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Management of idiopathic cutaneous small vessel vasculitis in adults

Management of idiopathic cutaneous small vessel vasculitis in adults
Idiopathic CSVV is defined as CSVV without an identifiable cause. Vasculitis in other organ systems must be absent, and direct immunofluorescence should not reveal IgA predominance. Deferring systemic treatment is an alternative for asymptomatic patients with uncomplicated, chronic (duration >4 weeks), or recurrent idiopathic CSVV who are not bothered by the skin lesions. Patients who defer therapy should receive periodic clinical follow-up.
CSVV: cutaneous small vessel vasculitis; NSAID: nonsteroidal anti-inflammatory drug; IBW: ideal body weight; IgA: immunoglobulin A; G6PD: glucose-6-phosphate dehydrogenase.
* A typical initial prednisone dose is between 20 and 40 mg per day.
¶ Rest, leg elevation, and compression stockings.
Δ Caution is indicated for colchicine use in patients with hepatic or renal insufficiency or who are taking certain drugs. Refer to Lexicomp drug information included in UpToDate for details.
◊ Dapsone therapy should be avoided in patients with G6PD deficiency. Testing for G6PD deficiency and other baseline laboratory tests are indicated prior to treatment. Refer to UpToDate content on the management of adults with idiopathic CSVV for additional details.
§ Refer to UpToDate content on the management of adults with idiopathic CSVV for details. We generally reserve a trial of pentoxifylline for select patients with relatively mild, uncomplicated CSVV and proceed to immunosuppressive therapy when this is insufficient.
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