasculitis is useful in determining possible etiologic factors or associated processes, in assessing for the presence of systemic disease, and in formulating a prognosis. Historical information can reveal the presence of a pre-existing disorder or disease, medications taken prior to the onset of the vasculitis, the presence of infection.
On physical examination, both the type of cutaneous lesion and the extent of disease are of prognostic importance. Purpura, livedo reticularis, subcutaneous nodules, and ulcerations may be manifestations of noninflammatory vascular compromise such as in hypercoagulable states, scurvy, left atrial myxoma, atheromatous emboli, or calciphylaxis; these conditions should be ruled out. Immunofluorescence microscopy is helpful when the possibility of Henoch-Schonlein purpura is considered.
Tissue confirmation of vasculitis is almost always necessary, and biopsy should be performed.
Perinuclear ANCA (p-ANCA) may be present in other vasculitic syndromes such as polyarteritis nodosa and microscopic polyarteritis, but may also be present in nonvasculitic states such as Sweet's syndrome, pyoderma gangrenosum.
If an associated condition or etiologic factor is present, its removal or therapy can result in a cure of the process. Identification and treatment of an infection, discontinuation of offending medicaments or other ingestants, and therapy directed against the production of an abnormal protein.
An open-label trial in Italy studied the effect of an elimination diet on five patients. With reintroduction of foods and food dyes, the offending agent was identified and sustained control of the disease.
Dietary restriction has been of limited value in my practice. Because lesions are more frequent or more severe in the skin overlying dependent areas and on cooler acral regions of the body, frequent turning, compression stockings, elevation, and a warm environment are helpful.
Hypersensitivity vasculitis and Henoch-Schonlein purpura are often self-limited, and only symptomatic therapy may be required. Polyarteritis nodosa, Wegener's granulomatosis, and systemic necrotizing vasculitis are potentially life-threatening conditions, and without treatment, patients may die from renal or central nervous system involvement. Thus, aggressive therapy is often necessary. Urticarial vasculitis may be a chronic condition with a benign course or, in the presence of hypocomplementemia, may be complicated by chronic obstructive pulmonary disease. The chronic nature of the process necessitates continual suppressive therapy. Manifestations of vasculitis complicating rheumatoid arthritis, systemic lupus erythematosus (SLE), or Sjogren's syndrome range from benign palpable purpura to severe
Administration of antihistamines has often been suggested as a first line of therapy, based on the observation that injected histamine allows deposition of immune complexes in the vessel walls with the eventual development of leukocytoclastic vasculitis. In patients with palpable purpura, antihistamines have rarely if ever been effective in my practice. However, in patients with urticarial vasculitis, I generally begin therapy with a
Various nonsteroidal anti-inflammatory drugs (NSAIDs) have been used in the treatment of vasculitic syndromes. In particular, patients with urticarial vasculitis may respond to
Pentoxifylline (Trental) has been reported to be useful in some patients. In particular, those patients with
Antimalarials, such as hydroxychloroquine (Plaquenil) and chloroquine (Aralen), have been used for
Diaminodiphenylsulfone (DDS), i.e., dapsone, has received some recent attention. It is effective in dermatitis herpetiformis, a disease characterized by neutrophilic papillitis, and has various effects on
Colchicine has been reported to be effective in open-label trials for cutaneous vasculitis as manifested by palpable purpura or urticarial lesions. Colchicine is an alkaloid derived from a crocus-like plant, Colchicum autumnale. Its effects in vasculitis may be related to a blockade of disease expression.
Systemic corticosteroids are useful in most patients with vasculitis, but because of multiple potential toxic effects, their use should be limited to patients with severe disease or cases in which the use is
Patients who fail to respond to systemic corticosteroids, who develop steroid-related side effects, or in whom the disease is severe may be given an immunosuppressive agent. The agents most often used in vasculitis are alkylating agents such as cyclophosphamide (Cytoxan) and chlorambucil (Leukeran), antimetabolites such as azathioprine (Imuran), the folate antagonist methotrexate (Rheumatrex), and cyclosporine (Sandimmune, Neoral). Earlier studies suggested that
Azathioprine has been reported to be useful in patients with severe refractory cutaneous vasculitis and in patients with rheumatoid vasculitis. It has also been used in severe necrotizing vasculitis, polyarteritis nodosa, and Wegener's granulomatosis but is probably less effective than cyclophosphamide in these conditions. It is administered in a single oral dose of 1 to 2 mg per kg. The primary toxic effects that occur are drug-induced fever, pancreatitis, hepatitis, and bone marrow toxicity. The onset of its action is
Low-dose weekly methotrexate (7.5 to 15 mg) has been used to treat patients with Wegener's granulomatosis, rheumatoid vasculitis, and cutaneous polyarteritis nodosa. Methotrexate can be
Cyclosporine is a relatively new agent developed to prevent transplant rejection. The mechanism of its action is unknown. It is administered in doses of 3 to 8 mg per kg per day, with effects and
Plasma exchange can be an adjunct to therapy for severe diseases characterized by circulating immunoreactants, such as vasculitis. A number of exchanges are required, and the procedure must be