- 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
Livedoid vasculopathy is a chronic, recurrent, painful skin disorder that involves the distal lower extremities and feet. The disorder is a thrombotic, noninflammatory 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, including systemic lupus erythematosus and the antiphospholipid syndrome. The disorder has also been linked to a growing list of clotting abnormalities. 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. The diagnosis and treatment of chronic venous insufficiency, as well as the diagnosis and treatment of small- and medium-vessel vasculitis of the skin, are discussed elsewhere. (See "Diagnostic evaluation of chronic venous insufficiency" and "Medical management of lower extremity chronic venous disease" and "Hypersensitivity vasculitis in adults" 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 lesions associated with this disorder, 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.
- Yang LJ, Chan HL, Chen SY, et al. Atrophie blanche. A clinicopathological study of 27 patients. Changgeng Yi Xue Za Zhi 1991; 14:237.
- Francès C, Barete S. Difficult management of livedoid vasculopathy. Arch Dermatol 2004; 140:1011.
- Drucker CR, Duncan WC. Antiplatelet therapy in atrophie blanche and livedo vasculitis. J Am Acad Dermatol 1982; 7:359.
- 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.
- Klein KL, Pittelkow MR. Tissue plasminogen activator for treatment of livedoid vasculitis. Mayo Clin Proc 1992; 67:923.
- Deng A, Gocke CD, Hess J, et al. Livedoid vasculopathy associated with plasminogen activator inhibitor-1 promoter homozygosity (4G/4G) treated successfully with tissue plasminogen activator. Arch Dermatol 2006; 142:1466.
- 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.
- Hsiao GH, Chiu HC. Low-dose danazol in the treatment of livedoid vasculitis. Dermatology 1997; 194:251.
- 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.
- Magy N, Algros MP, Racadot E, et al. [Liveoid vasculopathy with combined thrombophilia: efficacy of iloprost]. Rev Med Interne 2002; 23:554.
- Rustin MH, Bunker CB, Dowd PM. Chronic leg ulceration with livedoid vasculitis, and response to oral ketanserin. Br J Dermatol 1989; 120:101.
- 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.
- Winkelmann RK, Schroeter AL, Kierland RR, Ryan TM. Clinical studies of livedoid vasculitis: (segmental hyalinizing vasculitis). Mayo Clin Proc 1974; 49:746.
- CLINICAL FEATURES
- Livedoid changes
- Atrophie blanche
- DISEASE ASSOCIATIONS
- HYPERCOAGULABLE RISK FACTORS
- DIFFERENTIAL DIAGNOSIS
- Excluding connective tissue diseases
- Excluding known hypercoagulability risk factors
- General measures
- Address hypercoagulability risk factors
- Empiric therapies
- - Antiplatelet therapies
- - Anticoagulation
- - Fibrinolysis
- - Other treatments
- SUMMARY AND RECOMMENDATIONS