- Mark DP Davis, MD
Mark DP Davis, MD
- Professor of Dermatology
- Mayo Clinic College of Medicine
- Section Editors
- Jeffrey Callen, MD, FACP, FAAD
Jeffrey Callen, MD, FACP, FAAD
- Editor-in-Chief — Dermatology
- Section Editor — Skin and Systemic Disease
- Professor of Medicine
- University of Louisville School of Medicine
- Eric L Matteson, MD, MPH
Eric L Matteson, MD, MPH
- Section Editor — Treatment Issues in Rheumatology
- Division of Rheumatology
- Professor of Medicine
- Mayo Clinic College of Medicine
- Deputy Editors
- Abena O Ofori, MD
Abena O Ofori, MD
- Deputy Editor — Dermatology
- Assistant Professor of Medicine, Dermatology Division
- Medical College of Georgia
- Monica Ramirez Curtis, MD, MPH
Monica Ramirez Curtis, MD, MPH
- Deputy Editor — Rheumatology
- Instructor of Medicine, Part-time
- Harvard Medical School
Livedoid vasculopathy is a chronic, painful, thrombo-occlusive cutaneous vasculopathy that involves the distal lower extremities and feet. Characteristic clinical features include livedoid skin changes (linear or angular, erythematous nodules), atrophie blanche (smooth, ivory-white plaques), and ulceration. The diagnosis is confirmed through a skin biopsy that demonstrates characteristic vascular abnormalities, including intraluminal thrombosis, endothelial proliferation, and subintimal hyaline degeneration.
The pathogenesis of livedoid vasculopathy is unclear. The disorder can occur either independently or in association with acquired or inherited thrombophilia or various systemic diseases.
The clinical manifestations, diagnosis, and treatment of livedoid vasculopathy are reviewed here. Cutaneous vasculitis and other causes of leg ulcers are reviewed separately. (See "Overview of cutaneous small vessel vasculitis" and "Approach to the differential diagnosis of leg ulcers".)
The literature on livedoid vasculopathy is highly problematic with regard to nomenclature . A variety of other terms have been used to refer to this condition, most often "atrophie blanche" and "livedoid (or livedo) vasculitis." However, "atrophie blanche" is most appropriately used as a descriptive term for the smooth, ivory-white plaques that characteristically occur in livedoid vasculopathy but may also occur in other disorders, particularly venous insufficiency. The use of "vasculitis" should be avoided because true vasculitis, as demonstrated by destructive inflammation within the blood vessel wall, is absent. (See 'Clinical features' below and 'Pathology' below.)
Less common terms that have been used to refer to livedoid vasculopathy in the literature include segmental hyalinizing vasculopathy, livedo reticularis with summer ulcerations, livedo reticularis with winter ulcerations, and painful purpuric ulcers with reticular patterning on the lower extremities (PURPLE).
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- Alavi A, Hafner J, Dutz JP, et al. Livedoid vasculopathy: an in-depth analysis using a modified Delphi approach. J Am Acad Dermatol 2013; 69:1033.
- Hairston BR, Davis MD, Pittelkow MR, Ahmed I. Livedoid vasculopathy: further evidence for procoagulant pathogenesis. Arch Dermatol 2006; 142:1413.
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- Davis MD, Wysokinski WE. Ulcerations caused by livedoid vasculopathy associated with a prothrombotic state: Response to warfarin. J Am Acad Dermatol 2008; 58:512.
- Kavala M, Kocaturk E, Zindanci I, et al. A case of livedoid vasculopathy associated with factor V Leiden mutation: successful treatment with oral warfarin. J Dermatolog Treat 2008; 19:121.
- Browning CE, Callen JP. Warfarin therapy for livedoid vasculopathy associated with cryofibrinogenemia and hyperhomocysteinemia. Arch Dermatol 2006; 142:75.
- Hairston BR, Davis MD, Gibson LE, Drage LA. Treatment of livedoid vasculopathy with low-molecular-weight heparin: report of 2 cases. Arch Dermatol 2003; 139:987.
- Abou Rahal J, Ishak RS, Otrock ZK, et al. Livedoid vasculopathy in a patient with lupus anticoagulant and MTHFR mutation: treatment with low-molecular-weight heparin. J Thromb Thrombolysis 2012; 34:541.
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- Kerk N, Drabik A, Luger TA, et al. Rivaroxaban prevents painful cutaneous infarctions in livedoid vasculopathy. Br J Dermatol 2013; 168:898.
- Winchester DS, Drage LA, Davis MD. Response of livedoid vasculopathy to rivaroxaban. Br J Dermatol 2015; 172:1148.
- Lee JM, Kim IH. Case series of recalcitrant livedoid vasculopathy treated with rivaroxaban. Clin Exp Dermatol 2016; 41:559.
- Evans JM, Jensen JD, Sami N. Successful treatment of livedoid vasculopathy with rivaroxaban. JAAD Case Rep 2015; 1:340.
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- Antunes J, Filipe P, André M, et al. Livedoid vasculopathy associated with plasminogen activator inhibitor-1 promoter homozygosity (4G/4G) and prothrombin G20210A heterozygosity: response to t-PA therapy. Acta Derm Venereol 2010; 90:91.
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- Johnson DW, Hawley CM, Strutton G, Gibbs HH. Dramatic response of livedoid vasculitis to tissue plasminogen activator (tPA). Aust N Z J Med 1995; 25:370.
- Juan WH, Chan YS, Lee JC, et al. Livedoid vasculopathy: long-term follow-up results following hyperbaric oxygen therapy. Br J Dermatol 2006; 154:251.
- Kreuter A, Gambichler T, Breuckmann F, et al. Pulsed intravenous immunoglobulin therapy in livedoid vasculitis: an open trial evaluating 9 consecutive patients. J Am Acad Dermatol 2004; 51:574.
- Purcell SM, Hayes TJ. Nifedipine treatment of idiopathic atrophie blanche. J Am Acad Dermatol 1986; 14:851.
- Lee JH, Choi HJ, Kim SM, et al. Livedoid vasculitis responding to PUVA therapy. Int J Dermatol 2001; 40:153.
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- Bisalbutra P, Kullavanijaya P. Sulfasalazine in atrophie blanche. J Am Acad Dermatol 1993; 28:275.
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- EPIDEMIOLOGY AND PATHOGENESIS
- CLINICAL FEATURES
- ASSOCIATED DISORDERS
- DIFFERENTIAL DIAGNOSIS
- ADDITIONAL EVALUATION
- General measures
- Pharmacologic therapy
- - Our approach
- - Antiplatelet agents
- Aspirin with or without dipyridamole
- - Anticoagulants
- Warfarin or heparin
- Direct oral anticoagulants
- - Other therapies
- SUMMARY AND RECOMMENDATIONS