Acta Derm Venereol 2018 May 8. Epub 2018 May 8.

Department of Dermatology, Hôpital Tenon, APHP, Paris, France.

Livedoid vasculopathy is a rare thrombotic cutaneous disease. This observational study aimed to assess the clinical and biological features of livedoid vasculopathy and the efficacy of treatments. Patients enrolled had typical livedoid vasculopathy both clinically and histologically. Read More

Int J Dermatol 2018 Jun 16;57(6):732-741. Epub 2018 Apr 16.

Centro da Pele Ecuador - CEPI, Quito, Ecuador.

Livedoid vasculopathy (LV) is a thrombotic skin disease characterized by episodic painful ulcerations of the distal aspects of the legs. Its healing process typically leaves small porcelain-white scars called atrophie blanche as a result of the occlusion of cutaneous microcirculation. The main goals of the treatment are pain management and the prevention of ulceration and of progressive scarring in the malleolar area. Read More

Am J Dermatopathol 2018 Feb 28. Epub 2018 Feb 28.

Division of Dermatopathology, Cockerell Dermatopathology, Dallas, TX.

We report a case in which a 43-year-old African American male with medical history of sickle cell disease (SCD) presented with a nonhealing ulcer. Biopsy revealed features of livedoid vasculopathy. Previously, livedoid vasculopathy had only been described in a patient with sickle cell trait, but never in a patient with SCD. Read More

Reumatol Clin 2018 Mar 8. Epub 2018 Mar 8.

Servicio de Dermatología, Hospital Universitario Doctor Peset, Valencia, España.

JAMA Dermatol 2018 Feb;154(2):193-202

Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.

Importance: Livedoid vasculopathy is a painful, ulcerative condition of the lower extremities for which no established treatment exists. Current treatment paradigms rely on low levels of evidence, primarily case reports and case series.

Objective: To systematically review the treatment for livedoid vasculopathy and synthesize the available clinical data. Read More

Actas Dermosifiliogr 2018 Apr 23;109(3):278-281. Epub 2017 Oct 23.

Unidad de Gestión Clínica de Dermatología Médico-Quirúrgica y Venereología, Hospital Universitario Puerta del Mar, Cádiz, España.

Indian J Dermatol 2017 Sep-Oct;62(5):508-511

Department of Dermatology, Venereology and Leprosy, Jawaharlal Nehru Medical College and AVBR Hospital, Wardha, Maharashtra, India.

We hereby present our experience of low-dose oral warfarin in the management of three cases of livedoid vasculopathy. Read More

J Cutan Pathol 2017 Dec 13;44(12):1033-1037. Epub 2017 Oct 13.

Dermatology Department of Hospital das Clínicas, University of Sao Paulo Medical School, São Paulo, Brazil.

Background: Coagulation disorders contribute to the development of livedoid vasculopathy (LV). Elevated plasma levels of lipoprotein(a) [Lp(a)] are an independent risk factor for the development of cardiovascular disease and associated with hypercoagulable states. Increased serum Lp(a) levels have been reported in patients with LV and may have an important role in the pathogenesis of LV. Read More

G Ital Dermatol Venereol 2017 Dec 28;152(6):615-637. Epub 2017 Jul 28.

Department of Dermatology, de La Princesa University Hospital, Madrid, Spain.

Most emergencies in dermatology comprise a variety of entities with a usually benign course. However, vasculopathies and vasculitis are not common, but they could represent respectively 1.9% and 4. Read More

Am J Dermatopathol 2018 Mar;40(3):212-215

Department of Pathology, Western General Hospital and the University of Edinburgh, Edinburgh, United Kingdom.

Nicolau syndrome is a rare form of iatrogenic cutaneous necrosis which affects injection sites. Although classically associated with intramuscular injections, it may develop after subcutaneous or other routes of parenteral drug administration. Clinically, it manifests as necrotic ulcers that often develop in a background of erythematous and livedoid reticular patches. Read More

Australas J Dermatol 2017 Jul 28. Epub 2017 Jul 28.

Department of Anatomical Pathology, St Vincent's Hospital Melbourne, Melbourne, Victoria, Australia.

We describe three patients who presented with a striking erythematous non-blanching annular eruption and features of lymphocytic thrombophilic arteritis (LTA), with a prominent lymphocytic vasculitis involving deep dermal vessels. Lymphocytic inflammation was also evident in the superficial vessels and one patient had small superficial ulcers over the ankle area resembling livedoid vasculopathy (LV). Multiple biopsies demonstrated a persistent absence of neutrophils in the infiltrate consistent with a lymphocytic process. Read More

BMJ Case Rep 2017 Jul 24;2017. Epub 2017 Jul 24.

Department of Dermatology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama, Japan.

Livedoid vasculopathy (LV) is a thrombotic skin disease characterised by recurrent painful ulcerations and irreversible scar formation on the lower legs, which is caused by occlusion of the cutaneous microcirculation. Edoxaban is one of new oral anticoagulants. It directly inhibits factor Xa in the coagulation pathway and prevents thrombus formation. Read More

J Eur Acad Dermatol Venereol 2017 Dec 12;31(12):e532. Epub 2017 Jul 12.

Universitaetsklinikum Münster, Muenster, Germany.

Arthritis Rheumatol 2017 Oct 15;69(10):2096. Epub 2017 Aug 15.

Institute of Autoimmune Systemic and Neurological Diseases and National and Kapodistrian University of Athens, Athens, Greece.

J Pain Res 2017 17;10:621-624. Epub 2017 Mar 17.

Department of Rheumatology, Chinese People's Liberation Army General Hospital, Beijing, People's Republic of China.

Livedoid vasculopathy (LV) is a chronic prothrombotic disease of cutaneous micro-circulation resulting in cutaneous ischemia and infarction. As a rare disease, LV has an estimated incidence of ten cases per million. Not only correct diagnosis but also effective treatments are very difficult for patients with LV. Read More

Medicine (Baltimore) 2017 Mar;96(11):e6284

aDepartment of Dermatology, College of Medicine, Kaohsiung Medical University bDepartment of Dermatology, Kaohsiung Medical University Hospital cDepartment of Dermatology, Kaohsiung Municipal Hsiao-Kang Hospital, Kaohsiung dDepartment of Otolaryngology, Chi Mei Medical Center eCenter of General education, Southern Taiwan University of Technology, Tainan City fDepartment of Otolaryngology, School of Medicine, Taipei Medical University, Taipei, Taiwan.

Livedoid vasculopathy (atrophie blanche) is a form of thrombotic vasculopathy. It is characterized by small ulcers that become crusted, and heal after several months to produce white atrophic scars. The most commonly affected sites are the lower legs, in particular the dorsum of the feet and ankles. Read More

Ann Dermatol Venereol 2017 May 7;144(5):374-377. Epub 2017 Mar 7.

Service de dermatologie, HIA Legouest et CHR Metz-Thionville, 57000 Metz, France.

Background: Livedoid vasculopathy (LV) is a painful ulcerative condition involving white atrophy and livedo; a histopathologic feature seen is occlusive dermal vasculopathy. This may be associated with coagulation disorders such as hyperhomocysteinaemia (HHC).

Patients And Methods: We report the case of a 52-year-old woman presenting LV in which an abnormal scan image led us to diagnose coeliac disease. Read More

J Eur Acad Dermatol Venereol 2017 Nov 29;31(11):1884-1889. Epub 2017 Mar 29.

Department of Dermatology, Universitätsklinikum Erlangen, Erlangen, Germany.

Background: Livedoid vasculopathy and calciphylaxis are rare skin disorders. Large cohorts of patients have been missing so far for detailed analysis.

Patients And Methods: Data from diagnosis-related groups (DRGs) of hospitalized cases of livedoid vasculopathy (n = 1357) and calciphylaxis (n = 699) were analysed for the years 2008-2013 concerning sex, age and frequency of diagnosis. Read More

Ann Dermatol 2017 Feb 3;29(1):125-127. Epub 2017 Feb 3.

Rheumatology Service, Hospital Nossa Senhora da ConceiçÃo-Grupo Hospitalar ConceiçÃo, Porto Alegre, Brazil.

J Vasc Surg Venous Lymphat Disord 2017 Mar 14;5(2):280-292. Epub 2016 Dec 14.

Division of Rheumatology, The George Washington University, School of Medicine and Health Sciences, Washington, D.C.

Objective: Chronic wounds are a major cause of morbidity and mortality. Approximately 20% to 23% of nonhealing wounds that are refractory to vascular intervention have other causes, including vasculitis, pyoderma gangrenosum, and other autoimmune diseases. The purpose of this article was to review the literature across medical and surgical specialties with regard to refractory chronic wounds associated with vasculitis and autoimmune diseases and to delineate clinical outcomes of these wounds in response to vascular and other interventions. Read More

Cardiovasc Ther 2017 Jun;35(3)

Department of Medical Biology, Faculty of Medicine, Marmara University, Istanbul, Turkey.

Aim: Thrombosis and inflammation play an important role in pathophysiology of livedoid vasculopathy (LV). Plasminogen activator inhibitor-1 (PAI-1) is the main physiological inhibitor of fibrinolysis and is a pivotal modulator in a broad range of biological processes.

Method: The study specimens were retrospectively selected from archives of pathology department. Read More

Clin Exp Dermatol 2017 Mar 4;42(2):237-239. Epub 2017 Jan 4.

Department of Dermatology, Otsu Municipal Hospital, Otsu, Japan.

Dermatol Surg 2017 06;43(6):892-894

Department of Dermatology, University of Texas McGovern Medical School at Houston, Houston, Texas.

Rev Bras Reumatol Engl Ed 2016 Nov - Dec;56(6):554-556. Epub 2016 Mar 19.

Pontifícia Universidade Católica de Campinas, Hospital e Maternidade Celso Pierro, Serviço de Radiologia, Campinas, SP, Brazil.

Ann Dermatol Venereol 2016 Dec;143 Suppl 3:S1-S10

Service de Dermatologie, CHU Amiens Picardie, EA 4666, Université Picardie Jules Verne. Electronic address:

Zika virus: what the dermatologist should know. Probably a new vaccine against herpes zoster and postherpetic neuralgia in older adults. Defining moderate, significant and extensive types of pemphigus with ABSIS et PDAI scores. Read More

Acta Med Okayama 2016 Oct;70(5):377-381

Department of Dermatology, Okayama City Hospital, Okayama 700-8557,

A 71-year-old man presented with a high fever, polyarthralgia, petechiae and palpable purpura accompanied by livedoid change on his legs and feet. Histopathological findings of the purpura revealed perivascular infiltration of neutrophils, mononuclear cells, and nuclear debris, and extravasation of red cells mainly in the upper dermis: all signs consistent with leukocytoclastic vasculitis. Small vessel thrombi, which are characteristic features of septic vasculopathy, were also observed. Read More

Acta Reumatol Port 2016 Jul-Sep;41(3):273-274

We present a rare association of idiopathic livedoid vasculopathy (LV) with peripheral sensory neuropathy successfully treated with intravenous immunoglobulin and warfarin and, after its failure, with rituximab. Although LV aetiology remains incompletely understood, the excellent clinical response observed in our patient suggests that B cells may be key players in its pathogenesis. Read More

J Dermatol 2017 Feb 24;44(2):198-201. Epub 2016 Oct 24.

Department of Dermatology, Aichi Medical University, Nagakute, Japan.

Livedoid vasculopathy (LV) is a chronic disease with recurrent reticularis and ulcers, mainly affecting the feet and lower legs. The pathogenesis of LV has not been yet thoroughly understood, but thrombosis is thought to play a major role because fibrin deposition within both the wall and lumen of affected vessels is pathologically detected. A 68-year-old woman first presented to our hospital in 2004 with a 6-year history of a reticular rash and ulceration on the lower legs. Read More

Am J Dermatopathol 2017 Sep;39(9):637-662

*Associate Staff of the Department of Dermatology, Department of Dermatology, Hospital Universitario de la Princesa, Universidad Autónoma, Madrid, Spain; †Associate Staff of the Department of Dermatology, Department of Dermatology, Fundación Jiménez Diaz, Universidad Autónoma, Madrid, Spain; ‡Director of Dermatopathology, Department of Dermatology, Hospital Clínico Universitario, Salamanca, Spain; §Director of Dermatopathology, Dermatopathology Research Unit, Medical University of Graz, Graz, Austria; ¶Director of Dermatopathology, Dermatopathologie Laboratory, Friedrichschafen, Germany; and ‖Chairman of the Department of Dermatology, Department of Dermatology, Hospital Clínico Universitario, Salamanca, Spain.

We review the most characteristic clinical and histopathologic findings of the cutaneous manifestations of the occlusive nonvasculitic vasculopathic disorders. Clinically, most of these conditions are characterized by retiform purpura. Histopathologic findings consist of occlusion of the vessel lumina with no vasculitis. Read More

Pediatr Rheumatol Online J 2016 Sep 8;14(1):51. Epub 2016 Sep 8.

UO Pediatria II, Istituto G. Gaslini, Genoa, Italy.

The deficiency of Adenosine Deaminase 2 (DADA2) is a new autoinflammatory disease characterised by an early onset vasculopathy with livedoid skin rash associated with systemic manifestations, CNS involvement and mild immunodeficiency.This condition is secondary to autosomal recessive mutations of CECR1 (Cat Eye Syndrome Chromosome Region 1) gene, mapped to chromosome 22q11.1, that encodes for the enzymatic protein adenosine deaminase 2 (ADA2). Read More

Medicine (Baltimore) 2016 Jul;95(29):e4212

aDepartment of Dermatology bDepartment of Vascular Surgery cDepartment of Pathology dDepartment of Infectious Diseases, Hospital del Mar, Parc de Salut Mar, Universitat Autònoma de Barcelona, Barcelona, Spain.

Background: Diffuse dermal angiomatosis (DDA) is a rare, acquired, reactive vascular proliferation, clinically characterized by livedoid erythematous-violaceous plaques, which frequently evolve to ulceration and necrosis. Histopathologically, it is manifested by a diffuse proliferation of endothelial cells within the full thickness of the dermis. DDA has been mainly associated with severe peripheral atherosclerosis. Read More

Indian J Dermatol Venereol Leprol 2016 Nov-Dec;82(6):626-640

Department of Dermatology, Venereology and Leprosy, Shri B. M. Patil Medical College, Hospital and Research Center, BLDE University, Vijayapur, Karnataka, India.

Anticoagulants are the cornerstone of treatment of venous thromboembolism associated with various medical conditions and surgical procedures. They act on different steps of the coagulation pathway and are broadly categorized into heparins, vitamin K antagonists, and inhibitors of thrombin and factor Xa. The classification is evolving as newer and better oral and parenteral anticoagulants are being added. Read More

Indian J Dermatol Venereol Leprol 2016 Sep-Oct;82(5):478-88

Department of Dermatology, Command Hospital, Pune, Maharashtra, India.

Livedoid vasculopathy is a rare cutaneous disease manifesting as recurrent ulcers on the lower extremities. The ulceration results in atrophic, porcelain white scars termed as atrophie blanche. The pathogenesis is yet to be understood with the main mechanism being hypercoagulability and inflammation playing a secondary role. Read More

J Eur Acad Dermatol Venereol 2017 Jan 11;31(1):e45-e46. Epub 2016 May 11.

Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

J Am Acad Dermatol 2016 May;74(5):795-804; quiz 805-6

Boston University School of Medicine, Boston, Massachusetts. Electronic address:

Both inherited and acquired hypercoagulable states can present with nonspecific clinical manifestations, such as petechiae, purpura, livedo reticularis, and ulcerations. A good history and physical examination are crucial to diagnoses of these conditions. Inherited conditions tend to present either in neonatal period or later in life, while acquired conditions typically occur later in life. Read More

JAAD Case Rep 2015 Nov 24;1(6):340-1. Epub 2015 Sep 24.

Department of Dermatology, University of Alabama at Birmingham, Birmingham, Alabama.

JAAD Case Rep 2015 Sep 25;1(5):310-1. Epub 2015 Aug 25.

Department of Dermatology, St. Marianna University School of Medicine, Kawasaki, Japan.

Acta Derm Venereol 2016 11;96(7):987-988

Department of Experimental and Clinical Medicine, Institute of Dermatology, University of Udine, Udine, Italy.

Clin Exp Dermatol 2016 Jul 19;41(5):559-61. Epub 2016 Mar 19.

Department of Dermatology, Korea University Ansan Hospital, Korea University, Ansan, Korea.

Rev Bras Reumatol 2016 Feb 17. Epub 2016 Feb 17.

Serviço de Radiologia, Hospital e Maternidade Celso Pierro, Pontifícia Universidade Católica de Campinas, Campinas, SP, Brasil.

Livedoid vasculopathy is a chronic and painful skin disease that progresses to ulceration. Due to its uncertain pathogenesis, there is no single effective treatment for this condition, and current therapeutic options are based on isolated case reports, or on case series. We report a case of a female patient with livedoid vasculopathy, which achieved excellent healing of lower limb ulcers after using an anti-TNF agent. Read More

Lancet Haematol 2016 Feb 16;3(2):e72-9. Epub 2016 Jan 16.

Department of Dermatology, University Hospital of Münster, Münster, Germany. Electronic address:

Background: Livedoid vasculopathy is a thrombotic skin disease characterised by recurrent occlusion of the cutaneous microcirculation in lower extremities, which results in skin infarctions with painful ulcerations and irreversible scar formation. Rivaroxaban is a direct factor Xa inhibitor that prevents thrombus formation. We investigated whether rivaroxaban is effective for the treatment of livedoid vasculopathy. Read More

Lancet Haematol 2016 Feb 16;3(2):e56-7. Epub 2016 Jan 16.

Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL 33136, USA. Electronic address:

Acta Derm Venereol 2016 08;96(6):844-5

Unit of Dermatology, Sorbonne Universités, UPMC Paris 6, Hôpital Tenon, AP-HP, Paris, France.

Indian J Dermatol 2015 Sep-Oct;60(5):524

Department of Marine Medicine, INHS Asvini, Mumbai, Maharashtra, India.

A 30-year-old male presented to the dermatology department with complaints of multiple ulcers over both legs of 6 years duration. The ulcers had a waxing and waning course with present exacerbation of lesions since 1 month. Dermatological examination revealed multiple ulcers distributed in a reticular pattern over medial and lateral aspects of both lower legs, extensor aspect of both ankles and dorsum of both feet. Read More

Indian J Dermatol Venereol Leprol 2016 Mar-Apr;82(2):222-4

Department of Dermatology, Venereology and Leprosy, Lady Hardinge Medical College and Associated Hospitals, New Delhi, India.

Med Hypotheses 2015 Nov 29;85(5):670-4. Epub 2015 Aug 29.

Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo, Department of Pathology, R. Dr. Eneas de Carvalho Aguiar, 255 - 3rd Fl, São Paulo, SP CEP 05403-000, Brazil.

Livedoid vasculopathy is a chronic disorder characterised by recurrent reticulated purpura on lower extremities, associated with painful purpuric or necrotic macules and ulcerations. Current knowledge indicates LV to be a thrombo-occlusive vasculopathy of cutaneous blood vessels; exact pathogenesis is yet to be understood. Elevated levels of lipoprotein(a) have been found in LV patients. Read More

Ann Dermatol Venereol 2015 Aug-Sep;142(8-9):513-8. Epub 2015 Jul 11.

Service de dermatologie, UF de dermatologie vasculaire, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. Electronic address:

Vascular acrosyndromes are associated with vasomotor disorders. They may be paroxysmal, like Raynaud's phenomenon, whitening of the fingers on exposure to cold, or erythromelalgia, a painful form of erythema induced by exposure to heat. Others are permanent or semi-permanent, such as acrocyanosis, chilblains, spontaneous haematoma of the fingers, acrocholose and digital ischaemia or necrosis. Read More

Neuropathol Appl Neurobiol 2015 Oct 30;41(6):849-52. Epub 2015 May 30.

Department of Internal Medicine and Clinical Immunology, Hospital University Department: Inflammation, Immunopathology and Biotherapy (DHU i2B), Assistance Publique - Hôpitaux de Paris, Pitié-Salpêtrière University Hospital, Paris, France.

Dermatol Ther 2015 Sep-Oct;28(5):287-90. Epub 2015 Apr 6.

Department of Dermatology, Seoul National University Boramae Hospital, Seoul, Korea.

Livedoid vasculopathy (LV) is a thrombotic vasculopathy of the skin of unknown origin. No treatment has been validated in this indication, but case reports demonstrated successful use of intravenous immunoglobulins (IVIg) in LV. We assessed the efficacy and tolerability of 2 g/kg IVIg therapy every month for 2∼3 cycles in patients with refractory LV. Read More

Dermatol Ther 2015 Jul-Aug;28(4):248-53. Epub 2015 Mar 30.

Dermatology and Cutaneous Surgery, University of Miami, Miami, Florida.

Livedoid vasculopathy (LV) is a thrombo occlusive disorder presenting with recurrent painful ulcers of lower extremities. Association of LV with increased level of lipoprotein (a) (LP(a)), a risk factor for cardiovascular disease, has been reported. Danazol has been used with success in the management of LV, but none of the previous studies looked at the correlation between response to the treatment and level of LP(a). Read More