Livedoid Vasculopathy Clinical Presentation

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

History

In livedoid vasculopathy, the initial findings are typically painful purpuric macules or papules on the ankles and the adjacent dorsum of the feet. Ulceration, red streaks, and hyperpigmentation may also be seen on the lower extremities. [27] Patients may have a history of livedo reticularis on their lower legs. The history may help exclude other diagnostic considerations.

Medium-sized vasculitides, such as polyarteritis nodosa (PAN) is occasionally present with ulceration, resulting in ivory-white, stellate scarring on the lower limbs. These conditions may potentially be misdiagnosed as livedoid vasculopathy.

Chronic periarteritis nodosa may be associated with painful ulcerations; however, the associated nodules should differentiate chronic periarteritis nodosa from livedoid vasculopathy.

Livedoid vasculopathy is not associated with edema or venous insufficiency, while stasis dermatitis with ulceration is usually not painful and is associated with obvious edema and signs of venous insufficiency.

Patients with livedoid vasculopathy may have a history of recurrent leg ulcerations. Such patients can have deficiencies in a variety of blood factors (eg, factor V Leiden, protein C). The factor V Leiden mutation is more frequent in patients with venous leg ulceration than in control subjects and the general population. Patients with the factor V Leiden mutation have an increased risk of developing deep venous thrombosis and recurrent leg ulceration. Patients may also have a history of increased plasma homocysteine levels, abnormalities in fibrinolysis, and increased platelet activation.

Livedoid racemosa is a dermatological condition characterized by discoloration of skin in a netlike pattern and is found on the limbs or trunk. [28] The presence of livedo racemosa in various locations throughout the body may be related to livedoid vasculopathy. In 2019, Weishaupt et al noted 23 of 27 patients in a multicenter study presented with livedo racemosa on the foot. Livedoid racemosa was present on the upper legs and arms in some patients. [29]

Livedoid vasculopathy and hypertensive ischemic ulcers are both painful, but hypertensive ischemic ulcers usually are larger and lack telangiectatic purple borders. [30]

History and careful follow-up care can rule out traumatic ulcers and distinguish them from livedoid vasculopathy.

Chronic periarteritis nodosa may be associated with painful ulcerations; however, the associated nodules should differentiate chronic periarteritis nodosa from livedoid vasculopathy.

In 2003, Toth et al [31] noted mononeuropathy multiplex in association with livedoid vasculopathy. It is possible that there are neural links underlying livedoid vasculopathy. [32]

In 2003, Marzano et al [33] described a 37-year-old woman with a 13-year history of widespread livedo reticularis and recurrent, painful, ulcerative skin lesions. The recurrent ulcerations involved almost the entire body surface. In addition, malar erythema and edema, nonscarring alopecia, and fever were also associated with this patient's condition. Routine laboratory data, immunological investigations, and coagulation parameters were normal or negative.

In 2007, Cardoso et al [34] noted livedoid vasculopathy and hypercoagulability in a patient with primary Sjögren syndrome.

The range of presentation of livedoid vasculopathy is wide, and Okada et al reported widespread livedoid vasculopathy with pain but no systemic symptoms, showing that sometimes the disease is confined to the skin. [35]

Livedoid vasculopathy in a woman with multiple myeloma has been reported. [36]

Livedoid vasculopathy has a complex relationship with lupus. [37]

In 2018, livedoid vasculopathy was reported in a patient with sickle cell disease. It has previously been reported in a patient with sickle cell trait. [38]

There have been several cases documenting associations between livedoid vasculopathy and solid-organ cancer. [30]

Anavekar and Kelly noted a heterozygous prothrombin gene mutation associated with livedoid vasculopathy. [39]

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Physical Examination

The initial lesions of livedoid vasculopathy, which often appear in clusters or groups, eventually ulcerate over a period of months to years and form irregular patterns of superficial ulcers. When the ulcers finally heal, they leave behind atrophic porcelain-white scars known as atrophie blanche, which can be seen below.

Atrophie blanche in livedoid vasculopathy. Courtes Atrophie blanche in livedoid vasculopathy. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/vascular/atrophie3.jpg).

Livedoid vasculopathy can manifest as livedo reticularis before ulceration, with or without ulcerations.

Patients with livedoid vasculopathy can have Raynaud phenomenon and acrocyanosis.

Lipodermatosclerosis can be associated with livedoid vasculopathy.

Patients who have systemic diseases, such as lupus, rheumatoid arthritis, and Klinefelter syndrome resulting in skin ulcers, can manifest atrophie blanche–like lesions. These patients do not have livedoid vasculopathy.

Livedoid vasculopathy has a complex relationship with lupus, which can cause inflammation in blood vessels in the lower extremities. [37] Circulating lupus anticoagulant should be noted. [40]

Livedoid vasculopathy and recurrent thrombosis in a patient with lupus has been recorded. This case, the first reported of livedoid vasculopathy in a patient with seronegative antiphospholipid syndrome and systemic lupus erythematosus, draws attention to livedoid vasculopathy, a thrombotic dermopathy that may be under-diagnosed in patients with antiphospholipid syndrome.

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Complications

Livedoid vasculopathy can result in atrophie blanche or white stellate scars.

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