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Livedoid Vasculopathy / Livedoid Vasculitis

By BSc (Hons)

Livedoid vasculopathy (LV) is a painful, recurrent and chronic disorder of the microcirculation in the skin. LV is also known as livedoid vasculitis and it is a relatively rare dermatosis that occurs most commonly in women from their youth to middle ages. The lesions are often located in the distal parts of the lower extremities and feet.

LV presents clinically with a triad of symptoms. These symptoms do not necessarily need to occur simultaneously or in any particular order. The symptoms are painful ulcerations, livedo racemosa (skin discoloration presenting as irregular broken circles)  and scars that are called atrophic blanche.

Pathophysiology of LV

The pathophysiology of LV is not well understood, but the condition may be categorized as either primary or secondary. The primary form does not appear to have any associations with other diseases. However, the secondary form of LV is associated with diseases that are characterized by hypercoagulability (i.e. thrombophilia). These diseases may arise due to a fibrinolytic disorder like protein C deficiency, hyperhomocysteinemia and prothrombin gene mutations. In other cases, LV appears to be associated with disorders affecting the connective tissue. These include, but are not limited to antiphophospholipid syndrome (APS), systemic lupus erythematosus and cryoglobulinemia.

The disturbed microcirculation seen in LV seems to be due to the deposition of fibrinoid material (clots) both within the walls and lumina of the upper and mid-dermal vessels. This causes a marbled discoloration of the skin which appears lacy – this is associated with livedo racemosa.

Following clot formation, there is poor perfusion of blood within areas served by the affected blood vessels. Thus, these areas become ischemic and acute ischemic pain (angina cutis) eventually results. The area of the affected skin may undergo necrosis and ulceration in cases where there is persistence of the developed ischemic state. This is often the stage at which patients consult a doctor, because the painful ulcerations of the feet, ankles, and legs begin to negatively impact their quality of life.

The ulcerations do not begin as particularly deep; however, as they heal, they undergo a form of remodeling and the skin is left with significant deep atrophic scars known as atrophie blanche. These porcelain-white and stellar scars are irreversible and often surrounded by punctate telangiectasia and hyperpigmentation, of which the latter can in time become partially resorbed.

Diagnosis of LV

The appropriate diagnosis of LV requires a specific skin biopsy at a site where the healthy skin transitions into the affected. LV is purely a cutaneous form of ischemia, therefore,  no systemic involvement is expected and thus no damage to any other organ and no other diagnostics required.

Histologic identification might allow for the detection of pro-thrombotic markers in the blood if LV is due to upregulated coagulation. However, even if these markers are present, they cannot definitely be considered as the cause of LV. In fact, one example is lipoprotein (a) which can be altered by dietary or medicinal means.

Treatment of LV

Part of the reason that there is no prominent and effective treatment for LV is the uncertain nature of its pathogenesis. Due to this, current treatment options might rely on data acquired solely from reports of single cases or a series of them. Many treatments exist to improve the physical manifestations (ulcerations) and reduce the pain. Alternatively, some treatments aim to control the disease process. However, cases can prove difficult to treat and the devised treatments may even lead to side effects that hamper enthusiasm for their use.

References

Reviewed by Jonas Wilson, Ing. Med.

Further Reading

    Last Updated: May 22, 2016

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