Livedoid Vasculopathy Questions & Answers

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2. Vasudevan B, Neema S, Verma R. Livedoid vasculopathy: A review of pathogenesis and principles of management. Indian J Dermatol Venereol Leprol. 2016 Sep-Oct. 82 (5):478-88. [QxMD MEDLINE Link].

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

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

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. [QxMD MEDLINE Link].

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

7. Micieli R, Alavi A. Treatment for Livedoid Vasculopathy: A Systematic Review. JAMA Dermatol. 2018 Feb 1. 154 (2):193-202. [QxMD MEDLINE Link].

8. 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. [QxMD MEDLINE Link].

9. 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. [QxMD MEDLINE Link].

10. 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. [QxMD MEDLINE Link].

11. 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. [QxMD MEDLINE Link].

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

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

14. 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. [QxMD MEDLINE Link]. [Full Text].

15. 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. [QxMD MEDLINE Link].

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

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

18. 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. [QxMD MEDLINE Link].

19. Yang CH, Shen SC, Hui RC, Huang YH, Chu PH, Ho WJ. Association between peripheral vascular endothelial dysfunction and livedoid vasculopathy. J Am Acad Dermatol. 2012 Jul. 67 (1):107-12. [QxMD MEDLINE Link].

20. Vasconcelos R, Criado PR, Belda W Jr. Livedoid vasculopathy secondary to high levels of lipoprotein(a). Br J Dermatol. 2011 May. 5:1111-3. [QxMD MEDLINE Link].

21. 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. [QxMD MEDLINE Link].

22. 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. [QxMD MEDLINE Link].

23. 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. [QxMD MEDLINE Link].

24. 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. [QxMD MEDLINE Link].

25. Criado PR, Rivitti EA, Sotto MN, de Carvalho JF. Livedoid vasculopathy as a coagulation disorder. Autoimmun Rev. 2011 Apr. 10 (6):353-60. [QxMD MEDLINE Link].

26. Sankar A, Hinshaw K. Livedoid vasculopathy and pregnancy. Int J Gynaecol Obstet. 2009. 107:248-9. [QxMD MEDLINE Link].

27. Kerk N, Goerge T. Livedoid vasculopathy - current aspects of diagnosis and treatment of cutaneous infarction. J Dtsch Dermatol Ges. 2013 May. 11 (5):407-10. [QxMD MEDLINE Link].

28. Sajjan VV, Lunge S, Swamy MB, Pandit AM. Livedo reticularis: A review of the literature. Indian Dermatol Online J. 2015 Sep-Oct. 6 (5):315-21. [QxMD MEDLINE Link].

29. Weishaupt C, Strölin A, Kahle B, Kreuter A, Schneider SW, Gerss J, et al. Characteristics, risk factors and treatment reality in livedoid vasculopathy - a multicentre analysis. J Eur Acad Dermatol Venereol. 2019 Sep. 33 (9):1784-1791. [QxMD MEDLINE Link].

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

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

32. 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. [QxMD MEDLINE Link].

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

34. 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. [QxMD MEDLINE Link].

35. 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. [QxMD MEDLINE Link].

36. 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. [QxMD MEDLINE Link].

37. 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. [QxMD MEDLINE Link].

38. Reagin H, Marks E, Weis S, Susa J. Livedoid Vasculopathy Presenting in a Patient With Sickle Cell Disease. Am J Dermatopathol. 2018 Sep. 40 (9):682-685. [QxMD MEDLINE Link].

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

40. Criado PR, Rivitti EA, Sotto MN, Valente NY, Aoki V, Carvalho JF, et al. Livedoid vasculopathy: an intringuing cutaneous disease. An Bras Dermatol. 2011 Sep-Oct. 86 (5):961-77. [QxMD MEDLINE Link].

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

42. 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.

43. 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. [QxMD MEDLINE Link].

44. Sams WM Jr. Livedo vasculitis. Therapy with pentoxifylline. Arch Dermatol. 1988 May. 124(5):684-7. [QxMD MEDLINE Link].

45. 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. [QxMD MEDLINE Link].

46. 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. [QxMD MEDLINE Link].

47. 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. [QxMD MEDLINE Link].

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

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

50. 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. [QxMD MEDLINE Link].

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

52. Bounfour T, Bouaziz JD, Bézier M, Petit A, Viguier M, Rybojad M, et al. Intravenous immunoglobulins in difficult-to-treat ulcerated livedoid vasculopathy: five cases and a literature review. Int J Dermatol. 2013 Sep. 52 (9):1135-9. [QxMD MEDLINE Link].

53. Yoshioka K, Tateishi C, Kato H, Chen KR. Systemic lupus erythematosus with refractory ulcerated livedoid vasculopathy: Successful treatment with intravenous immunoglobulin and warfarin. Clin Case Rep. 2018 Nov. 6 (11):2045-2047. [QxMD MEDLINE Link].

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

55. 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. [QxMD MEDLINE Link].

56. 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. [QxMD MEDLINE Link].

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

58. Lee JH, Choi HJ, Kim SM, Hann SK, Park YK. Livedoid vasculitis responding to PUVA therapy. Int J Dermatol. 2001 Feb. 40 (2):153-7. [QxMD MEDLINE Link].

59. Marsch WC, Komatsuzaki S, Mueller A, Hagemann M, Lange D, Maemecke L, et al. Livedoid vasculopathy: does hyperhomocysteinaemia play an aetiological role?. Eur J Dermatol. 2019 Jun 1. 29 (3):287-293. [QxMD MEDLINE Link].

60. Gardette E, Moguelet P, Bouaziz JD, Lipsker D, Dereure O, Le Pelletier F, et al. Livedoid Vasculopathy: A French Observational Study Including Therapeutic Options. Acta Derm Venereol. 2018 May 8. [QxMD MEDLINE Link].

61. 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. [QxMD MEDLINE Link].

62. 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. [QxMD MEDLINE Link].

63. 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. [QxMD MEDLINE Link].

64. Weishaupt C, Strölin A, Kahle B, Kreuter A, Schneider SW, Gerss J, et al. Anticoagulation with rivaroxaban for livedoid vasculopathy (RILIVA): a multicentre, single-arm, open-label, phase 2a, proof-of-concept trial. Lancet Haematol. 2016 Feb. 3 (2):e72-9. [QxMD MEDLINE Link].

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Fnu Nutan, MD, FACP Assistant Professor, Department of Dermatology, Virginia Commonwealth University School of Medicine

Fnu Nutan, MD, FACP is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, Indian Association of Dermatologists, Venereologists and Leprologists, Society of General Internal Medicine, Society of Hospital Medicine

Disclosure: Nothing to disclose.

Jaya Manjunath St Bonaventure University

Disclosure: Nothing to disclose.

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

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.

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: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

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.

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: Nothing to disclose.

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.

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