Livedoid Vasculopathy Medication

Updated: Apr 23, 2020
  • Author: Fnu Nutan, MD, FACP; Chief Editor: William D James, MD  more...
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Medication

Medication Summary

Drugs stimulating endogenous fibrinolytic activity and drugs inhibiting thrombus formation (antiplatelet and anticoagulant) are possible treatments of livedoid vasculopathy.

Severe livedoid vasculopathy related to antibodies involving the antiphosphatidylserine-prothrombin complex has been successfully treated with warfarin. [61]

Treatment of livedoid vasculopathy using alprostadil (PGE-1) seems like a possible treatment option for livedoid vasculopathy. [62]

Low molecular weight heparin has been used to treat livedoid vasculopathy in a patient with a positive test for lupus anticoagulant and the presence of the MTHFR mutation. [63]

A newer drug, rivaroxaban, which has undergone phase II trials for livedoid vasculopathy, is undergoing development. One of the trials was in Germany, where 25 patients were treated with rivaroxaban and 6 patients required alternative treatment. [64] Rivaroxaban exerts effects on the coagulation cascade. Rivaroxaban inhibits the factor Xa–dependent transformation of prothrombin to thrombin. Thus, rivaroxaban substantially reduces the risk of thrombosis. [65, 66] In 2019, Marques at al noted that rivaroxaban has resulted in successful treatments in four cases tested in Brazil. [67] A study in Korea published in 2019 with 40 patients showed effective treatment results with 10 mg daily of rivaroxaban. [68]

Intravenous immunoglobulin (IVIG) holds promise of treating livedoid vasculopathy and was effective at 2 g/kg of IVIG every 4 weeks in a trial of 11 patients in ameliorating livedoid vasculopathy, with long-term follow up. [69] Improvement occurred in 59% of patients after 3 cycles and 86% after 6 cycles. After 7 and 8 cycles in previous treatment failure, a 93% success rate was achieved. After two IVIG cycles, subscore analysis demonstrated resolution of pain in 80% of patients. Quality of life and disease severity and were very much improved after 6 cycles. The median duration of remissions appeared to be 26.7 months after the initial treatment and 7.5 months in subsequent disease episodes. [69]

Treatment of sensory ganglionopathy with livedoid vasculopathy controlled by immunotherapy failed treatment with prednisolone and mycophenolate mofetil but succeeded when rituximab was added, in terms of nerve conduction stabilization and symptoms. [70]

Anabolic steroids including danazol, betamethasone sodium and methylprednisolone have been used as therapy for livedoid vasculopathy. Criado et al. tested 4 patients prescribed 200mg/daily of danazol, which has fibrinolytic action. This treatment led to healing of the ulcers and the level of LP(a) was reduced by 70%. [71]

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Cardiovascular agents

Class Summary

These agents are the drugs of choice. They may be useful in reducing pain and ulceration.

Pentoxifylline (Trental)

Pentoxifylline may alter the rheology of red blood cells, which, in turn, reduces blood viscosity.

Dipyridamole (Persantine, Aggrenox)

Dipyridamole is a platelet adhesion inhibitor that possibly inhibits red blood cell uptake of adenosine, which is an inhibitor of platelet reactivity. In addition, it may inhibit phosphodiesterase activity, leading to increased cyclic-3',5'-adenosine monophosphate within platelets and formation of the potent platelet activator thromboxane A2. Dipyridamole is a vasodilator. Use with aspirin.

Aspirin (Bayer, Ascriptin, Empirin)

Aspirin inhibits prostaglandin synthesis, preventing the formation of platelet-aggregating thromboxane A2. Use in a low dose to inhibit platelet aggregation and to improve complications of venous stases and thrombosis.

Nifedipine (Procardia)

Nifedipine relaxes coronary smooth muscle and produces coronary vasodilation, which, in turn, improves myocardial oxygen delivery. Nifedipine causes vasodilation.

Enoxaparin (Lovenox)

Enoxaparin prevents deep vein thrombosis, which may lead to pulmonary embolism in patients undergoing surgery who are at risk for thromboembolic complications. Enoxaparin enhances the inhibition of factor Xa and thrombin by increasing antithrombin III activity. In addition, it preferentially increases the inhibition of factor Xa. The average duration of treatment is 7-14 days.

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Factor Xa Inhibitors

Class Summary

Rivaroxaban is a direct factor Xa inhibitor and prevents clot formation.

Rivaroxaban (Xarelto)

Rivaroxaban is a factor Xa inhibitor that inhibits platelet activation by selectively blocking the active site of factor Xa without requiring a cofactor (eg, antithrombin III) for activity.

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