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Livedoid Vasculopathy: Clinical, Histopathological, and Therapy Evaluation

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Uncommon Ulcers of the Extremities

Abstract

Livedoid vasculopathy (LV), livedo vasculopathy is classified as a rare disease [1], with an estimated prevalence in general North American population is around 1 case per 100,000 inhabitants [2], and this disease is an orphan-condition (Orphanet classification for rare diseases ORPHA:542643) [3]. It is a chronic disorder that usually presents as recurrent reticulated purpura and/or livedo racemosa on the lower limbs, with recurrent, painful purpuric and/or necrotic macules that may lead to often ulcerative lesions, especially the ankles and dorsum of the feet [4]. The pain, functional disability, and cosmetic defects in these patients lead to severe impairment in quality of life [5].

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References

  1. Criado PR, Rivitti EA, Sotto MN, de Carvalho JF. Livedoid vasculopathy as a coagulation disorder. Autoimm Rev. 2011;10:33–60.

    Article  Google Scholar 

  2. Stevanovic DV. Atrophie blanche: a sign of dermal blood occlusion. Arch Dermatol. 1974;109:858–62.

    Article  CAS  PubMed  Google Scholar 

  3. The portal for rare diseases and orphan drugs. https://www.orpha.net/consor/cgi-bin/Disease_Classif.php?lng=EN&data_id=187&PatId=28055&search=Disease_Classif_Simple&new=1. Accessed 30 Dec 2019.

  4. Espinel DPGS, Di Giacomo TB, Pincelli TP, Pereira NV, Sotto MN, Kirsner RS, Criado PR. Analysis of serum levels and cutaneous expression of lipoprotein (a) in 38 patients with livedoid vasculopathy. J Cutan Pathol. 2017;44(12):1033–7.

    Article  PubMed  Google Scholar 

  5. Polo Gascón MR, de Carvalho JF, de Souza Espinel DP, Barros AM, Alavi A, Criado PR. Quality-of-life impairment in patients with livedoid vasculopathy. J Am Acad Dermatol. 2014;71(5):1024–6. https://doi.org/10.1016/j.jaad.2014.06.030.

    Article  PubMed  Google Scholar 

  6. McCalmont CS, McCalmont TH, Jorizzo JL, White WL, Leshin B, Rothberger H. Livedo vasculitis: vasculitis or thrombotic vasculopathy? Clin Exp Dermatol. 1992;17:4–8.

    Article  CAS  PubMed  Google Scholar 

  7. Flores G, Culton DA, Prisayanh P, Qaqish BF, James K, Maldonado M, Aoki V, Hans-Filho G, Rivitti EA, Diaz LA. IgG autoantibody response against keratinocyte cadherins in endemic pemphigus foliaceus (Fogo selvagem). J Invest Dermatol. 2012;132(11):2573–80. https://doi.org/10.1038/jid.2012.232; Epub 2012 Jul 19.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Suarez-Kurtz G, Paula DP, Struchiner CJ. Pharmacogenomic implications of population admixture: Brazil as a model case. Pharmacogenomics. 2014;15(2):209–19. https://doi.org/10.2217/pgs.13.238.

    Article  CAS  PubMed  Google Scholar 

  9. Bilgic A, Ozcobanoglu S, Bozca BC, Alpsoy E. Livedoid vasculopathy: a multidisciplinary clinical approach to diagnosis and management. Int J Womens Dermatol. 2021;7(5Part A):588–99.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Weishaupt C, Strölin A, Kahle B, Kreuter A, Schneider SW, Gerss J, Eveslage M, Drabik A, Goerge T. Characteristics, risk factors and treatment reality in livedoid vasculopathy—a multicentre analysis. J Eur Acad Dermatol Venereol. 2019;33(9):1784–91.

    Article  CAS  PubMed  Google Scholar 

  11. Gan EY, Tang MB, Tan SH, Chua SH, Tan AW. A ten-year retrospective study on livedo vasculopathy in Asian patients. Ann Acad Med Singap. 2012;41(9):400–6.

    Article  PubMed  Google Scholar 

  12. Feng S, Su W, Jin P, Shao C. Livedoid vasculopathy: clinical features and treatment in 24 Chinese patients. Acta Derm Venereol. 2014;94(5):574–8. https://doi.org/10.2340/00015555-1711.

    Article  CAS  PubMed  Google Scholar 

  13. Feng SY, Jin PY, Shao CG. The significance of anticardiolipin antibody and immunologic abnormality in livedoid vasculitis. Int J Dermatol. 2011;50(1):21–3. https://doi.org/10.1111/j.1365-4632.2010.04569.x.

    Article  PubMed  Google Scholar 

  14. Lee JS, Cho S. Livedoid vasculopathy in Koreans: clinical features and response to rivaroxaban treatment. J Eur Acad Dermatol Venereol. 2019;34:e176. https://doi.org/10.1111/jdv.16129; [Epub ahead of print].

    Article  Google Scholar 

  15. Tsai TF, Yang CH, Chu CY, Liou YH, Hsiao WC, Lin CT, Wu LS. Polymorphisms of MTHFR gene associated with livedoid vasculopathy in Taiwanese population. J Dermatol Sci. 2009;54(3):214–6. https://doi.org/10.1016/j.jdermsci.2008.

    Article  CAS  PubMed  Google Scholar 

  16. Gao Y, Jin H. Livedoid vasculopathy and peripheral neuropathy: a retrospective cohort study of 55 Chinese patients and literature review. Int Wound J. 2023;20(5):1498–505. https://doi.org/10.1111/iwj.14004.

    Article  PubMed  Google Scholar 

  17. Hairston BR, Davis MD, Pittelkow MR, Ahmed I. Livedoid vasculopathy: further evidence for procoagulant pathogenesis. Arch Dermatol. 2006;142(11):1413–8.

    Article  PubMed  Google Scholar 

  18. Gardette E, Moguelet P, Bouaziz JD, Lipsker D, Dereure O, Le Pelletier F, Lok C, Maisonobe T, Bessis D, Conard J, Francès C, Barete S. Livedoid vasculopathy: a French observational study including therapeutic options. Acta Derm Venereol. 2018;98(9):842–7.

    Article  CAS  PubMed  Google Scholar 

  19. Callegari-Jacques SM, Salzano FM. Brazilian Indian/non Indian interactions and their effects. Cienc Cult. 1999;51:166–74.

    Google Scholar 

  20. Reagin H, Marks E, Weis S, Susa J. Livedoid vasculopathy presenting in a patient with sickle cell disease. Am J Dermatopathol. 2018;40(9):682–5. https://doi.org/10.1097/DAD.0000000000001133.

    Article  PubMed  Google Scholar 

  21. Goerge T, Weishaupt C, Metze D, Nowak-Göttl U, Sunderkötter C, Steinhoff M, Schneider SW. Livedoid vasculopathy in a pediatric patient with elevated lipoprotein(a) levels: prompt response to continuous low-molecular-weight heparin. Arch Dermatol. 2010;146(8):927–8.

    Article  PubMed  Google Scholar 

  22. Weishaupt C, Strölin A, Kahle B, et al. Characteristics, risk factors and treatment reality in livedoid vasculopathy—a multicentre analysis. J Eur Acad Dermatol Venereol. 2019;33(9):1784–91. https://doi.org/10.1111/jdv.15639.

    Article  CAS  PubMed  Google Scholar 

  23. Algahtani FH, Stuckey R. High factor VIII levels and arterial thrombosis: illustrative case and literature review. Ther Adv Hematol. 2019;10:2040620719886685. https://doi.org/10.1177/2040620719886685.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Salvagno GL, Pavan C, Lippi G. Rare thrombophilic conditions. Ann Transl Med. 2018;6(17):342. https://doi.org/10.21037/atm.2018.08.12.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Riches K, Porter KE. Lipoprotein(a): cellular effects and molecular mechanisms. Cholesterol. 2012;2012:923289. https://doi.org/10.1155/2012/923289; Epub 2012 Sep 6. PMID: 22991657.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Criado PR, Espinell DP, Barreto P, Di Giacomo TH, Sotto MN. Lipoprotein(a) and livedoid vasculopathy: a new thrombophilic factor? Med Hypotheses. 2015;85(5):670–4. https://doi.org/10.1016/j.mehy.2015.08.009.

    Article  CAS  PubMed  Google Scholar 

  27. Criado PR, de Souza EspinelI DP, Valente NS, Alavi A, Kirsner RS. Livedoid vasculopathy and high levels of lipoprotein (a): response to danazol. Dermatol Ther. 2015;28(4):248–53. https://doi.org/10.1111/dth.12225.

    Article  PubMed  Google Scholar 

  28. Criado PR, Pagliari C, Morita TCAB, Marques GF, Pincelli TPH, Valente NYS, Garcia MSC, de Carvalho JF, Abdalla BMZ, Sotto MN. Livedoid vasculopathy in 75 Brazilian patients in a single-center institution: clinical, histopathological and therapy evaluation. Dermatol Ther. 2021;34(2):e14810. https://doi.org/10.1111/dth.14810.

    Article  PubMed  Google Scholar 

  29. Criado PR, Rivitti EA, Sotto MN, Valente NY, Aoki V, Carvalho JF, et al. Livedoid vasculopathy: an intriguing cutaneous disease. An Bras Dermatol. 2011;86(5):961–77.

    Article  PubMed  Google Scholar 

  30. Majmundar VD, Baxi K. Livedoid vasculopathy. In: StatPearls. Treasure Island, FL: StatPearls Publishing; 2021.

    Google Scholar 

  31. Callen JP. Livedoid vasculopathy: what it is and how the patient should be evaluated and treated. Arch Dermatol. 2006;142:1481–2.

    Article  PubMed  Google Scholar 

  32. Gao Y, Jin H. Rivaroxaban for treatment of livedoid vasculopathy: a systematic review. Dermatol Ther. 2021;34(5):e15051. https://doi.org/10.1111/dth.15051.

    Article  CAS  PubMed  Google Scholar 

  33. Gao Y, Jin H. Efficacy of an anti-TNF-alpha agent in refractory livedoid vasculopathy: a retrospective analysis. J Dermatolog Treat. 2020;9:1–6. https://doi.org/10.1080/09546634.2020.1737634.

    Article  CAS  Google Scholar 

  34. Song CH, Shin DS, Jang JW, Kim TL, Kim YG, Kim JS, Seo HM. A case of livedoid vasculopathy successfully treated with Sulodexide. Ann Dermatol. 2020;32(6):508–11. https://doi.org/10.5021/ad.2020.32.6.508.

    Article  PubMed  PubMed Central  Google Scholar 

  35. Provenza JR, Pedri LE, Provenza GM. Livedoid vasculopathy. Rev Bras Reumatol. 2016;S0482–5004(16):00027–9. https://doi.org/10.1016/j.rbr.2015.09.011; English, Portuguese.

    Article  Google Scholar 

  36. Alix JJ, Hadjivassiliou M, Ali R, Slater D, Messenger AG, Rao DG. Sensory ganglionopathy with livedoid vasculopathy controlled by immunotherapy. Muscle Nerve. 2015;51(2):296–301.

    Article  CAS  PubMed  Google Scholar 

  37. Mendiratta V, Malik M, Yadav P, Nangia A. Cilostazol: a novel agent in recalcitrant livedoid vasculopathy. Indian J Dermatol Venereol Leprol. 2016;82(2):222–4.

    Article  PubMed  Google Scholar 

  38. Eswaran H, Googe P, Vedak P, Marston WA, Moll S. Livedoid vasculopathy: a review with focus on terminology and pathogenesis. Vasc Med. 2022;27(6):593–603.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Franco Marques G, Criado PR, Alves Batista Morita TC, Cajas García MS. The management of livedoid vasculopathy focused on direct oral anticoagulants (DOACs): four case reports successfully treated with rivaroxaban. Int J Dermatol. 2018;57(6):732–41.

    Article  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Dermatology Department, Hospital das Clinicas da Faculdade de Medicina da, Universidade de Sao Paulo, Sao Paulo, Brazil

    Paulo Ricardo Criado

  2. Faculdade de Ciências Médicas de Santos (Fundação Lusíada—UNILUS), Santos, SP, Brazil

    Paulo Ricardo Criado

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  1. Paulo Ricardo Criado
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Editors and Affiliations

  1. Institute of Medical Sciences, Department of Surgery, Banaras Hindu University, Varanasi, India

    Ajay K. Khanna

  2. Institute of Medical Sciences, Department of General Surgery, Banaras Hindu University, Varanasi, India

    Satyendra Kumar Tiwary

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Criado, P.R. (2023). Livedoid Vasculopathy: Clinical, Histopathological, and Therapy Evaluation. In: Khanna, A.K., Tiwary, S.K. (eds) Uncommon Ulcers of the Extremities. Springer, Singapore. https://doi.org/10.1007/978-981-99-1782-2_5

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  • DOI: https://doi.org/10.1007/978-981-99-1782-2_5

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