J Eur Acad Dermatol Venereol 2017 Jun 13. Epub 2017 Jun 13.

Universitaetsklinikum Münster, Muenster, Germany.

Arthritis Rheumatol 2017 May 16. Epub 2017 May 16.

Institute of Autoimmune Systemic and Neurological Diseases, 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 Mar 2. Epub 2017 Mar 2.

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 (ntotal = 1357) and calciphylaxis (ntotal = 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.

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.

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 2016 Oct 18. Epub 2016 Oct 18.

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

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

J Neurol Sci 2015 Apr 14;351(1-2):214-5. Epub 2015 Mar 14.

Neurology Department, Santa Chiara Hospital, Trento, Italy.

Pan Afr Med J 2014 27;18:339. Epub 2014 Aug 27.

Service de Dermatologie et de Vénéréologie, CHU Ibn Rochd, Casablanca, Maroc.

G Ital Dermatol Venereol 2015 Feb 17;150(1):51-71. Epub 2014 Dec 17.

Department of Clinical-Surgical Diagnostic and Pediatric Science, Unit of Dermatology, University of Pavia, IRCCS Fondazione Policlinico San Matteo, Pavia, Italy -

The most recent pediatric vasculitis classifications (EULAR/PRINTO/PRES) have proposed the use of an integration of clinical signs and symptoms, laboratory data, imaging and pathologic data. Pediatric vasculitis represent a peculiar clinical-diagnostic model, compared to the corresponding adult pathology chapter, and in particular, dermatopathologic aspects of these diseases identify more specific issues, made contingent by crucial variables such as duration of vasculitis lesion, site of the biopsy, proper biopsy depth, and possibility to correlate histopathological findings with immunopathological results. Possible additional diagnostic difficulties may arise from the fact that, in children, the same systemic disease, such as lupus erythematosus, may present with different clinical manifestations, with histopathological features of a precise type of vasculitis specific for that type of clinical manifestation. Read More

JMIR Res Protoc 2014 Dec 10;3(4):e73. Epub 2014 Dec 10.

Clinical Trial Support, Muenster, Germany.

Background: Livedoid vasculopathy is an orphan skin disease characterized by recurrent thrombosis of the cutaneous microcirculation. It manifests itself almost exclusively in the ankles, the back of the feet, and the distal part of the lower legs. Because of the vascular occlusion, patients suffer from intense local ischemic pain. Read More

J Am Acad Dermatol 2014 Nov 15;71(5):1024-6. Epub 2014 Oct 15.

Department of Dermatology, University of São Paulo, São Paulo, Brazil.

Rheum Dis Clin North Am 2015 ;41(1):141-60, ix

Rheumatology Unit, Massachusetts General Hospital, Yawkey 2, 55 Fruit Street, Boston, MA 02114, USA.

The need to distinguish true primary systemic vasculitis from its multiple potential mimickers is one of the most challenging diagnostic conundrums in clinical medicine. This article reviews 9 challenging vasculitis mimickers: fibromuscular dysplasia, calciphylaxis, segmental arterial mediolysis, antiphospholipid syndrome, hypereosinophilic syndrome, lymphomatoid granulomatosis, malignant atrophic papulosis, livedoid vasculopathy, and immunoglobulin G4-related disease. Read More

Pediatr Rheumatol Online J 2014 10;12:46. Epub 2014 Oct 10.

Department of Pediatrics C, Schneider Children's Medical Center of Israel, Tel Aviv University, Sackler School of Medicine, Petach Tikvah, Israel.

Cutaneous Polyarteritis Nodosa (cPAN) was first described in 1931. cPAN is considered a rare disease, its true incidence is unknown. The age of onset is diverse. Read More

Adv Skin Wound Care 2014 Nov;27(11):518-24; quiz 525-6

Afsaneh Alavi, MD, MSc • Lecturer • Department of Medicine (Dermatology), University of Toronto • Ontario, Canada Jurg Hafner, MD • Professor of Dermatology and Venereology, Senior Staff Physician • Department of Dermatology, University Hospital of Zurich • Switzerland Jan P. Dutz, MD • Professor • Department of Dermatology and Skin Science, University of British Columbia • Vancouver, Canada Dieter Mayer, MD • Associate Professor • Vascular Surgery • Head of Wound Care • University Hospital of Zurich • Switzerland R. Gary Sibbald, BSc, FRCPC(Med Derm), MACP, FAAD, MAPWCA • Professor of Medicine and Public Health • University of Toronto • Ontario, Canada Paulo Ricardo Criado, MD, PhD • Professor • Dermatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo • Brazil Patricia Senet, MD • Associate Professor • Department of Dermatology, Assitance Pubique-Hôpitaux de Paris • France Jeffery P Callen, MD • Professor of Medicine and Chief • Division of Dermatology, University of Louisville • Kentucky Tania J Phillips, MD • Professor • Department of Dermatology, Boston University School of Medicine • Massachusetts Marco Romanelli, MD, PhD • Professor • Department of Dermatology, University of Pisa • Italy Robert S Kirsner, MD, PhD • Professor and Vice Chair • Department of Dermatology, University of Miami Miller School of Medicine • Florida.

Purpose: The purpose of this learning activity is to provide information about the etiology and treatment of atrophie blanche.

Target Audience: This continuing education activity is intended for physicians and nurses with an interest in skin and wound care.

Objectives: After participating in this educational activity, the participant should be better able to:1. Read More

Vasc Med 2014 Oct;19(5):415-6

Hôtel-Dieu de France, Beirut, Lebanon.

Br J Dermatol 2015 Apr 25;172(4):1148-50. Epub 2015 Feb 25.

Department of Dermatology, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, U.S.A.

Muscle Nerve 2015 Feb 23;51(2):296-301. Epub 2014 Dec 23.

Department of Clinical Neurophysiology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Glossop Road, Sheffield, S10 2JF.

Introduction: Livedoid vasculopathy is a rare dermatological condition characterized by painful ulceration, atrophic scarring, and persistent livedo reticularis. The pathogenesis is unclear.

Methods: We report a patient with biopsy-proven livedoid vasculopathy who developed a progressive sensory ganglionopathy with profound sensory ataxia. Read More

An Bras Dermatol 2014 Sep-Oct;89(5):822-4

Fundação Alfredo da Matta, Manaus, AM, Brazil.

The livedoid vasculopathy is an obstructive vascular process of etiology not yet fully known, being possibly associated with several prothrombotic events. It is clinically characterized by the presence of painful and recurring purpuric lesions, which usually suffer ulceration and evolve with formation of white atrophic scars usually located in the lower limbs. Two cases are here reported of painful ulcerated lesions on the lower limbs, in which the identification of VL enabled the diagnosis of systemic diseases. Read More

Am J Dermatopathol 2015 Jul;37(7):581-4

Departments of *Dermatology, and †Pathology, La Paz University Hospital, Madrid, Spain.

Diffuse dermal angiomatosis is a rare benign condition considered a variant of reactive angioendotheliomatosis, usually related to vascular disease such as arteriovenous fistula or severe peripheral vascular disease. The most frequent clinical manifestations range from a solitary erythematous patch to an indurated plaque that may ulcerate. A clinical case of a 60-year-old woman who developed generalized livedoid lesions 2 days after the administration of intravenous trabectedin and subcutaneous pegfilgrastim for a recidivant myxoid liposarcoma has been reported. Read More

Int J Dermatol 2014 Dec 29;53(12):1495-7. Epub 2013 Oct 29.

Dermatology Department, Hospital Central "Ignacio Morones Prieto", Universidad Autónoma de San Luis Potosí, San Luis Potosí, SLP, México.