Livedoid vasculopathy and peripheral neuropathy: A retrospective cohort study of 55 Chinese patients and literature review.

Livedoid vasculopathy (LV) is a thrombo-occlusive vascular disease with an uncertain aetiology. In addition to cutaneous manifestations, LV patients may develop peripheral neuropathy. This study aimed to examine features of peripheral neuropathy in Chinese LV patients. We retrospectively reviewed and analysed the clinical data of 55 LV patients treated at Peking Union Medical College Hospital and conducted a literature review of peripheral neuropathy in LV patients. The incidence of peripheral neuropathy in our cohort was 12.73%. Among the seven patients with neuropathy, five were women and two were men. Median age at enrollment and disease onset in these patients was 27.29 and 22.57 years, respectively. Mean time from the appearance of cutaneous manifestations to the development of neurological symptoms was 38.67 months. Peripheral neuropathy was generally refractory to treatment, asymmetric in the distal extremities, and slowly progressive. The main symptom was numbness; hypoesthesia and neuromuscular manifestations occurred occasionally. The proportion of patients reporting seasonal worsening of symptoms was significantly higher in LV patients with peripheral neuropathy than in LV patients without neuropathy (P < .05). Peripheral neuropathy is a potential complication of LV. LV patients with peripheral neuropathy require long-term follow-up.

Livedoid vasculopathy in 75 Brazilian patients in a single-center institution: Clinical, histopathological and therapy evaluation.

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 São 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.

Intravenous immunoglobulin in resistant livedoid vasculopathy: Analysis of a case series.

We report a retrospective analysis the efficacy of high-dose intravenous immunoglobulins on 9 patients with Livedoid vasculopathy for whom resistant to immunosuppressants plus anticoagulants or could not be prescribed. Intravenous immunoglobulins were used 2 g/kg per month in these patients. The treatment induced stabilization of the disease and all patients demonstrated improvement with IVIg. Complete and partial clinical response was achieved in 6 and 3 patients respectively.

Atheroembolism to the Breast.

We report the case of a woman presenting with livedo reticularis of the breast who was found to have atheroembolism to the breast following upper extremity percutaneous access. Atheroembolism is the embolization of cholesterol crystals off an atherosclerotic plaque that can occur spontaneously or as a result of vascular intervention. This is a unique presentation of an otherwise well-described complication of vascular catheterization, and we propose that livedo reticularis of the breast can be interpreted as a sign of atheroembolism in the appropriate clinical context.

Characteristics, risk factors and treatment reality in livedoid vasculopathy - a multicentre analysis.

BACKGROUND: Livedoid vasculopathy (LV) 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. OBJECTIVE: The purpose of this study was to better characterize epidemiology, clinical appearance and treatment reality of LV in a well-defined patient cohort. METHODS: The cohort was allocated within a prospective, multicentre, phase IIa trial that investigated the effect of rivaroxaban in LV. RESULTS: Analysis of 27 patients revealed that LV patients had an increased Body Mass Index (BMI; 11/27), hypertension (19/27) and increased levels of lipoprotein (a) (5/12) and homocysteine (10/12) in the blood. The female-to-male ratio was 2.1 : 1, and the median age was 53.0 years [interquartile range (IQR) 40.5-68]. Investigation of the clinical appearance found that 82% of patients had livedo racemosa, and the ankle region was most likely to be affected by ulceration (56-70%). The analysis of patient treatment history showed that heparin was most effective (12/17), while anti-inflammatory regimens were, although often used (17/24), not effective (0/17). CONCLUSION: We add clinical clues for a data supported diagnosis of LV, and we provide evidence that anticoagulants should be administered in monotherapy first line (EudraCT number 2012-000108-13-DE).

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.

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.

Lymphocytic thrombophilic arteritis complicated by systemic involvement.

Lymphocytic thrombophilic arteritis (LTA) is a recently described entity defined by primary lymphocytic vasculitis; it typically has a chronic indolent course. We describe a patient who presented with clinical and histological findings consistent with LTA and later developed bilateral focal testicular infarcts as well as an acute median nerve neuropathy.

Placas violáceas y necróticas rápidamente progresivas en un niño de 5 años de edad / Rapidly progressive violaceous and necrotic plaques in a 5 years-old boy

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Cutaneous polyarteritis nodosa causing refractory skin deformation and pigmentation as sequel.

A 39-year-old woman presented with prominent and painful livedo reticularis lesions spreading on her upper and lower extremities. Histopathologically, the small-to medium-sized arteries in the deep dermis and subcutis showed necrotizing vasculitis with cellular infiltration, suggesting cutaneous polyarteritis nodosa. The serum levels of inflammatory markers normalized with aspirin 100mg/day and prednisolone 10mg/day within 2 months, and there was no other skin or organ involvement over 18 months of follow up. However, serious refractory skin depressions and pigmentation remained after two years of treatment. This suggests the importance of early and aggressive therapy for cutaneous polyarteritis nodosa to prevent unsightly skin sequel, as well as control of disease activity.

Livedoid eruption in a patient affected by T-γδ large granular lymphocyte leukaemia.

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. To the best of our knowledge, this is the first report describing the association between livedo reticularis and T-γδ large granular lymphocyte leukaemia. It is plausible that a pathogenetic role of the neoplastic process is based on a cytotoxic antiendothelial activity.

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. Edoxaban is one of new oral anticoagulants. It directly inhibits factor Xa in the coagulation pathway and prevents thrombus formation. A 17-year-old Japanese male presented with a 1-year history of recalcitrant cutaneous ulcers and livedo racemosa on his lower extremities. Initially, the ulcers were treated with antiplatelets therapies; however, he experienced recurrence of ulcerations during summer time. A histological examination revealed dermal vessel thrombosis consistent with occlusive vasculopathy. These findings were diagnostic for LV. The patient was treated with oral low-dose edoxaban (15 mg/day). The skin ulcers were epithelised and livedo racemosa disappeared within 8 weeks. We herein report the successful treatment of recalcitrant LV with low-dose edoxaban in a patient with no identifiable coagulopathy.