CLINICAL MANIFESTATIONS AND MANAGEMENT OF LIVEDOID VASCULOPATHY Clinical Manifestations and Management of Livedoid Vasculopathy

Livedoid vasculopathy (LV) is an extremely rare and distinct hyalinizing vascular disease affecting only one in 100,000 individuals per year.1,2 Formerly described by Feldaker in 1955 as livedo reticularis with summer ulcerations, LV is a unique non-inflammatory condition that manifests with thrombi formation and painful ulceration of the lower extremities.3 Clinically, the disease often displays a triad of livedo racemosa, slow-healing ulcerations, and atrophie blanche scarring.4 Although still not fully understood, the primary pathogenic mechanism is related to intraluminal thrombosis of the dermal microvessels causing occlusion and tissue hypoxia.4 We review a case in which the patient had LV undiagnosed and therefore inappropriately treated for more than 20 years. To reduce the current average five-year period from presentation to diagnosis, and to improve management options, we review the typical presentation, pathogenesis, histology, and treatment of LV.4 Case Report A 62-year-old Caucasian male presented in an assisted living facility setting with chronic, rightlower-extremity ulcers present for more than 20 years. The patient had a past medical history of chronic osteomyelitis, hypertension, and a belowthe-knee amputation of the left lower extremity secondary to a motorcycle accident. He denied having seen a primary care physician for more than 15 years as well as any medications, vitamins or supplements. Social history was significant for “many years” of tobacco use, heavy alcohol use, inconsistent housing with reoccurring assistedliving-facility admissions, low economic status, and medical non-adherence. The patient reported that throughout the last 20 years his wound-care management for these lesions was limited to the periods of time in which he was living in assistedliving facilities. Upon physical exam, the patient was found to have a wound on the right medial malleolus measuring 6.4 cm x 4.0 cm x 0.7 cm with moderate serous exudate, approximately 30% yellow necrosis and 70% granulation, with macerated wound margins (Figure 1), and a second wound on the right lateral malleolus measuring 6.0 cm x 5.5 cm x 0.4 cm with moderate serous exudate, approximately 20% sloughing tissue and 80% granulation (Figure 2). The patient was treated for one year, although inconsistently due to housing circumstances, with a series of necrotic-tissue debridements and a variety of topical antibiotics; however, despite therapy, the wounds failed to close. Throughout the duration of management, the patient repeatedly refused biopsy and hyperbaric oxygen therapy. Following one year of multiple topical antibiotics and debridement treatment resulting in minimal improvement, the patient finally consented to biopsy. The pathology report identified ulceration with fibrin in vessel walls associated with stasis dermatitis characterized by thick-walled capillaries and hemosiderin deposition consistent with livedoid vasculopathy (Figures 3). The patient refused any further treatment and was lost to follow-up. Discussion Livedoid vasculopathy affects women three times more than men, with a median age of 45 years, though it can vary from ages 10 to 85.1,2 Likely secondary to the rarity of the condition, there is much discussion regarding the name of the condition, as authors have written about LV under a wide array of monikers. Common names used synonymously with livedoid vasculopathy include: livedoid vasculitis, segmental hyalinizing vasculitis, livedo reticularis with summer ulcerations, and atrophie blanche en plaque.3,4 Although commonly used, the denotation of “vasculitis” is a bit misleading, since the primary mechanism of action is not actually inflammation.4 Due to the bilateral and symmetrical lower-extremity distribution of the eruptions, LV has also been nicknamed “PURPLE syndrome” (Painful Purpuric Ulcers with Reticular Pattern of the Lower Extremities).4 The pathogenesis of LV is not yet fully understood, but there is a consensus that it consists of dermal blood-vessel thrombosis leading to superficial tissue ischemia and necrosis, thus propagating pain and ulceration.1 According to the Virchow triad theory, the three factors that contribute to the development of thrombi, and therefore theoretically LV, include endothelial damage, inadequate blood flow, and hypercoagulability.2,5 It has been demonstrated that patients with LV exhibit decreased flow-mediated vasodilation of the brachial artery, signifying endothelial dysfunction.5 A decreased production or activity of nitrous oxide in endothelial cells has been detected in some cases, supporting the contribution of endothelial damage to LV.6 Additionally, the fact that fibrinolytics, antiplatelets and thrombolytic Figure 1. Right medial malleolus. 6.4 cm x 4.0 cm x 0.7 cm ulceration exhibiting moderate serous exudate, approximately 30% yellow necrosis, 70% granulation, and macerated wound margins. Figure 2. Right lateral malleolus. 6.0 cm x 5.5 cm x 0.4 cm ulcerations with moderate serous exudate, approximately 20% sloughing tissue and 80% granulation.