Livedoid Vasculopathy Research Articles

The management of livedoid vasculopathy focused on direct oral anticoagulants (DOACs): four case reports successfully treated with rivaroxaban.

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.

Livedoid Vasculopathy: A French Observational Study Including Therapeutic Options.

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. Investigation of thrombophilia was performed. Electromyography was undertaken in the presence of symptoms suggesting peripheral neuropathy. Eighteen women and 8 men were included, with a mean age of 35.5 years at onset. Twenty patients had at least one thrombophilia factor. Ten patients had a peripheral neuropathy with 2 of these patients demonstrating a specific thrombo-occlusive vasculopathy on muscle biopsy. Anticoagulation with low molecular weight heparin was the most prescribed therapy and was associated with the best outcome (effective in 14 patients). Eight patients had severe disease refractory to anticoagulation and required intravenous immunoglobulins, producing a good response in 6 patients.

Dermoscopy of early non-ulcerated livedoid vasculopathy

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. A mixture of ivory-white atrophic areas with multiple erythematous papules in the center of the lesions is characteristic. With dermoscopy, these papules correspond to glomerular vessels. In the periphery of the lesions some telangiectatic linear vessels complete the picture, although they could be absent in some patients.

Livedoid vasculopathy: a compelling diagnosis.

Livedoid vasculopathy: a compelling diagnosis.

Ayurvedic Management of Atrophie Blanche - A Case Study

Atrophie Blanche (AB) is typically described as a variable dimensioned, smooth, ivory-white plaque stippled with telangiectases and is surrounded by hyper pigmentation. AB commonly occurs in middle-aged women on the lower legs or feet, often associated with ulcerations and chronic venous insufficiency (CVI). The ulcers are slow to heal and painful. We report the case of an Atrophie Blanche (Livedoid Vasculopathy) which inadequately treated for more than 8 years. We review the pathogenesis (Samprapti), typical clinical presentation (Purva Roopa and Roopa), diagnostic workup and treated through various Panchakarma procedure and Shamana Yogas.

Analysis of serum levels and cutaneous expression of lipoprotein (a) in 38 patients with livedoid vasculopathy.

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. To investigate Lp(a) expression in skin lesions and circulating serum Lp(a) levels in patients with LV. 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. 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. 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.

Vasculopathies, cutaneous necrosis and emergency in dermatology.

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 4.4% of these entities according to some studies of Emergency Dermatology Department. They become an important disease which has to be identified early to establish appropriate management and treatment. Some of them are well known, such as the leukocitoclastic vasculitis, Schölein-Henoch, panarteritis nodosa, antineutrophil cytoplasmic antibody associated vasculitis, giant cell arteritis, cryoglobulinemic vasculitis and antiphospholipid syndrome. More frequent vasculopathies are livedoid vasculopathy, pigmented purpuric dermatosis and calciphylaxis. Less common ones are caused by interferon and cholesterol crystal embolization. Others are very infrequent as Degos disease and Sneddon Syndrome. Among the more recently described ones there are deficiency of adenosine deaminase type 2 and crystalglobulinemia. The other group is composed of vasculopathies associated to microorganism as infective endocarditis, septic vasculopathy, aspergillosis, fusariosis, strongiloidosis, ecthyma gangrenosum, lucio phenomenon of leprosy and necrotic arachnidism. Finally, among these entities we can also find diseases associated with proinflammatory stages as disseminated intravascular coagulation, myeloproliferative disorders, intravascular lymphoma, metastasis intravascular. When we face cutaneous lesions characterized by reticulated violaceous lesions, palpable purpura or cutaneous necrosis, a careful clinico-pathological correlation as well as some laboratory or radiological tests are mandatory to further delineate a diagnosis and a proper first line empirical treatment.

Efficacy of new low-dose oral anticoagulants in recalcitrant livedoid vasculopathy

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....

Livedoid vasculopathy: a clinical case proposition

Livedoid vasculopathy is a chronic occlusive vasculopathy dermatosis, with a pauciinflammatory or noninflammatory characteristic, and is extremely painful. Its ethiology is not entirely known, being associated with pro-thrombotic events. Characterized by erythematous purpuric maculopapular lesions on the legs, especially on the ankles and feet, that ulcerates and heals with white atrophic scars, named atrophie blanche. Histopathology shows occlusion of dermal vases, segmental hyalinization and endothelial proliferation; there is also a discreet perivascular inflammatory infiltrate. We describe a case of livedoid vasculopathy showing the histopathological findings on the biopsy.

Successful treatment of recalcitrant livedoid vasculopathy with rituximab: Report of 2 cases

Successful treatment of recalcitrant livedoid vasculopathy with rituximab: Report of 2 cases

Two successful cases of combination therapy with rivaroxaban and stanozolol for livedoid vasculopathy

Two successful cases of combination therapy with rivaroxaban and stanozolol for livedoid vasculopathy

Enhanced mRNA expression of plasminogen activator inhibitor-1 in livedoid vasculopathy lesions.

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. The study specimens were retrospectively selected from archives of pathology department. We investigated PAI-1 mRNA expression in the paraffin blocks of patients with biopsy-proven LV and controls. We analyzed the presence of thrombus, fibrinoid necrosis, ulcer, and epidermal atrophy in study samples. The correlation between histologic findings and PAI-1 expression was investigated. Analyses were performed in 14 LV patients (mean age 31±20, 79% female) and 4 controls (mean age 64±19, 50% female). PAI-1 gene expression was significantly higher in LV compared to the control group (median 7.74 (Iqr:13.94) vs 1.0 (0.31)), P=.011. Histological analysis displayed that fibrinoid necrosis was present on all patients with LV, 61.5% displayed thrombus, 46.2% displayed ulcer, and 15.4% displayed epidermal atrophy. Overall, we did not observe any discerning difference in PAI-1 expression between the LV blocks with or without thrombus, fibrinoid necrosis, or epidermal atrophy, yet the LV specimens that displayed ulcer histologically had higher PAI-1 mRNA expression compared to those without ulcer (median 13.98 (Iqr:19.21) vs 2.86 (5.59)), (P=.046). PAI-1 mRNA expression is significantly increased in cutaneous lesions of patients with LV. Histological finding of ulcer is associated with increased PAI-1 expression in LV specimen. In the current era of PAI-1 inhibitors, enhanced local PAI-1 expression can form a novel and local therapeutic target in LV.

Analysis of the German DRG data for livedoid vasculopathy and calciphylaxis.

Livedoid vasculopathy and calciphylaxis are rare skin disorders. Large cohorts of patients have been missing so far for detailed analysis. 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. To avoid deviations to non-relevant secondary diagnosis and due to changes in ICD-10 indices, we selected the two most recent available years 2013 and 2014 for evaluation of the accompanying diagnoses for both, livedoid vasculopathy (n = 519) and calciphylaxis (n = 324). Those were additionally evaluated as possible comorbidity. The male-female ratio for livedoid vasculopathy was 2.1:1. Patients older than 45 years comprehended 74.7% of all patients with peaks between the ages of 45-50 and 70-75. Livedoid vasculopathy patients suffered from cardiovascular and renal diseases. Coding of coagulation disorders was found rarely in our analysis. For calciphylaxis, we calculated a male-female ratio of 1.7:1. Most of the patients were at an age between 65 and 80 years. Diagnosis at an age under 35 years was rare. In general, most calciphylaxis patients showed end-stage renal disease with need of dialysis and presented with the resulting complications. Our data analysis shows relevant comorbidity and cofactors of these rare diseases like livedoid vasculopathy and calciphylaxis in Germany by a large number of cases.

Vasculopathie livédoïde secondaire à une maladie cœliaque

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). We report the case of a 52-year-old woman presenting LV in which an abnormal scan image led us to diagnose coeliac disease. This enteropathy had caused vitamin B12 and folic acid deficiency, as well as HHC. Vitamin supplementation and a gluten-free diet resulted in complete healing of the lesions. This case underlines the importance of screening for and correction of coagulation disorders in patients with LV. It also suggests that in the event of HHC, coeliac disease should be sought, even in the absence of gastrointestinal symptoms.

Treatment application of rivaroxaban in Chinese patients with livedoid vasculopathy.

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. Due to the lack of large-scale studies in this rare disease, LV poses a great challenge to the doctors, and existing treatment has always been an individual attempt with off-label application. The main goals in the treatment of patients with LV are to avoid the repeated occurrence of active cutaneous lesions and prevent painful ulceration and irreversible scarring. The current report describes the cases of three Chinese patients with LV receiving rivaroxaban treatment, an oral direct inhibitor of factor Xa inhibitor, and observes the treatment effect of rivaroxaban during the follow-up. As an injection-free alternative to low-molecular-weight heparin (LMWP) and monitoring-free alternative to warfarin, rivaroxaban improves the quality of life and enhances the compliance of patients. All patients consider rivaroxaban as more tolerable than previous drugs and, therefore, continue the application of rivaroxaban, effectively improving the treatment effect of drugs and successfully avoiding the repeated occurrence of active cutaneous lesions. Treatment application of rivaroxaban in Chinese patients with LV successfully avoids the recurrence of active cutaneous lesions and prevents the progressive ulceration and scarring.

Dermoscopic features of livedoid vasculopathy.

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. To date, the dermoscopic features of livedoid vasculopathy have not been clearly described in the literature. In this observational study, we sought to evaluate the dermoscopic patterns of livedoid vasculopathy and determine whether the dermoscopic features are associated with certain histopathological characteristics. We evaluated 9 patients with livedoid vasculopathy by dermoscopy. Skin biopsy specimens were stained with hematoxylin and eosin for histopathologic examination, and dermoscopic features were correlated with histopathological characteristics. In the majority of patients with livedoid vasculopathy, examination with dermoscopy revealed central crusted ulcers or ivory white areas associated with peripheral pigmentation in a reticular pattern. In addition, increased vascular structures including linear and glomerular vessels were found. On histopathological examination, the central ivory white areas correlated with dermal fibrosis, the reticular pigmentation corresponded to epidermal basal layer hyperpigmentation or melanin within melanophages in the dermal papillae, and the vascular structures correlated with dilatation and proliferation of capillaries in the upper dermis. In summary, the most common dermoscopic features of livedoid vasculopathy identified in this study were central crusted ulcers or ivory white scar-like areas associated with peripheral reticular pigmentation and increased vascular structures. The characterization of dermoscopic criteria for livedoid vasculopathy may improve the accuracy in the clinical diagnosis and follow-up of this disease.

Use of Low-dose Oral Warfarin in Three Cases of Livedoid Vasculopathy.

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

Fast Cicatrization of Extensive Livedoid Vasculopathy Ulcers under Treatment with Sildenafil

Fast Cicatrization of Extensive Livedoid Vasculopathy Ulcers under Treatment with Sildenafil

A pediatric case of atrophie blanche secondary to livedoid vasculopathy.

A pediatric case of atrophie blanche secondary to livedoid vasculopathy.

Livedoid vaskülopatide anestezi ve düşük molekül ağırlıklı heparin kullanımı

Livedoid vasculopathy (LV) is a hyalinizing vascular disease characterized by painful purple macules and papules that subsequently ulcerate. This vasculopathy may be associated with chronic venous insufficiency, deep venous thrombosis, factor V Leiden mutation, protein C deficiency, antiphospholipid syndrome, increased homocysteine levels, abnormalities in fibrinolysis, increased platelet activation and sickle cell disease. Difficult venous access, unreliable measurement of peripheral O2 saturation and increased susceptibility to venous embolic events may be a challenge for anesthetists. There is limited data about anesthetic management of livedoid vasculopathy in the literature. This case report describes successful anesthetic management of two patients with livedoid vasculopathy.