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Paulo Ricardo Criado - One of the best experts on this subject based on the ideXlab platform.
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Livedoid Vasculopathy in 75 brazilian patients in a single center institution clinical histopathological and therapy evaluation
2021Co-Authors: Paulo Ricardo Criado, Jozelio Freire De Carvalho, Neusa Yuriko Sakai Valente, Carla Pagliari, Thâmara Cristiane Alves Batista Morita, Gabriela Franco Marques, Thais P Pincelli, Maria Salome Cajas Garcia, Beatrice Martinez Zugaib Abdalla, Mirian Nacagami SottoAbstract:This study presents a single center experience with Livedoid Vasculopathy (LV). A rare disease that can lead to severe quality of life impairment. Characterize clinical data of LV patients at the Dermatology Division at the University of Sao Paulo. A retrospective and transversal study was conducted, from 1 January 2005 to 31 December 2019. About 75 patients diagnosed as LV and confirmed by skin biopsy were included. Epidemiology, clinical appearance, histopathology data, and treatment history were observed. There were 78.66% Caucasian women, with a mean age of 39.9 years. Frequent cutaneous manifestations were ulcers, atrophic blanche-like scars, hyperpigmentation, purpuras, telangiectasias, and livedo racemosa. Pain, pruritus, and hypoesthesia were the main symptoms. After treatment, almost 40% of cases relapsed during spring and summer months. About 66% of cases had thrombophilia factors associated, such as high levels of lipoprotein(a). Frequent treatments included acetylsalicylic acid, pentoxifylline, and diosmin with hesperidin. Not being a prospective study. This research provides useful data on Latin American LV patients, indicating multifactorial conditions involved in LV pathogenesis. An extensive work-up including autoimmune laboratory tests, thrombophilia factors, and other conditions associated with venous stasis should be part of LV investigation and controlled to improve treatment response.
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Livedoid Vasculopathy diagnosis and treatment in pregnant women
2020Co-Authors: Alexandre Sacchetti Bezerra, Accacio De Almeida Abussamra Junqueira De Andrade, Afonso Cesar Polimanti, Rafael Vilhena De Carvalho Furst, Paulo Ricardo Criado, Joao Antonio CorreaAbstract:Abstract Livedoid Vasculopathy is a disease characterized by occlusion of the capillaries of the dermis, without inflammatory signs. It begins with purpuric papules or macules that develop into painful ulcers, mainly involving the ankles and feet. In this case report, we describe diagnosis and treatment in a young pregnant patient, with excellent clinical response.
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the management of Livedoid Vasculopathy focused on direct oral anticoagulants doacs four case reports successfully treated with rivaroxaban
2018Co-Authors: Gabriela Franco Marques, Paulo Ricardo Criado, Thâmara Cristiane Alves Batista Morita, Maria Salome Cajas GarciaAbstract: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. The therapeutic management is still a challenge, however, and most treatments were based on anecdotal off-label protocols. Over such context, direct oral anticoagulants (DOACS) arise as a potential treatment for this disease. This class of medications became an alternative from initial large studies applied on different pathologic scenarios regarding thromboembolic events. In that line, recent case series using DOACS, including rivaroxaban, started to emerge in the literature related to LV and reported successful prevention of cutaneous infarctions and ulcerations, providing physicians with a new promising alternative. The current report describes four cases of long-term recalcitrant LV, in which rivaroxaban monotherapy effectively reduced pain and cutaneous ulcerations in a few weeks of treatment without relevant side effects. The authors also review therapy management of the disease, focused on DOACS, and suggest a step-by-step approach to treat these patients, taking into consideration different resource profiles of each level of local health centers, the gravity of the cases, and risks/benefits for patients.
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analysis of serum levels and cutaneous expression of lipoprotein a in 38 patients with Livedoid Vasculopathy
2017Co-Authors: Danielle P G S Espinel, Robert S Kirsner, T H B Di Giacomo, Thais P Pincelli, Naiura Vieira Pereira, Miriam N Sotto, Paulo Ricardo CriadoAbstract: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. OBJECTIVES To investigate Lp(a) expression in skin lesions and circulating serum Lp(a) levels in patients with LV. METHODS Skin biopsy samples from 38 patients (27 women and 11 men) with active lesions diagnosed as LV and 9 samples of normal skin (5 women and 4 men) from control patients without LV were evaluated for skin expression of Lp(a) by immunohistochemistry. Plasma levels of Lp(a) were analyzed by immunoturbidimetry. RESULTS We found that lesional skin in patients with LV expressed 10-fold higher Lp(a) immunostaining than controls. High plasma levels of Lp(a) were observed in LV patients. We did not find a correlation (P = .02) between expression of Lp(a) in the skin and plasma levels of Lp(a) in patients with LV. CONCLUSIONS Increased Lp(a) expression in lesional skin of LV patients suggests the role of Lp(a) in the thrombo-occlusive Vasculopathy observed in this disease.
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lipoprotein a and Livedoid Vasculopathy a new thrombophilic factor
2015Co-Authors: Paulo Ricardo Criado, D P S Espinell, P Barreto, T H B Di Giacomo, Mirian Nacagami SottoAbstract: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. To date, elevated plasma levels of lipoprotein(a) are considered an independent and causal genetic risk factor for the development of cardiovascular disease, as well as a relevant factor in hypercoagulable states. Because of its structural homology with plasminogen, Lp(a) might have important anti-fibrinolytic properties. Altered endothelial function and participation in immune and autoimmune processes, such as antiphospholipid syndrome, are also potential mechanisms of Lp(a) involvement in LV pathogenesis. Lp(a) is part of the wound healing process; the possibility of Lp(a) serum elevation to reflect an acute-phase reagent in LV scenario is also considered. The objective of this review is to examine the possible association of lipoprotein(a) with LV pathogenesis, based on its effects on thrombogenesis, fibrinolysis and autoimmunity.
Ali T. Taher - One of the best experts on this subject based on the ideXlab platform.
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Livedoid Vasculopathy in a patient with lupus anticoagulant and MTHFR mutation: treatment with low-molecular-weight heparin
2012Co-Authors: Jihane Abou Rahal, Rim S. Ishak, Zaher K. Otrock, Abdul-ghani Kibbi, Ali T. TaherAbstract:Livedoid Vasculopathy is characterized by painful purpuric lesions on the extremities which frequently ulcerate and heal with atrophic scarring. For many years, Livedoid Vasculopathy has been considered to be a primary vasculitic process. However, there has been evidence considering Livedoid Vasculopathy as an occlusive Vasculopathy due to a hypercoagulable state. We present the case of Livedoid Vasculopathy in a 21-year-old female who had been suffering of painful lower extremity lesions of 3 years duration. The patient was found to be lupus anticoagulant positive and homozygous for methylenetetrahydrofolate reductase C677T mutation. The patient was successfully treated with low-molecular-weight heparin.
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Livedoid Vasculopathy associated with sickle cell trait significant improvement on aspirin treatment
2012Co-Authors: Jinane El Khoury, Abdul-ghani Kibbi, Ali T. Taher, Mazen Kurban, Ossama AbbasAbstract:Livedoid Vasculopathy (LV) is a chronic, recurrent, painful cutaneous disease manifesting as longstanding distal lower extremity ulcers that scar leaving stellate atrophic lesions known as 'atrophie blanche'. A significant number of cases have been associated with thrombophilic abnormalities. In this study, we describe, to the best of our knowledge, the first report of LV only associated with sickle cell trait with significant improvement on aspirin.
Afsaneh Alavi - One of the best experts on this subject based on the ideXlab platform.
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Livedoid Vasculopathy an updated review
2018Co-Authors: Robert Micieli, Afsaneh AlaviAbstract:To highlight the most recent findings in the literature on Livedoid Vasculopathy while providing a basic review of the disease. Lipoprotein(a) and plasminogen activator inhibitor continue to develop evidence supporting their involvement in the pathogenesis of Livedoid Vasculopathy. Systematic review of the literature has revealed that anticoagulants are the most commonly reported treatments in case series followed by anabolic steroids, antiplatelets, and intravenous immunoglobulins. No randomized or controlled trials have been performed studying treatment efficacy. The literature continues to support the hypothesis that Livedoid Vasculopathy is a disease of hypercoagulability and impaired fibrinolysis. There is no established treatment for Livedoid Vasculopathy, although a wide variety of treatments have been used to achieve partial or complete remission. Anticoagulants continue to be the most commonly reported treatment in the literature with rivaroxaban used most frequently.
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treatment for Livedoid Vasculopathy a systematic review
2017Co-Authors: Robert Micieli, Afsaneh AlaviAbstract: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. Evidence Review A systematic review of the literature using Ovid MEDLINE (covering the period January 1, 1946, through June 9, 2017) and Ovid EMBASE (covering January 1, 1947, through June 9, 2017) databases was performed with a broad and inclusive search strategy along with a subsequent search of the references of retrieved articles. All case series reports published in the English language and in a peer-reviewed journal discussing the treatment for Livedoid Vasculopathy diagnosis were included. Findings A total of 29 case series reports published in the English language and in a peer-reviewed journal discussed the treatment for Livedoid Vasculopathy. These reports represented a total of 339 patients, of whom 230 (68%) were female and 69 (20%) were male; sex was not stated for 40 patients. Treatment with anticoagulants, antiplatelets, anabolic steroids, thrombolytics, hyperbaric oxygen, intravenous immunoglobulins, vitamin supplementation, UV light, and a combination of 1 or more of these among other therapies had a favorable outcome. Anticoagulants were the most commonly used monotherapy, achieving a favorable response in 62 of 63 patients (98%). Anabolic steroids, intravenous immunoglobulins, and antiplatelets were the second, third, and fourth most commonly used treatments, respectively. All of these therapies were associated with good clinical outcomes. Adverse events were observed in 44 patients (13%). Conclusions and Relevance A variety of treatments with varying degrees of success have been used to treat Livedoid Vasculopathy. Randomized clinical trials should be performed in the future to better establish these treatments in clinical practice.
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Livedoid Vasculopathy and high levels of lipoprotein a response to danazol
2015Co-Authors: Paulo Ricardo Criado, Afsaneh Alavi, Danielle Priscilia De Souza Espineli, Neusayuriko Sakai Valentef, Robert S KirsnerAbstract: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). The aim of this study was to demonstrate the efficacy of low-dose danazol in the treatment of LV and its effects on LP(a). We present four cases with LV who were successfully treated with low-dose danazol, assessing the clinical characteristics and laboratory tests including the level of LP(a). The average age of the patients was 45 years and the mean duration of the disease was 19 years. The treatment regime of danazol 200 mg daily led to complete healing of ulcers and reduction in pain and a 70% (ranging from 52 to 87%) reduction in the level of LP(a). The limitation of this study is "small sample size." In our patients with LV, low-dose danazol led to clinical improvement along with significant reduction in the level of LP(a).
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therapeutic hotline short papers Livedoid Vasculopathy and high levels of lipoprotein a response to danazol
2015Co-Authors: Paulo Ricardo Criado, Afsaneh Alavi, Neusa Yuriko, Sakai Valente, Robert S KirsnerAbstract: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). The aim of this study was to demonstrate the efficacy of low-dose danazol in the treatment of LV and its effects on LP(a). We present four cases with LV who were successfully treated with low-dose danazol, assessing the clinical characteristics and laboratory tests including the level of LP(a). The average age of the patients was 45 years and the mean duration of the disease was 19 years. The treatment regime of danazol 200 mg daily led to complete healing of ulcers and reduction in pain and a 70% (ranging from 52 to 87%) reduction in the level of LP(a). The limitation of this study is "small sample size." In our patients with LV, low-dose danazol led to clinical improvement along with significant reduction in the level of LP(a).
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atrophie blanche is it associated with venous disease or Livedoid Vasculopathy
2014Co-Authors: Afsaneh Alavi, Paulo Ricardo Criado, Jurg Hafner, Jan P Dutz, Dieter Mayer, Gary R Sibbald, P Senet, J P Callen, Tania J Phillips, Marco RomanelliAbstract: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. Discuss the pathophysiology of atrophie blanche.2. Explore treatment options for Livedoid Vasculopathy. ABSTRACT: Atrophie blanche (AB) is a porcelain-white scar that may be seen at the base of a healed ulcer or in association with Livedoid Vasculopathy (LV). The term AB originally had been used synonymously with LV, whereas LV is a noninflammatory thrombotic condition presenting as either a primary or secondary event (often associated with coagulation).
C H Yang - One of the best experts on this subject based on the ideXlab platform.
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Livedoid Vasculopathy long term follow up results following hyperbaric oxygen therapy
2006Co-Authors: Weihsin Juan, Y S Chan, H S Hong, L C Yang, Jennifer C Lee, C H YangAbstract:Summary Background Livedoid Vasculopathy, also known as atrophie blanche, is a recurrent painful Vasculopathy appearing mostly on the lower limbs. Treatment is challenging and relapses are frequent. Objectives To analyse the long-term effect and safety of hyperbaric oxygen (HBO) therapy in treating Livedoid Vasculopathy. Methods Twelve patients with active Livedoid Vasculopathy were included in this study. All patients underwent HBO therapy five times a week. Each week photographs were taken and the total dose of analgesics was recorded. Side-effects were documented and assessed. Recurrence was defined as the presence of skin ulceration. Results Of the eight patients who completed the treatment, resumption of ambulation and reduction of analgesics were achieved at an average of 4·9 HBO therapy sessions. Leg ulcers in all eight patients healed completely at a mean of 3·4 weeks (range 2–5 weeks). Six patients suffered relapses of ulceration and responded to additional HBO therapy. No significant side-effects were found. Conclusions HBO is a relatively safe, fast and effective method to treat patients with Livedoid Vasculopathy.
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intractable Livedoid Vasculopathy successfully treated with hyperbaric oxygen
2003Co-Authors: C H Yang, Y S Chan, L B Liou, H S Hong, L C YangAbstract:We describe a new method for treating Livedoid Vasculopathy. The typical presentation of Livedoid Vasculopathy includes chronic, recurrent painful ulcers, satellite scar-like atrophy and telangiectasia involving the lower extremities. Histologically, these lesions show areas of ulceration and dermal vessel occlusion without frank inflammatory cell infiltration. There is currently no satisfactory therapy available for this disease. Hyperbaric oxygen (HBO) has recently established itself as one of the most effective methods of treating ischaemic wounds, including diabetic ulcers. We used this therapy in two patients whose lesions were resistant to multiple therapeutic modalities. Not only did their ulcers respond rapidly to the HBO therapy, but the disturbing wound pain also resolved at the same time. To our knowledge, this is the first successful trial of HBO therapy in Livedoid Vasculopathy. We believe this to be a very promising new therapy for Livedoid Vasculopathy and to be worth further investigation.
Hongzhong Jin - One of the best experts on this subject based on the ideXlab platform.
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real world data on pain management and effectiveness of anti tumor necrosis factor agents in refractory Livedoid Vasculopathy
2021Co-Authors: Yimeng Gao, Hongzhong JinAbstract:Livedoid Vasculopathy (LV) is a thrombo-occlusive vascular disease characterized by livedo reticularis, ulceration, and atrophie blanche on lower extremities. The treatment of LV is challenging and controversial; however, anti-inflammatory therapy is of proven effectiveness in its acute ulcerative stage1,2 .
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rivaroxaban for treatment of Livedoid Vasculopathy a systematic review
2021Co-Authors: Yimeng Gao, Hongzhong JinAbstract:Rivaroxaban is a direct inhibitor of activated coagulation factor X and competitively targets factor Xa via reversible binding. We conducted a systematic review of the efficacy and safety of rivaroxaban for treatment of Livedoid Vasculopathy (LV) by searching the PubMed, Cochrane and Embase databases. A total of 22 articles and 1 registered clinical trial were identified in the search of which 13 were included. The studies included 73 LV patients receiving rivaroxaban therapy (10-20 mg per day). Overall, 60 patients (82.2%) had responses to therapy, achieving remission of both pain and ulceration. Few adverse effects were observed. Thus, the consensus of the clinical evidence is that rivaroxaban is a well-tolerated and effective treatment for LV. However, this still needs to be confirmed by large prospective and/or case control studies.
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Livedoid Vasculopathy and its association with genetic variants a systematic review
2021Co-Authors: Yimeng Gao, Hongzhong JinAbstract:Livedoid Vasculopathy (LV) is considered a disease of hypercoagulability. Association of LV with genetic variants is poorly characterised and large-scale genetic association studies have not been performed. The aim of the study was to systematically review variants in LV patients and to analyse the available clinical data. A systematic search of the literature in PubMed and Embase databases was performed to identify articles investigating genetic variation in LV patients. Thirty studies or case reports were identified that reported 265 LV patients tested for at least one out of six genetic variations. Among them, PAI-1 -675 4G/5G was the most common, accounting for 85.26% (81/95). Heterozygous 4G/5G was the major genotype. PAI-1 A844G, MTHFR C677T, and MTHFR A1298C were the second, third, and fourth most common variants in LV patients. Prothrombin G20210A and Factor V G1691A were mainly present in LV patients from Europe, North America, and South America. This review highlights the associations between LV and genetic variants. The distribution of variants may be geographically or ethnicity dependent; however, large sample case-control studies are needed to clarify associations.
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plasminogen activator inhibitor 1 a potential etiological role in Livedoid Vasculopathy
2020Co-Authors: Yimeng Gao, Hongzhong JinAbstract:Livedoid Vasculopathy (LV) is a chronic, recurrent skin disorder with unknown aetiology and pathogenesis that seriously affects the quality of life of people who suffer from it. Plasminogen activator inhibitor (PAI)-1 is a primary inhibitory component of the endogenous fibrinolytic system in blood coagulation. PAI-1 also plays a role in many other physiological processes and activities, including thrombosis, fibrosis, wound healing, angiogenesis, inflammation, cell migration, and adhesion. Enhanced expression and genotype polymorphism of PAI-1 have been observed in LV patients. In this review, we summarise the known functions of PAI-1 with emphasis on the roles that PAI-1 probably plays in the pathogenesis of LV, thereby illustrating that PAI-1 represents a potential LV biomarker and therapeutic target for treating LV.
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efficacy of an anti tnf alpha agent in refractory Livedoid Vasculopathy a retrospective analysis
2020Co-Authors: Yimeng Gao, Hongzhong JinAbstract:Background: Livedoid Vasculopathy is a recurrent thrombo-occlusive Vasculopathy of cutaneous blood vessels and its standard or first-line therapy is still controversial. Besides hypercoagulability, inflammatory factors may also play a secondary role in the pathogenesis of this disease. Monotherapy of thrombolytics cannot achieve satisfactory results because of concomitant inflammation.Objective: This pilot study aimed to determine the efficacy of an anti-TNF-alpha agent in patients with refractory Livedoid Vasculopathy.Methods: We studied five patients with Livedoid Vasculopathy who were resistant to steroids, antiplatelets, or danazol therapy, and were treated with etanercept 25-50 mg once a week for 12 consecutive weeks. We assessed clinical characteristics, laboratory findings, and etanercept's efficacy on skin lesions, pain, and quality of life.Results: Etanercept therapy resulted in fast relief of pain in a mean time of 2 weeks. The median duration for the disappearance of erythema and ulcer healing was 8.8 weeks and 10.6 weeks, respectively. There was a reduction in pain by 34.3% after 12 consecutive weeks of etanercept treatment. Disease severity and quality of life significantly improved.Conclusions: In refractory Livedoid Vasculopathy patients, etanercept therapy is efficient for skin lesions and pain, and improvement of quality of life, especially in rapid relief of pain.