Última actualización:
6/16/2025
Años publicados: 2025
NORD gratefully acknowledges Harish Eswaran, MD, Assistant Professor, Department of Pediatrics, University of Texas Health Science Center Houston, for assistance in the preparation of this report.
Summary
Livedoid vasculopathy is a rare blood vessel disorder in which skin discoloration, painful ulcers and scarring occur in both lower legs (usually near the ankle). These symptoms occur because of blood clots forming in small blood vessels of the skin.1,2
Livedoid vasculopathy can occur by itself or as part of other diseases that cause the blood to clot more easily, like systemic lupus erythematosus, scleroderma, antiphospholipid syndrome and different types of blood clotting disorders (thrombophilia).3
Livedoid vasculopathy is more likely to occur in middle-aged females.3 It is diagnosed based on a skin biopsy.1 Treatment involves a combination of therapies which may include wound care, leg compression, pain medications, blood thinners, clot-busting medications (thrombolytics) and immunotherapy.1
Introduction
The name livedoid vasculopathy comes from the purple skin discoloration (known as livedo in Latin) seen in the condition, as well as the observation that it is a disease of blood vessels (vasculopathy) in the skin. Livedoid vasculopathy has undergone name changes as more has been learned about the underlying disease process that contributes to the condition. Â Early on, it was given names such as livedo reticularis with ulceration or atrophie blanche based on its appearance.1 However, these features are not unique to livedoid vasculopathy. Livedo reticularis, a weblike pattern of purple skin discoloration, is a common, harmless condition that typically occurs without painful ulcers. Atrophie blanche, the white scars left after ulcers heal, can also occur with other conditions such as chronic venous insufficiency.4 Livedoid vasculopathy was also incorrectly referred to as livedoid vasculitis in the past. Vasculitis implies that the condition causes inflammation of the blood vessels, but this type of inflammation does not occur in livedoid vasculopathy.1 The term segmental hyalinizing vasculopathy has also been used to describe livedoid vasculopathy and is based on microscopic findings seen in the blood vessels of people with the condition.5
The main signs and symptoms of livedoid vasculopathy include a combination of flat or raised red bumps, livedo reticularis, painful ulcers and atrophie blanche, usually near both ankles.1,3 Rarely, these findings may occur on just one leg.1 The skin changes of livedoid vasculopathy can also affect the upper part of the feet and shins.1,2
The first symptoms of livedoid vasculopathy are clusters of painful, flat or raised spots in the skin around the ankle.1,6 These spots are star shaped and red or purplish in color. They usually turn into ulcers over months to years and may be accompanied by livedo reticularis.6 Livedo reticularis is red to purplish skin discoloration that creates a lacy, netlike pattern.3 Livedo reticularis usually appears before ulcers appear.3
The ulcers of livedoid vasculopathy are typically small (1 to 5 mm in diameter) and occur in the superficial layers of the skin in an irregular pattern.1,6 The ulcers of livedoid vasculopathy are usually crusty in appearance and painful, with a burning sensation.3,4 People with livedoid vasculopathy usually have multiple ulcers at different stages of healing.6
Atrophie blanche refers to the whitish scars that appear as the ulcers of livedoid vasculopathy heal.5,6 These scars are also star shaped.
Less typical symptoms that have occurred in people with livedoid vasculopathy include bluish discoloration and numbness of the feet and skin hardening of the lower legs.6
Livedoid vasculopathy is often a chronic and relapsing disease, meaning symptoms come and go but continue to recur over time. Complications of livedoid vasculopathy can also occur and include ulcers that fail to heal, skin breakdown and skin infections.3
Livedoid vasculopathy can occur on its own or can be part of another disease. Diseases associated with livedoid vasculopathy include certain blood clotting disorders (thrombophilia), autoimmune connective tissue diseases and some cancers.3,6 When people have livedoid vasculopathy due to another disease, they will present with additional symptoms that affect other body parts. These additional symptoms will vary based on the underlying diagnosis.
Livedoid vasculopathy occurs because small blood vessels in the skin (capillaries) of the lower legs become blocked with blood clots (thrombi) which reduces blood flow to the skin and causes the characteristic skin findings.3 The exact cause of blood clot formation in people with livedoid vasculopathy is unknown but these blood clots are thought to occur because of abnormal proteins and enzymes involved in the processes of blood clotting and blood clot breakdown.3 As a result, clots form more easily and the natural and regular process of breaking down clots (known as fibrinolysis) becomes impaired. Inflammatory damage to the inner lining of these blood vessels may also play a role in triggering flares but is not the root cause.3 Sluggish blood flow may explain why it tends to affect the legs.
More specifically, people with livedoid vasculopathy are thought to have increased blood clotting because of enhanced fibrin formation, lower levels of tissue-type plasminogen activator and increased platelet aggregation (ie, increased clumping of platelets).5 Fibrin is a protein that makes up blood clots.7 Tissue-type plasminogen activator is a protein released by the cells of the inner lining of blood vessels to promote the breakdown of fibrin.5 Platelets are blood cells that clump together to form clots and stop a damaged blood vessel from continuing to bleed.8
Some cases of livedoid vasculopathy occur in people with diseases that promote blood vessel damage and clot formation, including sickle cell disease, factor V Leiden, protein C deficiency, protein S deficiency, antithrombin deficiency, antiphospholipid syndrome, systemic lupus erythematosus, scleroderma, rheumatoid arthritis and certain cancers.3,5 These diseases are thought to contribute to the development of livedoid vasculopathy in people who have them.
Livedoid vasculopathy occurs in 1 out of 100,000 people in North America per year. It is around three times more common in females than males.5 Livedoid vasculopathy can start at any age but it typically affects people between the ages of 15 and 50 years old. The average age when the condition is diagnosed is 32 years old.3,5 Livedoid vasculopathy is reported to occur at a higher rate in pregnant females and during the summertime.3
Because the signs and symptoms of livedoid vasculopathy overlap with those of other diseases, a skin biopsy is necessary to diagnose the condition.1 For a skin biopsy, a sample of skin is taken from the edge of a new raised spot or ulcer so that it can be observed under a microscope. Characteristic findings of livedoid vasculopathy under the microscope include a blood clot (thrombus) within a vessel, an increase in endothelial cells (cells that form the inner lining of blood vessels) and thickened blood vessel walls with a glassy appearance to some cells (hyaline degeneration).1,18
After livedoid vasculopathy is confirmed through a biopsy, other disorders that are associated with livedoid vasculopathy must be ruled out, including connective tissue disease, paraproteinemias and inherited or acquired diseases that make blood clots more likely to develop. Blood tests can be performed to help diagnose these diseases.1 Which specific tests are ordered will depend on any additional signs and symptoms the person has.
Ankle-brachial index testing, which compares blood pressure in the leg with blood pressure in the arm, should be done to rule out peripheral artery disease that is another potential cause of leg ulcers. Lower limb venous Doppler ultrasound can also be performed to test blood flow in the veins of the lower legs and rule out chronic venous insufficiency.1,3,11
Effective treatment regimens for livedoid vasculopathy vary by patient and are often determined by trial and error. For the best outcomes, patients with livedoid vasculopathy should be seen by multiple specialists, including dermatologists, hematologists and rheumatologists, especially when livedoid vasculopathy is associated with another illness. Early diagnosis and treatment are associated with better outcomes.3
Treatment includes a combination of non-medication and medication options. Smoking cessation may be encouraged for people with livedoid vasculopathy who smoke. Livedoid vasculopathy is thought to be more likely to occur in people who smoke. Additionally, smoking slows the ability of wounds to heal.1,19
To encourage faster wound healing, patients usually receive education on wound care which involves keeping the ulcerated skin clean and moistened with topical gels that promote healing.1,19 Sometimes removal of dead skin tissue (debridement) by a wound care specialist is recommended to promote healing. Compression therapy, in which tight stockings are worn on the lower legs to encourage blood flow, may be recommended to help with wound healing as well. Compression therapy is also used to treat venous insufficiency in people who also have chronic venous insufficiency.2,3
Drug therapies used to treat livedoid vasculopathy may include antibiotics for people with signs of skin infection in the affected regions.19 Pain medications used to treat the painful ulcers include acetaminophen, nonsteroidal anti-inflammatory drugs, amitriptyline, gabapentin, pregabalin or carbamazepine.1,3 Topical lidocaine may also be helpful.1 Medications often prescribed for livedoid vasculopathy to target the clotting aspect of the disease process are anticoagulants (blood thinners that target proteins called clotting factors to prevent them from clotting blood so easily) and antiplatelets (that keep platelets from clumping together and blocking blood vessels). Some of these medications are aspirin, dipyridamole, pentoxifylline, low–molecular weight heparin, warfarin, rivaroxaban and dabigatran.3 If a patient continues to have symptoms with anticoagulant or antiplatelet therapy, they may also be prescribed steroids to reduce inflammation. Some of the steroids prescribed for livedoid vasculopathy include danazol, betamethasone sodium and methylprednisolone.20 Drugs that target the immune system may be especially helpful for people with livedoid vasculopathy who have a co-occurring autoimmune connective tissue disease (eg, scleroderma, lupus, rheumatoid arthritis).19
Long-term treatment is usually necessary to keep symptoms of livedoid vasculopathy under control.1 Some people continue to have ulcers despite taking standard treatments. For these more resistant cases, additional therapies can be tried.
Some people with livedoid vasculopathy can be resistant to standard treatments. However, other, less common treatments are available for these patients.2 Alternative methods for wound care to promote healing of ulcers include hyperbaric oxygen therapy and ultraviolet light therapy. Hyperbaric oxygen therapy promotes the growth of new skin and blood vessels by exposing patients to pure oxygen in a pressurized chamber.21 Ultraviolet light therapy involves exposing the affected skin to ultraviolet radiation from machines to stimulate skin growth. Ultraviolet light therapy exposes patients to ultraviolet radiation similar to that from the sun, but in safer doses that are meant to minimize skin damage.22
Thrombolytic, or fibrinolytic, therapy can be used as an alternative to anticoagulants and antiplatelet medications when patients do not respond to these medications.2 Anticoagulants and antiplatelets are meant to prevent blood clots from forming. Fibrinolytic therapy, by contrast, is meant to dissolve clots that have already formed; it does so by breaking up fibrin, a major component of blood clots.23 An example of a fibrinolytic drug that may be prescribed for livedoid vasculopathy is recombinant tissue plasminogen activator.3,19
Alternative drugs to steroids for altering the immune system in patients with livedoid vasculopathy (especially patients with an associated autoimmune disease) include intravenous immunoglobulin (IVIG) therapy, rituximab, tumor necrosis factor–alpha (TNF-alpha) inhibitors and Janus-activated kinase (JAK) inhibitors.2,19 IVIG therapy involves treating patients with antibodies donated by other people. This treatment reportedly provides rapid pain relief and improves atrophie blanche in livedoid vasculopathy patients.3,19 Rituximab is a type of monoclonal antibody made in a lab that targets specific receptors on certain immune cells to dampen inflammation. Rituximab has been reported to reduce pain and heal ulcers in some livedoid vasculopathy patients who had a limited response to more typical treatments.20,24 JAK and TNF-alpha inhibitors block proteins that can promote inflammation; the JAK inhibitor baricitinib and the TNF-alpha inhibitor etanercept have reportedly been used successfully in some livedoid vasculopathy patients.19
Other drugs known as vasodilators may be used to treat livedoid vasculopathy, alongside the more standard therapies. Vasodilators cause blood vessels to dilate, promoting increased blood flow to the skin. Some examples of vasodilators used for livedoid vasculopathy are nifedipine and cilostazol.3
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www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
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