In patients with livedoid vasculopathy (LV) and confirmed antiphospholipid antibodies, oral anticoagulation is the initial treatment approach. When that therapy fails to maintain the therapeutic INR target, a further treatment step is indicated. This protocol addresses exactly that clinical scenario.
This protocol is for patients with livedoid vasculopathy in whom antiphospholipid antibodies are present (antiphospholipid syndrome). This antibody status directly shapes therapy selection — not all anticoagulant agents are appropriate in this population.
The prior line was oral anticoagulation with phenprocoumon (e.g. Marcumar) or warfarin (e.g. Coumadin), targeting an INR of 2.5–3.5. This protocol is indicated when the INR has not been maintained within that therapeutic range.
For treatment-refractory livedoid vasculopathy in this setting, the protocol calls for intravenous immunoglobulin (IVIG) therapy. The primary clinical goal is significant pain relief. The complete regimen — including dosing, schedule, and clinical considerations — is in the structured protocol below.
DOI: 10.1111/ddg.14520
If antiphospholipid antibodies are present, DOAC should not be used since treatment with rivaroxaban in patients with antipospholipid syndrome has been shown to increase the risk of recurring thrombotic events.
IVIG treatment is recommended in treatment-refractory LV (off label).
The authors recommend a dose of 2 g/kg BW which may be split into several doses and administered over a period of 2–5 days (off label).
Monshi et al. reported significant pain relief and an increase in DLQI after only six treatment cycles.
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