Livedoid Vasculopathy Clinical Presentation

  • Author: Noah S Scheinfeld, MD, JD, FAAD; Chief Editor: William D James, MD   more...
 
Updated: Aug 1, 2011
 

History

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. Note the following:

  • 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, Toth et al[18] noted mononeuropathy multiplex in association with livedoid vasculitis.
  • In 2003, Marzano et al[19] 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[20] 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.[21]
  • Livedoid vasculopathy in a woman with multiple myeloma has been reported.[22]
  • Livedoid vasculopathy has a complex relationship with lupus.[23]
Next

Physical

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. Note the following:

  • 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.
Previous
Next

Causes

The etiology of livedoid vasculopathy is unknown.

Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

Noah S Scheinfeld, MD, JD, FAAD  Assistant Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, and New York Eye and Ear Infirmary; Private Practice

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

Disclosure: Optigenex Consulting fee Independent contractor

Specialty Editor Board

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, and United States and Canadian Academy of Pathology

Disclosure: Apsara Consulting fee Independent contractor

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

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

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD  Assistant 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, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Glen H Crawford, MD  Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

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

Disclosure: Elsevier Royalty Other

Acknowledgments

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. Apr 1998;134(4):491-3. [Medline].

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

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

  4. Papi M, Didona B, De Pita O, et al. Livedo vasculopathy vs small vessel cutaneous vasculitis: cytokine and platelet P-selectin studies. Arch Dermatol. Apr 1998;134(4):447-52. [Medline].

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

  6. Baccard M, Vignon-Pennamen MD, Janier M, Scrobohaci ML, Dubertret L. Livedo vasculitis with protein C system deficiency. Arch Dermatol. Oct 1992;128(10):1410-1. [Medline].

  7. 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. Nov 2006;142(11):1466-9. [Medline].

  8. Meiss F, Marsch WC, Fischer M. Livedoid vasculopathy. The role of hyperhomocysteinemia and its simple therapeutic consequences. Eur J Dermatol. Mar-Apr 2006;16(2):159-62. [Medline].

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

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

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

  12. Pizzo SV, Murray JC, Gonias SL. Atrophie blanche. A disorder associated with defective release of tissue plasminogen activator. Arch Pathol Lab Med. Jun 1986;110(6):517-9. [Medline].

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

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

  15. 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. Aug 2008;26(1):31-4. [Medline].

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

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

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

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

  20. Cardoso R, Goncalo M, Tellechea O, et al. Livedoid vasculopathy and hypercoagulability in a patient with primary Sjögren's syndrome. Int J Dermatol. Apr 2007;46(4):431-4. [Medline].

  21. 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].

  22. Verma R, Hadid TH, Dhamija R, Moad JC, Loehrke ME. Livedoid vasculopathy in a woman with multiple myeloma. South Med J. Aug 2010;103(8):848-9. [Medline].

  23. 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. Jul 21 2010;[Medline].

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

  25. 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.

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

  27. Hairston BR, Davis MD, Gibson LE, Drage LA. Treatment of livedoid vasculopathy with low-molecular-weight heparin: report of 2 cases. Arch Dermatol. Aug 2003;139(8):987-90. [Medline].

  28. Yang CH, Ho HC, Chan YS, Liou LB, Hong HS, Yang LC. Intractable livedoid vasculopathy successfully treated with hyperbaric oxygen. Br J Dermatol. Sep 2003;149(3):647-52. [Medline].

  29. 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. Feb 2006;154(2):251-5. [Medline].

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

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

  32. 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].

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

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

  35. 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].

  36. Davis MD, Wysokinski WE. Ulcerations caused by livedoid vasculopathy associated with a prothrombotic state: Response to warfarin. J Am Acad Dermatol. Mar 2008;58(3):512-5. [Medline].

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

  38. 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. May 2011;5:621-3. [Medline].

  39. Bisalbutra P, Kullavanijaya P. Sulfasalazine in atrophie blanche. J Am Acad Dermatol. Feb 1993;28(2 Pt 1):275-6. [Medline].

  40. Bollinger A. [White atrophy: skin infarction of various pathogeneses?]. Vasa. 1981;10(1):67-9. [Medline].

  41. Bollinger A, Leu AJ. Evidence for microvascular thrombosis obtained by intravital fluorescence videomicroscopy. Vasa. 1991;20(3):252-5. [Medline].

  42. Bugatti L, Filosa G, Nicolini M, Filosa A, Verdolini R. Atrophie blanche associated with interferon-alfa adjuvant therapy for melanoma: a cutaneous side effect related to the procoagulant activity of interferon?. Dermatology. 2002;204(2):154. [Medline].

  43. Callen JP. Livedoid vasculopathy: what it is and how the patient should be evaluated and treated. Arch Dermatol. Nov 2006;142(11):1481-2. [Medline].

  44. Frances C, Barete S. Difficult management of livedoid vasculopathy. Arch Dermatol. Aug 2004;140(8):1011. [Medline].

  45. Grattan CE, Burton JL. Antiphospholipid syndrome and cutaneous vasoocclusive disorders. Semin Dermatol. Sep 1991;10(3):152-9. [Medline].

  46. Kluken N, Zabel M. [Pathologic anatomy of white atrophy]. Phlebologie. Jul-Sep 1986;39(3):561-6. [Medline].

  47. Krasner D. Painful venous ulcers: themes and stories about their impact on quality of life. Ostomy Wound Manage. Sep 1998;44(9):38-42, 44, 46 passim. [Medline].

  48. Leonard A, Pomeranz MK, Franks AG Jr. A case of livedoid vasculopathy in a 22-year-old man. J Drugs Dermatol. Nov-Dec 2004;3(6):678-9. [Medline].

  49. Lewerenz V, Burchardt T, Büchau A, Ruzicka T, Megahed M. [Livedoid vasculopathy with heterozygous factor V Leiden mutation and sticky platelet syndrome]. Hautarzt. Apr 2004;55(4):379-81. [Medline].

  50. McCalmont CS, McCalmont TH, Jorizzo JL, White WL, Leshin B, Rothberger H. Livedo vasculitis: vasculitis or thrombotic vasculopathy?. Clin Exp Dermatol. Jan 1992;17(1):4-8. [Medline].

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

  52. Milstone LM, Braverman IM. PURPLE (oops! Atrophie blanche) revisited. Arch Dermatol. Dec 1998;134(12):1634. [Medline].

  53. Pascarella L, Bergan JJ, Mekenas LV. Severe chronic venous insufficiency treated by foamed sclerosant. Ann Vasc Surg. Jan 2006;20(1):83-91. [Medline].

  54. Phillips T, Stanton B, Provan A, Lew R. A study of the impact of leg ulcers on quality of life: financial, social, and psychologic implications. J Am Acad Dermatol. Jul 1994;31(1):49-53. [Medline].

  55. Schroeter AL, Diaz-Perez JL, Winkelmann RK, Jordan RE. Livedo vasculitis (the vasculitis of atrophie blanche). Immunohistopathologic study. Arch Dermatol. Feb 1975;111(2):188-93. [Medline].

  56. Shornick JK, Nicholes BK, Bergstresser PR, Gilliam JN. Idiopathic atrophie blanche. J Am Acad Dermatol. Jun 1983;8(6):792-8. [Medline].

  57. Suarez SM, Paller AS. Atrophie blanche with onset in childhood. J Pediatr. Nov 1993;123(5):753-5. [Medline].

  58. Suster S, Ronnen M, Bubis JJ, Schewach-Millet M. Familial atrophie blanche-like lesions with subcutaneous fibrinoid vasculitis. The Georgian ulcers. Am J Dermatopathol. Oct 1986;8(5):386-91. [Medline].

  59. Valencia IC, Falabella A, Kirsner RS, Eaglstein WH. Chronic venous insufficiency and venous leg ulceration. J Am Acad Dermatol. Mar 2001;44(3):401-21; quiz 422-4. [Medline].

  60. Vowden K. Lipodermatosclerosis and atrophie blanche. J Wound Care. Oct 1998;7(9):441-3. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.