- Mark DP Davis, MD
Mark DP Davis, MD
- Professor of Dermatology
- Mayo Clinic College of Medicine
- Section Editors
- Gene G Hunder, MD
Gene G Hunder, MD
- Section Editor — Vasculitis
- Emeritus Consultant
- Professor Emeritus
- Mayo Clinic College of Medicine
- 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
- 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, recurrent, painful skin disorder that involves the distal lower extremities and feet. The disorder is a thrombotic condition associated with the formation of clots within the lumina of medium-sized arterioles and with hyalinizing changes within the subintimal region of involved vessels.
Livedoid vasculopathy can occur by itself or in the setting of a variety of systemic disorders, such as systemic lupus erythematosus and antiphospholipid syndrome. The disorder has also been linked to a growing list of hypercoagulable states. Livedoid vasculopathy is often misdiagnosed as either chronic venous insufficiency or some form of systemic or cutaneous vasculitis.
The clinical manifestations, diagnosis, and treatment of livedoid vasculopathy are presented here. Chronic venous insufficiency and small- and medium-vessel vasculitis of the skin are discussed elsewhere. (See "Clinical manifestations of lower extremity chronic venous disease" and "Diagnostic evaluation of chronic venous insufficiency" and "Overview of cutaneous small vessel vasculitis" and "Clinical manifestations and diagnosis of polyarteritis nodosa in adults".)
The literature related to livedoid vasculopathy is highly problematic with regard to nomenclature . There are three major sources of confusion:
●Atrophie blanche, the descriptive term for the major cutaneous manifestations of livedoid vasculopathy, is often employed interchangeably with the more appropriate names for the disease entity (livedoid vasculopathy or segmental hyalinizing vasculopathy) . (See 'Atrophie blanche' below.)
- Jorizzo JL. Livedoid vasculopathy: what is it? Arch Dermatol 1998; 134:491.
- Callen JP. Livedoid vasculopathy: what it is and how the patient should be evaluated and treated. Arch Dermatol 2006; 142:1481.
- Bard JW, Winkelmann RK. Livedo vasculitis. Segmental hyalinizing vasculitis of the dermis. Arch Dermatol 1967; 96:489.
- Gray HR, Graham JH, Johnson W, Burgoon CF Jr. Atrophie blanche: periodic painful ulcers of lower extremities. A clinical and histopathological entity. Arch Dermatol 1966; 93:187.
- Hairston BR, Davis MD, Pittelkow MR, Ahmed I. Livedoid vasculopathy: further evidence for procoagulant pathogenesis. Arch Dermatol 2006; 142:1413.
- Milian G. Les Atrophies Cutanees Syphilitiques. Bull Soc Franc Derm Syph 1929; 36:865.
- Alegre VA, Gastineau DA, Winkelmann RK. Skin lesions associated with circulating lupus anticoagulant. Br J Dermatol 1989; 120:419.
- Grob JJ, Bonerandi JJ. Thrombotic skin disease as a marker of the anticardiolipin syndrome. Livedo vasculitis and distal gangrene associated with abnormal serum antiphospholipid activity. J Am Acad Dermatol 1989; 20:1063.
- Lee JS, Kossard S, McGrath MA. Lymphocytic thrombophilic arteritis: a newly described medium-sized vessel arteritis of the skin. Arch Dermatol 2008; 144:1175.
- Vasudevan B, Neema S, Verma R. Livedoid vasculopathy: A review of pathogenesis and principles of management. Indian J Dermatol Venereol Leprol 2016; 82:478.
- 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.
- Allenby F, Boardman L, Pflug JJ, Calnan JS. Effects of external pneumatic intermittent compression on fibrinolysis in man. Lancet 1973; 2:1412.
- Yang LJ, Chan HL, Chen SY, et al. Atrophie blanche. A clinicopathological study of 27 patients. Changgeng Yi Xue Za Zhi 1991; 14:237.
- El Khoury J, Taher A, Kurban M, et al. Livedoid vasculopathy associated with sickle cell trait: significant improvement on aspirin treatment. Int Wound J 2012; 9:344.
- Maessen-Visch MB, Koedam MI, Hamulyák K, Neumann HA. Atrophie blanche. Int J Dermatol 1999; 38:161.
- Drucker CR, Duncan WC. Antiplatelet therapy in atrophie blanche and livedo vasculitis. J Am Acad Dermatol 1982; 7:359.
- Sauer GC. Pentoxifylline (Trental) therapy for the vasculitis of atrophie blanche. Arch Dermatol 1986; 122:380.
- Sams WM Jr. Livedo vasculitis. Therapy with pentoxifylline. Arch Dermatol 1988; 124:684.
- 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.
- Weishaupt C, Strölin A, Kahle B, et al. Anticoagulation with rivaroxaban for livedoid vasculopathy (RILIVA): a multicentre, single-arm, open-label, phase 2a, proof-of-concept trial. Lancet Haematol 2016; 3:e72.
- 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.
- 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.
- Tuchinda C, Leenutaphong V, Sudtim S, Lim HW. Refractory livedoid vasculitis responding to PUVA: a report of four cases. Photodermatol Photoimmunol Photomed 2005; 21:154.
- Bisalbutra P, Kullavanijaya P. Sulfasalazine in atrophie blanche. J Am Acad Dermatol 1993; 28:275.
- Gupta AK, Goldfarb MT, Voorhees JJ. The use of sulfasalazine in atrophie blanche. Int J Dermatol 1990; 29:663.
- 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.
- Klein KL, Pittelkow MR. Tissue plasminogen activator for treatment of livedoid vasculitis. Mayo Clin Proc 1992; 67:923.
- 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.
- CLINICAL FEATURES
- Livedoid changes
- Atrophie blanche
- DISEASE ASSOCIATIONS
- ASSOCIATED HYPERCOAGULABLE STATES
- DIFFERENTIAL DIAGNOSIS
- DIAGNOSIS AND EVALUATION
- Excluding connective tissue diseases
- Excluding known hypercoagulability risk factors
- General measures
- Pharmacologic therapy
- - Our approach
- First-line therapy
- Second-line therapy
- Other interventions
- SUMMARY AND RECOMMENDATIONS