Livedoid Vasculopathy Treatment & Management

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

Medical Care

While ruling out the various disease states that have been associated with livedoid vasculopathy, physicians can offer a number of therapies that have been very helpful in reducing pain and ulceration. Instituting treatment as soon as possible is best.

Pentoxifylline (Trental) (400 mg 3 times/d) may be effective. Pentoxifylline is believed to enhance the blood flow in the capillaries. The blood flow enhancement is attributed to making red blood cells more flexible and thereby reducing viscosity. [44]

In 2003, Hairston et al [45] described treatment of livedoid vasculopathy with low molecular weight heparin (LMWH).

As reported by Yang et al [46] in 2003, intractable livedoid vasculopathy was successfully treated with hyperbaric oxygen therapy. Additionally, Juan et al [47] reported a study of 12 subjects with active livedoid vasculopathy. Subjects received hyperbaric oxygen therapy 5 times/wk. Eight completed the study. Resumption of ambulation and reduction of analgesics were achieved after an average of 4.9 hyperbaric oxygen therapy sessions. Leg ulcers healed completely in these 8 subjects at a mean of 3.4 weeks (range, 2-5 wk). Six patients had relapses of ulceration and responded to additional hyperbaric oxygen therapy. No patients had adverse effects.

Also in 2003, Marzano et al [33] noted a good clinical response was obtained using intravenous methylprednisolone combined with pentoxifylline for wide spread livedoid vasculopathy.

Dipyridamole (Persantine) (75 mg 4 times/d) with up to 325 mg of aspirin per day is reported to reduce pain after 3-6 weeks of therapy. Similar results have been reported using 50 mg of dipyridamole 3 times a day and 325 mg of aspirin once a day. [48] Note that aspirin is not to be administered in conjunction with coumarin anticoagulants. Dipyridamole is not considered safe in children or breastfeeding mothers.

Nifedipine (Procardia) (20 mg 3 times/d) is reported to maintain perfusion in the superficial vessels; therefore, the deposition of fibrin in the vessel walls is impeded. [49]

Deng et al [10] noted that livedoid vasculopathy associated with plasminogen activator inhibitor-1 (PAI-1) promoter homozygosity (4G/4G) was effectively abated with tissue-type plasminogen activator (tPA). tPA was used to treat a patient with elevated levels of PAI-1 and led to a reduction of ulceration. [10] Antunes et al also reported on livedoid vasculopathy associated with PAI-1 promoter homozygosity (4G/4G) and prothrombin G20210A heterozygosity that responded to tPA treatment. [50]

Several reports have noted that intravenous immunoglobulin (IVIG) can be useful in treating atrophie blanche and livedoid vasculopathy, but this remains an experimental treatment. [51] Bounfour et al treated five patients with livedoid vasculopathy with IVIG and four patients experienced successful outcomes. [52] In 2018, Yoshioka et al reported healing of ulcers in a patient with systemic lupus erythematosus and livedoid vasculopathy treated with IVIG. [53]

The combination of phenformin and ethylestrenol, which enhances endogenous blood fibrinolytic activity by increasing plasminogen activating enzymes, has been suggested as a treatment.

Browning and Callen [54] reported that warfarin is a useful and effective treatment for livedoid vasculopathy associated with cryofibrinogenemia and hyperhomocysteinemia. Kavala et al reported successful warfarin therapy in livedoid vasculopathy associated with factor V Leiden mutation. [55] Additionally, Davis and Wysokinski reported that livedoid vasculopathy associated with a prothrombotic state responded to warfarin. [56]

Some reports have noted the use of heparin, [57] LMWH, psoralen plus ultraviolet A (PUVA), and low molecular weight dextran.

The use of psoralen plus ultraviolet A (PUVA) resulted in the healing of primary lesions and a reduction of livedoid vasculopathy symptoms in a study of eight patients. [58]

If ulcers are superinfected, they should be treated with oral antibiotics.

Marsch et al noted that hyperhomocysteinemia is associated with livedoid vasculopathy, and the combination of folic acid, vitamin B-12, and vitamin B-6 (cofactors of homocysteine metabolism) is an effective treatment. [59]

In a study of 26 men and women, 20 patients had at least one thrombophilia factor. [60] Ten patients had a peripheral neuropathy, with two of these patients demonstrating a specific thromboocclusive vasculopathy on muscle biopsy. Anticoagulation with LMWH was the most prescribed therapy and was effective in 14 patients. Eight patients had severe disease refractory to anticoagulation and required IVIG, producing a good response in six patients. Thus, IVIG can be used when patients are resistant to anticoagulation therapy alone.

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Consultations

To fully evaluate for the comorbid conditions of livedoid vasculopathy, consult a hematologist (to evaluate for factors that lead to hypercoagulable states) and vascular surgeons (to evaluate and treat underlying defects of coagulation).

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