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Livedoid vasculopathy
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Livedoid vasculopathy, or livedoid vasculitis, is a painful ulcerative condition of the lower extremities with characteristic clinical and histopathologic features. Pain is a prominent feature, associated with tiny infarctive, shallow ulcerations most often involving the legs around the feet and ankles. This condition is difficult to treat and often recalcitrant to therapy. Healed ulcers have the non-specific appearance of atrophie blanche, characterized by smooth, porcelain-white lesions surrounded by punctate telangiectasia and hyperpigmentation; this appearance also may be seen in the context of healed ulcers due to venous insufficiency.
Management Strategy
Clinicopathologic correlation for a diagnosis of livedoid vasculopathy is necessary before treatment options can be considered. Histologic identification of the characteristic segmental hyalinized appearance of the dermal blood vessels is important to exclude other causes of lower extremity ulcers. Increasing numbers of reports suggest that the disease has a procoagulant predisposition, with both hereditary and acquired hypercoagulable states; therefore, a comprehensive coagulation screen is recommended.
Typically shallow and numerous, the ulcers in livedoid vasculopathy are painful and slow healing. Wound care is an important facet of treatment. Excellent dressings and topical products are available to treat chronic ulcerative diseases, and selection depends on the moisture content of the wound and the possibility of superinfection. Pain management is also essential.
Because of the increasing evidence supporting procoagulant mechanisms involved in disease etiology, medical therapy involves prevention and treatment of dermal vessel thrombosis and improvement of vascular perfusion. Medical management has included aspirin (acetylsalicylic acid), niacin (nicotinic acid), pentoxifylline, dipyridamole, warfarin, minidose heparin, subcutaneous low-molecular-weight heparin injections, danazol, and more recently direct-acting oral anticoagulants such as rivaroxaban . Systemic corticosteroids are not considered a primary therapy; however, some patients’ conditions have improved with immunosuppressants in combination therapy. Psoralen with ultraviolet A (PUVA) has also been used. Patients with livedoid vasculitis recalcitrant to traditional management have been treated with certain prostanoids, intravenous immunoglobulin (IVIG) , and a tissue-type plasminogen activator (tPA) .
Specific Investigations
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Livedoid vasculopathy: an intriguing cutaneous disease
Criado PR, Rivitti EA, Sotto MN, et al. An Bras Dermatol 2011; 86: 961–77.
Livedoid vasculopathy was originally described as a clinical expression of vasculitis; however, current understanding of the main pathogenetic mechanism suggests a vasoocclusive phenomenon due to intraluminal thrombosis of dermal vessels. The authors review etiologic associations, publications on livedoid vasculopathy associated with hypercoagulable conditions, and options for treatment.
Livedoid vasculopathy: an in-depth analysis using a modified Delphi approach
Alavi A, Hafner J, Dutz JP, et al. J Am Acad Dermatol 2013; 69: 1033–42.
This article reviews nomenclature changes with this condition. An illustrative approach to diagnosis and review of treatments are well presented.
Association between peripheral vascular endothelial dysfunction and livedoid vasculopathy
Yang CH, Shen SC, Hui RC, et al. J Am Acad Dermatol 2012; 67: 107–12.
Sixteen patients diagnosed with livedoid vasculopathy were compared to 16 matched control subjects in this prospective study using flow-mediated vasodilation of the brachial artery, an indicator of vascular endothelial function, using high-resolution, two-dimensional ultrasonic imaging. Peripheral vascular endothelial dysfunction was demonstrated in patients, providing some evidence of endothelial dysfunction in the development of the disease. Decreased nitric oxide bioavailability may be the main reason for this dysfunction; however, the authors noted that more studies are needed to clarify the mechanism.
Livedoid vasculopathy: further evidence for procoagulant pathogenesis
Hairston BR, Davis MD, Pittelkow MR, et al. Arch Dermatol 2006; 142: 1413–8.
This retrospective study of 45 patients with biopsy-proven livedoid vasculopathy analyzed the presence of coagulation abnormalities. The laboratory results revealed numerous heterogeneous coagulation abnormalities, including factor V Leiden mutations, protein C or S abnormalities, prothrombin G20210A gene mutations, lupus anticoagulant, anticardiolipin antibodies, and elevated homocysteine, thus providing further evidence of procoagulant mechanisms for this disease.
Livedoid vasculopathy: the role of hyperhomocysteinemia and its simple therapeutic consequences
Meiss F, Marsch WC, Fischer M. Eur J Dermatol 2006; 16: 159–62.
Hypercoagulability due to hyperhomocysteinemia was recognized as a potentially contributing factor to livedoid vasculopathy in a 49-year-old woman.
Livedoid vasculopathy and its association with factor V Leiden mutation
Yong AA, Tan AW, Giam YC, et al. Singapore Med J 2012; 53: 258–60.
Two cases of livedoid vasculopathy with factor V Leiden mutations are described.
Livedoid vasculopathy associated with heterozygous protein C deficiency
Boyvat A, Kundakci N, Babikir MO, et al. E. Br J Dermatol 2000; 143: 840–2.
Livedoid vasculitis in association with protein C deficiency is described in one patient.
Livedoid vasculitis: a manifestation of the antiphospholipid syndrome
Acland KM, Darvay A, Wakelin SH, et al. Br J Dermatol 1999; 140: 131–5.
Four patients with ulcerative livedoid vasculitis are described, all of whom had associated elevated anticardiolipin antibody levels but no other evidence of systemic disease.
Atrophie blanche: a clinicopathological study of 27 patients
Yang LJ, Chan HL, Chen SY, et al. Changgeng Yi Xue Za Zhi 1991; 14: 237–45.
Twenty-seven patients were reviewed with respect to mean age at onset, disease duration, natural course, and clinical morphology. Thirteen patients responded to local wound care, bed rest, and low-dose aspirin plus dipyridamole as treatment for the first attack or recurrent episodes.
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