Leukocytoclastic vasculitis

Published on 19/03/2015 by admin

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Last modified 19/03/2015

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Leukocytoclastic vasculitis

Nicole Fett and Jeffrey P. Callen

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

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Vasculitis is best classified based on the size of the vessels involved. Large vessel vasculitides, giant cell arteritis and Takayasu arteritis, involve large arteries and rarely have associated cutaneous findings. Pure medium vessel vasculitides such as polyarteritis nodosa, cutaneous polyarteritis nodosa and Kasawaki disease affect vessels with muscular walls. Cutaneous features of medium vessel vasculitides include livedo reticularis, retiform purpura, nodules, ulcers, and infarcts. Vasculitides that involve both medium and small vessels include granulomatosis with polyangiitis (previously Wegener granulomatosis), eosinophilic granulomatous angiitis (previously Churg–Strauss Syndrome), microscopic polyangiitis, and cryoglobulinemic vasculitis. Overlap medium and small vessel vasculitides may present with features of medium vessel involvement and features classic for small vessel involvement (urticaria, palpable purpura). Lastly, pure small vessel vasculitides (cutaneous small vessel vasculitis) include IgA vasculities (previously Henoch–Schönlein purpura (HSP)), erythema elevatum diutinum, urticarial vasculitis, and ‘leukocytoclastic vasculitis’ (LCV). LCV is a term that defines a histopathological pattern, not cutaneous findings. The majority of patients labeled with LCV have cutaneous small vessel vasculitis (CSVV); however, the most common cutaneous manifestation of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and connective tissue disease associated vasculitis is LCV and therefore careful evaluation for systemic involvement is necessary in these patients. CSVV is secondary to exposure to medications and viruses over 60% of the time and in general tends to be self-limited.

Management strategy

Management of small vessel vasculitis requires evaluation for systemic involvement, removal of potential causative agents, management of symptoms in those patients with self-limited disease, and targeted therapy for patients with recurrent or recalcitrant disease. Patients with systemic vasculitis require immediate referral to rheumatology, pulmonology, or nephrology and generally require treatment with systemic corticosteroids and systemic immunosuppressive agents.

Patients with CSVV in whom there is an identifiable cause, such as a drug, are treated symptomatically in addition to removing the presumed causative agent. Symptomatic measures include rest, elevation, gradient support stockings, and antihistamines.

The challenge is to treat the patient who has chronic CSVV without a defined etiology and without systemic involvement. In patients with asymptomatic disease who are not bothered by the appearance of their vasculitis, no treatment is necessary. For those patients who develop pain, ulcerations, or psychological distress, the risks and benefits of therapy should be discussed. Treatment recommendations for CSVV are largely based on case reports, case series, and expert opinion. If systemic therapy is considered for disease confined to the skin, colchicine and dapsone are first-line agents given their relative safety. Systemic corticosteroids, methotrexate, and azathioprine have been used in patients who are refractory to colchicine and dapsone.

Specific investigations

The purpose of evaluating the patient with cutaneous vasculitis is to identify a cause of the process and assess for the presence of systemic involvement. The evaluation begins with a careful history and physical examination, followed by selected testing based on the acuteness of the process and the findings from the history and physical examination. Direct immunofluorescence is required to differentiate IgA vasculitis from other causes of CSVV.

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