Livedoid vasculopathy: A review of pathogenesis and principles of management

Abstract

Livedoid vasculopathy is a rare cutaneous disease manifesting as recurrent ulcers on the lower extremities. The ulceration results in atrophic, porcelain white scars termed as atrophie blanche. The pathogenesis is yet to be understood with the main mechanism being hypercoagulability and inflammation playing a secondary role. The important procoagulant factors include protein C and S deficiency, factor V Leiden mutation, antithrombin III deficiency, prothrombin gene mutation and hyperhomocysteinemia. Histopathology of livedoid vasculopathy is characterized by intraluminal thrombosis, proliferation of the endothelium and segmental hyalinization of dermal vessels. The treatment is multipronged with anti-thrombotic measures such as anti-platelet drugs, systemic anticoagulants and fibrinolytic therapy taking precedence over anti-inflammatory agents. Colchicine, hydroxychloroquine, vasodilators, intravenous immunoglobulin, folic acid, immunosuppressive therapy and supportive measures are also of some benefit. A multidisciplinary approach would go a long way in the management of these patients resulting in relief from pain and physical as well as psychological scarring.

Characteristics, risk factors and treatment reality in livedoid vasculopathy – a multicentre analysis

“…In the stage of atrophie blanche, there is hyalinization of the dermis and capillary walls. It is important to recognize that the histopathological appearance varies at different stages of the disease . All participating patients provided written‐informed consent.…”

The management of livedoid vasculopathy focused on direct oral anticoagulants (DOACs): four case reports successfully treated with rivaroxaban

“…With a similar mechanism of action, Dapsone is another option recommended in doses of 50–100 mg daily. Doxycycline at a dose of 100 mg twice a day can also be used . Furthermore, ASA is habitually used in doses ranging from 75 to 325 mg. Higher doses may increase the risk of bleeding and should be used only in specific cases, such as when there is associated sickle cell trait …”

“…Weighing the functional repercussions on the quality of life of these individuals puts great importance on the fact that despite numerous available therapeutic agents known to date, there has been not one that emerged as an efficacious treatment for LV for a long time. Part of the problem dwells in the low incidence of the disease, causing most of the treatment options to be based on isolated case reports or case series . Therefore, livedoid vasculopathy has always been a great therapeutic challenge, and the goals to prevent pain and the progressive scarring in the malleolar region were unseemly with the needs of patients.…”

Topical becaplermin gel is an effective adjuvant for long‐standing ulcers of livedoid vasculopathy recalcitrant to anticoagulant therapy

“…A tapering 2-week course of oral prednisolone (30 mg once daily for 1 week, 20 mg once daily for another week and then stopped) and nifedipine 60 mg/day failed to produce any relief. 1 Treatment of LV is particularly difficult as immunosuppressives have no role and relief, if any, is partial and temporary. Within 2 months, new lesion activity reduced but older lesions showed poor healing and there was significant pain (Fig.…”

“…Enoxaparin 40 mg was given SC for 10 days followed by oral warfarin (initial dose range 3-5 mg/day) titrated to prothrombin time and international 4 normalized ratio [(PT/INR); initially kept at 2-2.5, later 1-1.5] with continuation of nifedipine and pentoxifylline produced a modest reduction in oozing, inflammation and pain. 1,2 Most chronic wounds show impaired angiogenesis leading to delayed healing. 2a).…”