Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Livedoid Vasculopathy Treatment & Management

  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: William D James, MD  more...
 
Updated: Jan 25, 2016
 

Medical Care

While ruling out the various disease states that have been associated with livedoid vasculopathy, physicians can offer a number of therapies that have been very helpful in reducing pain and ulceration. Instituting treatment as soon as possible is best.

Pentoxifylline (Trental) (400 mg 3 times/d) may be effective. Pentoxifylline is believed to enhance the blood flow in the capillaries. The blood flow enhancement is attributed to making red blood cells more flexible and thereby reducing viscosity.[35]

In 2003, Hairston et al[36] described treatment of livedoid vasculopathy with low molecular weight heparin (LMWH).

As reported by Yang et al[37] in 2003, intractable livedoid vasculopathy was successfully treated with hyperbaric oxygen therapy. Additionally, Juan et al[38] reported a study of 12 subjects with active livedoid vasculopathy. Subjects received hyperbaric oxygen therapy 5 times/wk. Eight completed the study. Resumption of ambulation and reduction of analgesics were achieved after an average of 4.9 hyperbaric oxygen therapy sessions. Leg ulcers healed completely in these 8 subjects at a mean of 3.4 weeks (range, 2-5 wk). Six patients had relapses of ulceration and responded to additional hyperbaric oxygen therapy. No patients had adverse effects.

Also in 2003, Marzano et al[26] noted a good clinical response was obtained using intravenous methylprednisolone combined with pentoxifylline for wide spread livedoid vasculopathy.

Dipyridamole (Persantine) (75 mg 4 times/d) with up to 325 mg of aspirin per day is reported to reduce pain after 3-6 weeks of therapy. Similar results have been reported using 50 mg of dipyridamole 3 times a day and 325 mg of aspirin once a day.[39] Note that aspirin is not to be administered in conjunction with coumarin anticoagulants. Dipyridamole is not considered safe in children or breastfeeding mothers.

Nifedipine (Procardia) (20 mg 3 times/d) is reported to maintain perfusion in the superficial vessels; therefore, the deposition of fibrin in the vessel walls is impeded.[40]

Deng et al[9] noted that livedoid vasculopathy associated with plasminogen activator inhibitor-1 (PAI-1) promoter homozygosity (4G/4G) was effectively abated with tissue-type plasminogen activator (tPA). Antunes et al also reported on livedoid vasculopathy associated with PAI-1 promoter homozygosity (4G/4G) and prothrombin G20210A heterozygosity that responded to tPA treatment.[41]

Some reports have noted that intravenous immunoglobulin can be useful in treating atrophie blanche and livedoid vasculopathy, but this remains an experimental treatment.[42]

The combination of phenformin and ethylestrenol, which enhances endogenous blood fibrinolytic activity by increasing plasminogen activating enzymes, has been suggested as a treatment.

Browning and Callen[43] reported that warfarin is a useful and effective treatment for livedoid vasculopathy associated with cryofibrinogenemia and hyperhomocysteinemia. Kavala et al reported successful warfarin therapy in livedoid vasculopathy associated with factor V Leiden mutation.[44] Additionally, Davis and Wysokinski reported that livedoid vasculopathy associated with a prothrombotic state responded to warfarin.[45]

Some reports have noted the use of heparin,[46] LMWH, psoralen plus ultraviolet A (PUVA), and low molecular weight dextran.

If ulcers are superinfected, they should be treated with oral antibiotics.

Intravenous immunoglobulin (IVIG) has been reported as a last-resort treatment when other treatments have failed.[47]

Meiss et al[10] noted that hyperhomocysteinemia can be another cause of hypercoagulability and livedoid vasculopathy and that the combination of folic acid, vitamin B-12, and vitamin B-6 (cofactors of homocysteine metabolism) is an effective treatment for hyperhomocysteinemia, hypercoagulability, and livedoid vasculopathy.

Next

Consultations

To fully evaluate for the comorbid conditions of livedoid vasculopathy, consult a hematologist (to evaluate for factors that lead to hypercoagulable states) and vascular surgeons (to evaluate and treat underlying defects of coagulation).

Previous
 
 
Contributor Information and Disclosures
Author

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie<br/>Received income in an amount equal to or greater than $250 from: Optigenex<br/>Received salary from Optigenex for employment.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD Associate Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Timothy McCalmont, MD Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; Editor-in-Chief, Journal of Cutaneous Pathology

Timothy McCalmont, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society of Dermatopathology, California Medical Association, College of American Pathologists, United States and Canadian Academy of Pathology

Disclosure: Received consulting fee from Apsara for independent contractor.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Richard H. Musgnug, MD, to the development and writing of this article.

References
  1. Jorizzo JL. Livedoid vasculopathy: what is it?. Arch Dermatol. 1998 Apr. 134(4):491-3. [Medline].

  2. 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].

  3. Feldaker M, Hines EA Jr, Kierland RR. Livedo reticularis with summer ulcerations. AMA Arch Derm. 1955 Jul. 72(1):31-42. [Medline].

  4. Bard JW, Winkelmann RK. Livedo vasculitis. Segmental hyalinizing vasculitis of the dermis. Arch Dermatol. 1967 Nov. 96(5):489-99. [Medline].

  5. 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].

  6. Hairston BR, Davis MD, Pittelkow MR, Ahmed I. Livedoid vasculopathy: further evidence for procoagulant pathogenesis. Arch Dermatol. 2006 Nov. 142(11):1413-8. [Medline].

  7. 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].

  8. 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].

  9. 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].

  10. 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].

  11. Maessen-Visch MB, Koedam MI, Hamulyák K, Neumann HA. Atrophie blanche. Int J Dermatol. 1999 Mar. 38(3):161-72. [Medline].

  12. Browse NL, Burnand KG. The cause of venous ulceration. Lancet. 1982 Jul 31. 2(8292):243-5. [Medline].

  13. Coleridge Smith PD, Thomas P, Scurr JH, Dormandy JA. Causes of venous ulceration: a new hypothesis. Br Med J (Clin Res Ed). 1988 Jun 18. 296(6638):1726-7. [Medline]. [Full Text].

  14. 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].

  15. Klein KL, Pittelkow MR. Tissue plasminogen activator for treatment of livedoid vasculitis. Mayo Clin Proc. 1992 Oct. 67(10):923-33. [Medline].

  16. Drucker CR, Duncan WC. Antiplatelet therapy in atrophie blanche and livedo vasculitis. J Am Acad Dermatol. 1982 Sep. 7(3):359-63. [Medline].

  17. 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].

  18. 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].

  19. 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].

  20. 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].

  21. 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].

  22. 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].

  23. Sankar A, Hinshaw K. Livedoid vasculopathy and pregnancy. Int J Gynaecol Obstet. 2009. 107:248-9. [Medline].

  24. Toth C, Trotter M, Clark A, Zochodne D. Mononeuropathy multiplex in association with livedoid vasculitis. Muscle Nerve. 2003 Nov. 28(5):634-9. [Medline].

  25. 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].

  26. Marzano AV, Vanotti M, Alessi E. Widespread livedoid vasculopathy. Acta Derm Venereol. 2003. 83(6):457-60. [Medline].

  27. 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].

  28. 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].

  29. 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].

  30. 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].

  31. Shankar S, Vasudevan B, Deb P, Langer V, Verma R, Nair V. Livedoid vasculopathy: A vasculitic mimic. Arthritis Rheum. 2012 Nov 30. [Medline].

  32. Anavekar NS, Kelly R. Heterozygous prothrombin gene mutation associated with livedoid vasculopathy. Australas J Dermatol. 2007 May. 48(2):120-3. [Medline].

  33. 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.

  34. 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].

  35. Sams WM Jr. Livedo vasculitis. Therapy with pentoxifylline. Arch Dermatol. 1988 May. 124(5):684-7. [Medline].

  36. 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].

  37. 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].

  38. 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].

  39. Kern AB. Atrophie blanche. Report of two patients treated with aspirin and dipyridamole. J Am Acad Dermatol. 1982 Jun. 6(6):1048-53. [Medline].

  40. Purcell SM, Hayes TJ. Nifedipine treatment of idiopathic atrophie blanche. J Am Acad Dermatol. 1986 May. 14(5 Pt 1):851-4. [Medline].

  41. 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].

  42. Amital H, Levy Y, Shoenfeld Y. Use of intravenous immunoglobulin in livedo vasculitis. Clin Exp Rheumatol. 2000 May-Jun. 18(3):404-6. [Medline].

  43. Browning CE, Callen JP. Warfarin therapy for livedoid vasculopathy associated with cryofibrinogenemia and hyperhomocysteinemia. Arch Dermatol. 2006 Jan. 142(1):75-8. [Medline].

  44. 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].

  45. 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].

  46. Heine KG, Davis GW. Idiopathic atrophie blanche: treatment with low-dose heparin. Arch Dermatol. 1986 Aug. 122(8):855-6. [Medline].

  47. 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].

  48. 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].

  49. 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].

  50. 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].

  51. 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].

  52. Winchester DS, Drage LA, Davis MD. Response of Livedoid Vasculopathy to Rivaroxaban. Br J Dermatol. 2014 Sep 25. [Medline].

  53. 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].

  54. 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].

  55. 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].

  56. Millan G. Les atrophies cutane'es syphilitques. Bull Soc Fr Derm Syph. 1929. 36:865-71.

 
Previous
Next
 
Preferred sites of vascular involvement by selected vasculitides.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.