Livedoid Vasculopathy Clinical Presentation
- Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: William D James, MD more...
In livedoid vasculopathy, the initial findings are typically painful purpuric macules or papules on the ankles and the adjacent dorsum of the feet. Patients may have a history of livedo reticularis on their lower legs. The history may help exclude other diagnostic considerations.
Medium-sized vasculitides, such as polyarteritis nodosa (PAN), occasionally present with ulceration, resulting in ivory-white, stellate scarring on the lower limbs. These conditions may potentially be misdiagnosed as livedoid vasculopathy.
Livedoid vasculopathy is not associated with edema or venous insufficiency, whereas stasis dermatitis with ulceration is usually not painful and is associated with obvious edema and signs of venous insufficiency.
Patients with livedoid vasculopathy may have a history of recurrent leg ulcerations. Such patients can have deficiencies in a variety of blood factors (eg, factor V Leiden, protein C). The factor V Leiden mutation is more frequent in patients with venous leg ulceration than in control subjects and the general population. Patients with the factor V Leiden mutation have an increased risk of developing deep venous thrombosis and recurrent leg ulceration. Patients may also have a history of increased plasma homocysteine levels, abnormalities in fibrinolysis, and increased platelet activation.
Livedoid vasculopathy is not particularly painful, whereas hypertensive ischemic ulcers are painful but are usually larger and lack telangiectatic purple borders.
History and careful follow-up care can rule out traumatic ulcers and distinguish them from livedoid vasculopathy.
Chronic periarteritis nodosa may be associated with painful ulcerations; however, the associated nodules should differentiate chronic periarteritis nodosa from livedoid vasculopathy.
In 2003, Marzano et al described a 37-year-old woman with a 13-year history of widespread livedo reticularis and recurrent, painful, ulcerative skin lesions. The recurrent ulcerations involved almost the entire body surface. In addition, malar erythema and edema, nonscarring alopecia, and fever were also associated with this patient's condition. Routine laboratory data, immunological investigations, and coagulation parameters were normal or negative.
In 2007, Cardoso et al noted livedoid vasculopathy and hypercoagulability in a patient with primary Sjögren syndrome.
The range of presentation of livedoid vasculopathy is wide, and Okada et al reported widespread livedoid vasculopathy with pain but no systemic symptoms, showing that sometimes the disease is confined to the skin.
Livedoid vasculopathy in a woman with multiple myeloma has been reported.
Livedoid vasculopathy has a complex relationship with lupus.
The initial lesions of livedoid vasculopathy, which often appear in clusters or groups, eventually ulcerate over a period of months and years and form irregular patterns of superficial ulcers. When the ulcers finally heal, they leave behind atrophic porcelain-white scars, which are atrophie blanche.
Patients with livedoid vasculopathy can manifest with livedo reticularis before ulceration, with ulcerations, or with no ulcerations.
Patients with livedoid vasculopathy can have Raynaud phenomenon and acrocyanosis.
Lipodermatosclerosis can be associated with livedoid vasculopathy.
Patients who have systemic diseases, such as lupus, rheumatoid arthritis, and Klinefelter syndrome, that can result in skin ulcers can manifest with atrophie blanche–like lesions. These patients do not have livedoid vasculopathy.
Livedoid vasculopathy and recurrent thrombosis in a patient with lupus has been recorded. This case, the first reported of livedoid vasculopathy in a patient with seronegative antiphospholipid syndrome and systemic lupus erythematosus, draws attention to livedoid vasculopathy, a thrombotic dermopathy that may be under-diagnosed in patients with antiphospholipid syndrome.
The etiology of livedoid vasculopathy is unknown.
Jorizzo JL. Livedoid vasculopathy: what is it?. Arch Dermatol. 1998 Apr. 134(4):491-3. [Medline].
Alavi A, Hafner J, Dutz JP, Mayer D, Sibbald RG, Criado PR, et al. Livedoid vasculopathy: an in-depth analysis using a modified Delphi approach. J Am Acad Dermatol. 2013 Dec. 69(6):1033-1042. [Medline].
Feldaker M, Hines EA Jr, Kierland RR. Livedo reticularis with summer ulcerations. AMA Arch Derm. 1955 Jul. 72(1):31-42. [Medline].
Bard JW, Winkelmann RK. Livedo vasculitis. Segmental hyalinizing vasculitis of the dermis. Arch Dermatol. 1967 Nov. 96(5):489-99. [Medline].
Papi M, Didona B, De Pita O, et al. Livedo vasculopathy vs small vessel cutaneous vasculitis: cytokine and platelet P-selectin studies. Arch Dermatol. 1998 Apr. 134(4):447-52. [Medline].
Hairston BR, Davis MD, Pittelkow MR, Ahmed I. Livedoid vasculopathy: further evidence for procoagulant pathogenesis. Arch Dermatol. 2006 Nov. 142(11):1413-8. [Medline].
Yong AA, Tan AW, Giam YC, Tang MB. Livedoid vasculopathy and its association with factor V Leiden mutation. Singapore Med J. 2012 Dec;. 53:e258-60. [Medline].
Baccard M, Vignon-Pennamen MD, Janier M, Scrobohaci ML, Dubertret L. Livedo vasculitis with protein C system deficiency. Arch Dermatol. 1992 Oct. 128(10):1410-1. [Medline].
Deng A, Gocke CD, Hess J, Heyman M, Paltiel M, Gaspari A. Livedoid vasculopathy associated with plasminogen activator inhibitor-1 promoter homozygosity (4G/4G) treated successfully with tissue plasminogen activator. Arch Dermatol. 2006 Nov. 142(11):1466-9. [Medline].
Meiss F, Marsch WC, Fischer M. Livedoid vasculopathy. The role of hyperhomocysteinemia and its simple therapeutic consequences. Eur J Dermatol. 2006 Mar-Apr. 16(2):159-62. [Medline].
Maessen-Visch MB, Koedam MI, Hamulyák K, Neumann HA. Atrophie blanche. Int J Dermatol. 1999 Mar. 38(3):161-72. [Medline].
Browse NL, Burnand KG. The cause of venous ulceration. Lancet. 1982 Jul 31. 2(8292):243-5. [Medline].
Pizzo SV, Murray JC, Gonias SL. Atrophie blanche. A disorder associated with defective release of tissue plasminogen activator. Arch Pathol Lab Med. 1986 Jun. 110(6):517-9. [Medline].
Klein KL, Pittelkow MR. Tissue plasminogen activator for treatment of livedoid vasculitis. Mayo Clin Proc. 1992 Oct. 67(10):923-33. [Medline].
Drucker CR, Duncan WC. Antiplatelet therapy in atrophie blanche and livedo vasculitis. J Am Acad Dermatol. 1982 Sep. 7(3):359-63. [Medline].
Irani-Hakime NA, Stephan F, Kreidy R, Jureidini I, Almawi WY. Livedoid vasculopathy associated with combined prothrombin G20210A and factor V (Leiden) heterozygosity and MTHFR C677T homozygosity. J Thromb Thrombolysis. 2008 Aug. 26(1):31-4. [Medline].
Vasconcelos R, Criado PR, Belda W Jr. Livedoid vasculopathy secondary to high levels of lipoprotein(a). Br J Dermatol. 2011 May. 5:1111-3. [Medline].
Castillo-Martínez C, Moncada B, Valdés-Rodríguez R, González FJ. Livedoid vasculopathy (LV) associated with sticky platelets syndrome type 3 (SPS type 3) and enhanced activity of plasminogen activator inhibitor (PAI-1) anomalies. Int J Dermatol. 2014 Dec. 53(12):1495-7. [Medline].
Criado PR, Alavi A, Kirsner RS. Elevated Levels of Coagulation Factor VIII in Patients With Venous Leg Ulcers. Int J Low Extrem Wounds. 2014 May 25. 13(2):130-134. [Medline].
Feng S, Su W, Jin P, Shao C. Livedoid Vasculopathy: Clinical Features and Treatment in 24 Chinese Patients. Acta Derm Venereol. 94(5). 2014 Sep:574-8. [Medline].
Tubone MQ, Escobar GF, Peruzzo J, Schestatsky P, Maldonado G. Livedoid vasculopathy associated with peripheral neuropathy: a report of two cases. An Bras Dermatol. 88(6 Suppl 1). 2013 Nov-Dec:227-9. [Medline].
Sankar A, Hinshaw K. Livedoid vasculopathy and pregnancy. Int J Gynaecol Obstet. 2009. 107:248-9. [Medline].
Toth C, Trotter M, Clark A, Zochodne D. Mononeuropathy multiplex in association with livedoid vasculitis. Muscle Nerve. 2003 Nov. 28(5):634-9. [Medline].
Malaguti MC, Cavallaro T, Speziali L, Zorzi MG, Marangoni S, Morini A. Mononeuritis multiplex associated with primary livedoid vasculopathy: neuropathological evidence of ischemic nerve damage. J Neurol Sci. 2015 Apr 15. 351 (1-2):214-5. [Medline].
Marzano AV, Vanotti M, Alessi E. Widespread livedoid vasculopathy. Acta Derm Venereol. 2003. 83(6):457-60. [Medline].
Cardoso R, Goncalo M, Tellechea O, et al. Livedoid vasculopathy and hypercoagulability in a patient with primary Sjögren's syndrome. Int J Dermatol. 2007 Apr. 46(4):431-4. [Medline].
Okada E, Nagai Y, Ishikawa O. A case of widespread livedoid vasculopathy with pain but no systemic symptoms. Acta Derm Venereol. 2008. 88(3):298-9. [Medline].
Verma R, Hadid TH, Dhamija R, Moad JC, Loehrke ME. Livedoid vasculopathy in a woman with multiple myeloma. South Med J. 2010 Aug. 103(8):848-9. [Medline].
Sopena B, Perez-Rodriguez MT, Rivera A, Ortiz-Rey JA, Lamas J, Freire-Dapena MC. Livedoid vasculopathy and recurrent thrombosis in a patient with lupus: seronegative antiphospholipid syndrome?. Lupus. 2010 Jul 21. [Medline].
Shankar S, Vasudevan B, Deb P, Langer V, Verma R, Nair V. Livedoid vasculopathy: A vasculitic mimic. Arthritis Rheum. 2012 Nov 30. [Medline].
Anavekar NS, Kelly R. Heterozygous prothrombin gene mutation associated with livedoid vasculopathy. Australas J Dermatol. 2007 May. 48(2):120-3. [Medline].
Ackerman AB, Chongchitnant N, Sanchez J. Histologic Diagnosis of Inflammatory Skin Diseases: An Algorithmic Method Based on Pattern Analysis. 2nd ed. Baltimore, Md: Williams & Wilkins; 1997.
Criado PR, Di Giacomo TH, Souza DP, Santos DV, Aoki V. Direct immunofluorescence findings and thrombophilic factors in livedoid vasculopathy: how do they correlate?. Clin Exp Dermatol. 2014 Jan. 39(1):66-8. [Medline].
Sams WM Jr. Livedo vasculitis. Therapy with pentoxifylline. Arch Dermatol. 1988 May. 124(5):684-7. [Medline].
Hairston BR, Davis MD, Gibson LE, Drage LA. Treatment of livedoid vasculopathy with low-molecular-weight heparin: report of 2 cases. Arch Dermatol. 2003 Aug. 139(8):987-90. [Medline].
Yang CH, Ho HC, Chan YS, Liou LB, Hong HS, Yang LC. Intractable livedoid vasculopathy successfully treated with hyperbaric oxygen. Br J Dermatol. 2003 Sep. 149(3):647-52. [Medline].
Juan WH, Chan YS, Lee JC, Yang LC, Hong HS, Yang CH. Livedoid vasculopathy: long-term follow-up results following hyperbaric oxygen therapy. Br J Dermatol. 2006 Feb. 154(2):251-5. [Medline].
Kern AB. Atrophie blanche. Report of two patients treated with aspirin and dipyridamole. J Am Acad Dermatol. 1982 Jun. 6(6):1048-53. [Medline].
Purcell SM, Hayes TJ. Nifedipine treatment of idiopathic atrophie blanche. J Am Acad Dermatol. 1986 May. 14(5 Pt 1):851-4. [Medline].
Antunes J, Filipe P, André M, Fraga A, Miltenyi G, Marques Gomes M. 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. 90:91-2. [Medline].
Amital H, Levy Y, Shoenfeld Y. Use of intravenous immunoglobulin in livedo vasculitis. Clin Exp Rheumatol. 2000 May-Jun. 18(3):404-6. [Medline].
Browning CE, Callen JP. Warfarin therapy for livedoid vasculopathy associated with cryofibrinogenemia and hyperhomocysteinemia. Arch Dermatol. 2006 Jan. 142(1):75-8. [Medline].
Kavala M, Kocaturk E, Zindanci I, Turkoglu Z, Altintas S. A case of livedoid vasculopathy associated with factor V Leiden mutation: successful treatment with oral warfarin. J Dermatolog Treat. 2008. 19(2):121-3. [Medline].
Davis MD, Wysokinski WE. Ulcerations caused by livedoid vasculopathy associated with a prothrombotic state: Response to warfarin. J Am Acad Dermatol. 2008 Mar. 58(3):512-5. [Medline].
Heine KG, Davis GW. Idiopathic atrophie blanche: treatment with low-dose heparin. Arch Dermatol. 1986 Aug. 122(8):855-6. [Medline].
Kim EJ, Yoon SY, Park HS, Yoon HS, Cho S. Pulsed intravenous immunoglobulin therapy in refractory ulcerated livedoid vasculopathy: seven cases and a literature review. Dermatol Ther. 2015 Sep-Oct. 28 (5):287-90. [Medline].
Noda S, Asano Y, Yamazaki M, Ichimura Y, Tamaki Z, Takekoshi T, et al. Severe livedoid vasculopathy associated with antiphosphatidylserine-prothrombin complex antibody successfully treated with warfarin. Arch Dermatol. 2011 May. 5:621-3. [Medline].
Mofarrah R, Aberer W, Aberer E. Treatment of livedoid vasculopathy with alprostadil (PGE-1): case report and review of published literature. J Eur Acad Dermatol Venereol. 2012 Feb 25. [Medline].
Abou Rahal J, Ishak RS, Otrock ZK, Kibbi AG, Taher AT. Livedoid vasculopathy in a patient with lupus anticoagulant and MTHFR mutation: treatment with low-molecular-weight heparin. J Thromb Thrombolysis. 2012 May 17. [Medline].
Drabik A, Hillgruber C, Goerge T. A Phase II Multicenter Trial With Rivaroxaban in the Treatment of Livedoid Vasculopathy Assessing Pain on a Visual Analog Scale. JMIR Res Protoc. 2014 Dec 10. 3(4):e73. [Medline].
Winchester DS, Drage LA, Davis MD. Response of Livedoid Vasculopathy to Rivaroxaban. Br J Dermatol. 2014 Sep 25. [Medline].
Monshi B, Posch C, Vujic I, Sesti A, Sobotka S, Rappersberger K. Efficacy of intravenous immunoglobulins in livedoid vasculopathy:long-term follow-up of 11 patients. J Am Acad Dermatol 44. 2014 Oct. 71(4):738-44. [Medline].
Alix JJ, Hadjivassiliou M, Ali R, Slater D, Messenger AG, Rao DG. Sensory ganglionopathy with livedoid vasculopathy controlled by immunotherapy. Muscle Nerve. 2014 Sep 8. [Medline].
Callen JP. Livedoid vasculopathy: what it is and how the patient should be evaluated and treated. Arch Dermatol. 2006 Nov. 142(11):1481-2. [Medline].
Millan G. Les atrophies cutane'es syphilitques. Bull Soc Fr Derm Syph. 1929. 36:865-71.