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

Abstract

Livedoid vasculopathy (LV) is a thrombotic skin disease characterized by episodic painful ulcerations of the distal aspects of the legs. Its healing process typically leaves small porcelain-white scars called atrophie blanche as a result of the occlusion of cutaneous microcirculation. The main goals of the treatment are pain management and the prevention of ulceration and of progressive scarring in the malleolar area. The therapeutic management is still a challenge, however, and most treatments were based on anecdotal off-label protocols. Over such context, direct oral anticoagulants (DOACS) arise as a potential treatment for this disease. This class of medications became an alternative from initial large studies applied on different pathologic scenarios regarding thromboembolic events. In that line, recent case series using DOACS, including rivaroxaban, started to emerge in the literature related to LV and reported successful prevention of cutaneous infarctions and ulcerations, providing physicians with a new promising alternative. The current report describes four cases of long-term recalcitrant LV, in which rivaroxaban monotherapy effectively reduced pain and cutaneous ulcerations in a few weeks of treatment without relevant side effects. The authors also review therapy management of the disease, focused on DOACS, and suggest a step-by-step approach to treat these patients, taking into consideration different resource profiles of each level of local health centers, the gravity of the cases, and risks/benefits for patients.

Livedoid vasculopathy: Clinical course and long‐term outcome in Asian patients with a review of the literature

“…Table 3 shows the articles from our review of the literature for studies that reported on anticoagulant treatment in LV patients (4 prospective studies, 2 retrospective studies, and 33 case reports) 2,5‐42 . From 39 studies, 219 patients with 139 (63.5%) females and 80 (36.5%) males were included.…”

A Case of Livedoid Vasculopathy Successfully Treated with Sulodexide

“…In the report of So et al 6 , a combination of low-dose aspirin with oral warfarin, an anticoagulant, achieved successful treatment of LV in primary antiphospholipid syndrome. Recently, Franco Marques et al 7 reported favorable treatment results of four LV cases using rivaroxaban, suggesting that direct oral anticoagulants (DOACs) could play a role in improving 'recalcitrant' LV, an unresponsive state to oral pentoxifylline and systemic corticosteroid treatment. Clinical evidence of oral sulodexide has been presented in some previous studies.…”

Recognition and Management of Cutaneous Polyarteritis Nodosum Versus Livedoid Vasculitis: A Case Report

“…However, proper disease recognition and diagnosis may foster more efficient pharmacologic management, including the prompt use of corticosteroids and other adjuvant immunosuppressant therapies such as cyclophosphamide, rather than initiating anti-platelet and or fibrinolytic therapy, perhaps decreasing the severity of exacerbations and risk of progression to systemic PAN [1,3]. Another therapeutic goal for CPAN patients is pain management and prevention of further ulceration, in which direct oral anticoagulants (DOACs), including rivaroxaban monotherapy, effectively reduced both without relevant side effects in some patients [6]. Patients presenting with refractory disease and atrophie blanche lesions are often placed on a myriad of pharmacologic trials and advised smoking cessation, avoid irritants, begin compression therapy, elevate distal extremities, and have basic wound care readily available [7].…”