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J Eur Acad Dermatol Venereol 2019 Apr 22. Epub 2019 Apr 22.

University Hospital of Muenster, Dept. of Dermatology, Von Esmarch Str. 58, 48149, Muenster, Germany.

Background: Livedoid vasculopathy is a rare cutaneous thrombotic disease. It is characterized by occlusion of dermal vessels resulting in livedo racemosa, ulceration and atrophie blanche. Clear guidelines for diagnosis and treatment are missing. Read More

Acta Dermatovenerol Alp Pannonica Adriat 2019 Mar;28(1):37-40

Department of Dermatovenerology, Maribor University Medical Center, Maribor, Slovenia.

A 72-year-old female patient presented with an end-stage renal disease on on-line hemodiafiltration and warfarin therapy with advanced ulcerated calciphylaxis on the lower extremities, complicated by two episodes of cellulitis. She was successfully treated for 8 months with intravenous sodium thiosulfate in combination with modification of medication and dialysis treatment, careful wound care, and other supportive measures. Calciphylaxis is an uncommon life-threatening systemic disease, mostly occurring in patients with chronic kidney disease and other risk factors. Read More

Dermatol Surg 2019 Mar 14. Epub 2019 Mar 14.

Department of Dermatology, University of Southern California, Los Angeles, California Department of Dermatology, University of Southern California, Los Angeles, California Department of Dermatology, University of Nebraska Medical Center, Omaha, Nebraska.

Clin Exp Dermatol 2019 Feb 14. Epub 2019 Feb 14.

Department of Dermatology, Jena University Hospital, Jena, Germany.

Clin Exp Dermatol 2019 Jan 31. Epub 2019 Jan 31.

Department of Dermatology, Venereology and Leprology, AIIMS, Jodhpur, Rajasthan, India.

Clin Case Rep 2018 Nov 12;6(11):2045-2047. Epub 2018 Sep 12.

Meguro Chen Dermatology Clinic Tokyo Japan.

We reported a patient with systemic lupus erythematosus complicated by livedoid vasculopathy (LV), who responded well to intravenous immunoglobulin and warfarin. Cutaneous lesions of LV resemble those of cutaneous vasculitis. LV should be included in the differential diagnosis of leg ulcerations even in the presence of autoimmune disorders. Read More

Acta Dermatovenerol Alp Pannonica Adriat 2018 Sep;27(3):147-148

Department of Dermatology and Allergology, Dresden Academic Teaching Hospital, Dresden, Germany.

Livedoid vasculopathy is a rare disease related to a hypercoagulable state. It can lead to painful and chronic relapsing ulcerations. We report dermoscopic findings of early, non-ulcerated livedoid vasculopathy in five patients. Read More

J Dermatol 2019 Apr 29;46(4):e142-e143. Epub 2018 Aug 29.

Department of Dermatology and Plastic Surgery, Akita University Graduate School of Medicine, Akita, Japan.

Pediatrics 2018 Sep;142(3)

Departments of Microbiology and Immunology and.

Multicentric Castleman disease (MCD) is a rare entity that, unlike unicentric Castleman disease, involves generalized polyclonal lymphoproliferation, systemic inflammation, and multiple-organ system failure resulting from proinflammatory hypercytokinemia, including, in particular, interleukin-6. A subset of MCD is caused by human herpesvirus-8 (HHV-8), although the etiology for HHV-8-negative, idiopathic MCD (iMCD) cases is unknown at present. Recently, a consensus was reached on the diagnostic criteria for iMCD to aid in diagnosis, recognize mimics, and initiate prompt treatment. Read More

Autops Case Rep 2018 Jul-Sep;8(3):e2018034. Epub 2018 Jul 30.

Universidade de São Paulo (USP), Faculty of Medicine, Department of Dermatology. São Paulo, SP, Brazil.

Explore (NY) 2018 09 10;14(5):357-366. Epub 2018 Aug 10.

Department of Preventive Medicine, College of Korean Medicine, Daejeon University, 62 Daehak-ro, Daejeon 34520, Republic of Korea. Electronic address:

Introduction: This study was performed to evaluate the clinical effect of a Korean medicine treatment for livedoid vasculopathy (LV).

Methods: Five patients with LV were selected who exhibited blood stasis due to qi stagnation () caused by external cold () or dual deficiency of qi and blood () and who had suffered from chronic episodic LV for at least 2 years. The treatment consisted of Korean herbal medicine, Haechungtang (, HC). Read More

J Dtsch Dermatol Ges 2018 Jul;16(7):873-890

Department of Dermatology, Venereology and Allergology, University Medical Center Bochum, Bochum, Germany.

Successful management of patients with leg ulcers requires identification of the underlying etiology, with subsequent initiation of causal treatment, if feasible. Supplementary measures of first choice include stage-adjusted wound treatment, usually combined with compression therapy. The significance of systemic drugs has been the subject of controversial debate, depending on the underlying cause of the condition. Read More

Skinmed 2018 1;16(2):125-127. Epub 2018 Apr 1.

Department of Dermatology and Sexually Transmitted Diseases, Lady Hardinge Medical College and Suchita Kriplani Hospital, Shaheed Bhagat Singh Marg, Delhi, India.

38-year-old married woman presented with multiple spontaneous painful ulcers that had involved both forearms for the previous month, along with a single episode of generalized tonic-clonic seizures, with no residual palsy or paresis. She complained of photosensitivity and diffuse hair loss for the past 6 months. She also had had arthralgia for the previous 3 years, which had initially involved the small joints of the fingers (especially the proximal interphalangeal joints) and gradually progressed to involve the larger joints. Read More

JAAD Case Rep 2018 Mar 3;4(2):203-205. Epub 2018 Feb 3.

University of Texas Southwestern Medical Center, Dallas, Texas.

Acta Derm Venereol 2018 Oct;98(9):842-847

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

Int J Rheum Dis 2019 Jan 6;22 Suppl 1:69-77. Epub 2018 Apr 6.

Vasculitis Clinic, Mount Sinai Hospital, Toronto, Ontario, Canada.

Deficiency of ADA2 (DADA2) is a recently described systemic inflammatory vasculopathy caused by mutations in the CERC1 gene that often, but not always, clinically resembles polyarteritis nodosa (PAN). The condition was originally characterized by livedoid rash, systemic inflammation, variable hypogammaglobulinemia, and early-onset stroke. The phenotypic spectrum has expanded to include patients with immunodeficiency syndromes and bone marrow dysfunction, which are not typical features of PAN. Read More

Am J Dermatopathol 2018 Sep;40(9):682-685

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.

J Cutan Pathol 2018 May 14;45(5):365-368. Epub 2018 Mar 14.

Department of Pathology, Hartford Hospital, Hartford, Connecticut.

Injection drug abuse (IDA) is known to cause a spectrum of systemic and cutaneous complications. Despite the increasing incidence of IDA around the world, there is a paucity of literature discussing cutaneous complications from a dermatopathologic perspective. We present a case of a 35-year-old male with a complex medical history of Von Willebrand disease, Beçhet disease and diverticular disease. Read More

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

BMJ Case Rep 2017 Sep 7;2017. Epub 2017 Sep 7.

Unit of Dermatology, University of Padua, Padova, Italy.

Livedo is an ischaemic dermopathy characterised by a reddish-blue to violaceous mottling of the skin with a net-like reticular appearance. Livedo has been described in association with several medical conditions including lymphoproliferative disorders. Here, we describe the case of a 60-year-old woman who was presented with asymptomatic and persistent livedoid eruption on her trunk, lower and upper extremities as manifestation of an indolent form of T-γδ large granular lymphocyte leukaemia. 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 2018 May 28;59(2):e127-e132. 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 03 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