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Chronic skin ulcers in patients with suspected pyoderma gangrenosum can, on closer inspection and further workup, have a different cause. Recognition of key features on clinical examination such as the presence of atrophie blanche is key to avoid misdiagnosis of pyoderma gangrenosum and its subsequent treatment with high-dose corticosteroids and other immunosuppressive medications.

Fei Qi,1– 3 Yimeng Gao,1– 3 Hongzhong Jin1– 3 1Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, People’s Republic of China; 2State Key Laboratory of Complex Severe and Rare Diseases, Beijing, People’s Republic of China; 3National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, People’s Republic of ChinaCorrespondence: Hongzhong Jin, Peking Union Medical College Hospital (Dongdan Campus), No. 1 Shuaifuyuan Wangfujing Dongcheng District, Beijing, 100730, People’s Republic of China, Tel +86 10 6915 1500, Email jinhongzhong@263.netBackground: Livedoid vasculopathy is an uncommon cutaneous ulcerative dermatosis that is challenging to diagnose. Diagnostic delay brought both pain and uncurable atrophied scar to patients.Purpose: We conducted this study to identify the factors responsible for the initial misdiagnosis of livedoid vasculopathy and to identify possible methods to increase the diagnostic accuracy of livedoid vasculopathy.Patients and Methods: We conducted a retrospective medical record review to confirm the diagnosis of livedoid vasculopathy in patients who visited the Department of Peking Union Medical College Hospital for the first time. We used the Diagnosis Error Evaluation and Research taxonomy to evaluate missed cases.Results: Twenty-three patients (85.18%) had an alternate diagnosis, including 10 (43.4%) with two or more diagnoses. The average time from disease onset to the final diagnosis of livedoid vasculopathy was 4.61 ± 0.69 years. The major diagnostic errors were clinician assessment failures and failures in the timely follow-up and rechecking of patients. Allergic vasculitis was the most common misdiagnosis. Other alternate diagnoses include Henoch-Schoenlein purpura, pigmented purpuric dermatosis, eczema, erythema nodosum, and reactive perforating collagenases. Twenty-three patients (65.21%) received systemic corticosteroid therapy before the final diagnosis of livedoid vasculopathy.Conclusion: It is critical to raise the awareness of clinicians about livedoid vasculopathy, especially when patient present with extensive livedo racemosa or long-lasting purpuric lesions on the lower limbs. Long-term follow-up is necessary, especially for younger patients. Skin biopsy is recommended before systematic therapy.Keywords: livedoid vasculopathy, atrophie blanche, diagnostic accuracy, vasculitis

Livedoid vasculopathy (LV) is a chronic, recurrent thrombotic vasculopathy characterized by painful ulcerations on the lower extremities, which heal slowly and leave atrophic white scars known as "atrophie blanche." This report presents the case of a 31-year-old woman with a 4-year history of recurrent painful ulcerations on her legs and feet. A skin biopsy revealed findings consistent with LV, and an exhaustive laboratory workup ruled out secondary causes such as thrombophilia, malignancies, autoimmune diseases, and peripheral arterial disease. The patient showed remarkable improvement with a treatment regimen of pentoxifylline, nifedipine, and warfarin, resulting in complete ulcer resolution and sustained remission over 5 months. Our case highlights the importance of a comprehensive diagnostic approach and a multidisciplinary treatment strategy in managing primary LV to achieve remission and prevent recurrence of skin ulcerations. [ABSTRACT FROM AUTHOR]

Fei Qi, Yimeng Gao, Hongzhong Jin Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, 100730, People’s Republic of ChinaCorrespondence: Hongzhong Jin, Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases and Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, 100730, People’s Republic of China, Tel +86-10-69151500, Email jinhongzhong@263.netAbstract: The application of biologics such as anti-tumor necrosis factor (TNF) has shown great efficacy in livedoid vasculopathy (LV). However, new biological options need to be identified for those with a high tuberculosis reactivation risk. In this study, we evaluated the efficacy of anti-17A biologics for LV therapy. Two patients with LV who were irresponsive to traditional anticoagulation therapy were studied at the outpatient dermatology clinic of Peking Union Medical College Hospital. All patients received anti-17A biological therapy for at least two–four weeks. Both patients reported an exacerbation of the skin lesions, which might indicate that the IL-17 pathway plays a critical role in LV pathogenesis.Keywords: livedoid vasculopathy, biologic therapy, TNF-alpha, IL-17, atrophied blanched

Livedoid vasculopathy is a type of vascular disorder with unclear etiology and pathogenesis, in addition to the lack of standardized diagnostic and treatment guidelines. Currently, it is believed that the development of this disease is associated with blood hypercoagulability and inflammation. However, studies also suggest the involvement of immune and inflammatory reactions in its pathogenesis. And anti-inflammatory therapy is effective. The drugs used for the treatment of livedoid vasculopathy include plant alkaloids (colchicine, tripterygium glycosides), glucocorticoids, JAK inhibitors, adalimumab, intravenous immunoglobulins and anti-inflammatory drugs. This review aims to summarize the anti-inflammatory therapy and methods for livedoid vasculopathy, providing references for clinicians in the management of this disorder.

Summary: The term occluding vasculopathies covers a large number of different conditions. These often manifest as skin ulcers. Occluding vasculopathies should be considered in the differential diagnosis of leg ulcers. The term "occlusive vasculopathies" encompasses pathophysiologically related entities that share structural or thrombotic obliteration of small cutaneous vessels. In this article, we will focus on livedoid vasculopathy with and without antiphospholipid syndrome and calciphylaxis with differentiation from hypertonic leg ulcer as the most relevant differential diagnoses of leg ulcer. The term also includes vascular occlusion, for example due to oxalate or cholesterol embolism, and septic vasculopathy. This often leads to acral ulceration and is therefore not a differential diagnosis with classic leg ulcers. It will not be discussed in this article. Occlusive vasculopathy may be suspected in the presence of the typical livedo racemosa or (non‐inflammatory) retiform purpura as a sign of reduced cutaneous perfusion in the wound area. Inflammatory dermatoses, especially vasculitides, must be differentiated. This is achieved by histopathological evaluation of a tissue sample of sufficient size and depth taken at the appropriate time. In addition, specific laboratory parameters, particularly coagulation parameters, can support the diagnosis. [ABSTRACT FROM AUTHOR]

Livedoid vasculopathy is a type of vascular disorder with unclear etiology and pathogenesis? in addition to the lack of standardized diagnostic and treatment guidelines. Currendy, it is believed that the development of this disease is associated w让h blood hypercoagulability and inflammation. However, studies also suggest the involvement of immune and inflammatory reactions in its pathogenesis. And anti-inflammatory therapy is effective. The drugs used for the treatment of livedoid vasculopathy include plant alkaloids (colchicine, tripterygium glycosides), glucocorticoids, JAK inhibitors, adalimumab, intravenous immunoglobulins and anti-inflammatory drugs. This review aims to summarize the anti - inflammatory therapy and methods for livedoid vasculopathy, providing references for clinicians in the management of this disorder. [ABSTRACT FROM AUTHOR]

Pingjiao Chen,1,* Jingyao Liang,2,3,* Changxing Li,1 Qian Li,2,3 Wenyan Liu,2,3 Junhui Zhu,2,3 Weifeng Chen,2,3 Xibao Zhang2,3 1Department of Dermatology, Nanfang Hospital, Southern Medical University, Guangzhou, 510515, People’s Republic of China; 2Institute of Dermatology, Guangzhou Medical University, Guangzhou, 510095, People’s Republic of China; 3Department of Dermatology, Guangzhou Institute of Dermatology, Guangzhou, 510095, People’s Republic of China*These authors contributed equally to this workCorrespondence: Xibao Zhang, Department of Dermatology Guangzhou Institute of Dermatology, 56 Hengfu Road, Guangzhou, 510095, People’s Republic of China, Tel +8620-83593476, Email gzpfbfzs@163.comAbstract: Janus kinase (JAK) inhibitors are increasingly being used in dermatology due to their broad potential in managing both local and systemic inflammation. More recently, abrocitinib, an oral JAK 1 inhibitor, has shown promising clinical efficacy in the treatment of various skin disorders beyond moderate to severe atopic dermatitis (AD). We firstly presented three cases, each with diagnosis of pyoderma gangrenosum (PG), livedoid vasculopathy (LV), or hidradenitis suppurativa (HS), and conducted a comprehensive scoping review of the available literature on the use of abrocitinib in the treatment of diverse skin disorders. We summarized a total of 16 skin disorders, including our cases. The results indicated that abrocitinib, whether used as monotherapy or in combination with other treatments, was effective and well-tolerated in these disorders. These findings expanded the range of diseases for which abrocitinib may serve as an alternative therapeutic choice.Keywords: JAK inhibitors, abrocitinib, atopic dermatitis, pyoderma gangrenosum, livedoid vasculopathy, hidradenitis suppurativa

Background: Livedoid vasculopathy (LV) is characterized by fibrin deposition and thrombosis in the small vessels of the superficial dermis. It is widely recognized as an occlusive disease, which is primarily treated with anticoagulation therapy. Methods: We retrospectively analyzed the clinical and histopathological characteristics of patients diagnosed with LV at a tertiary dermatology department to explore the characteristics of lymphocytic vasculitis in LV. The frequency of vasculitis and the types of vessels involved were examined based on the diameters and elastic fiber distribution of the involved vessels. In addition, the immunophenotypes of infiltrating lymphocytes were analyzed. Results: In a large retrospective series including 358 LV cases, we identified 137 (38.3%) cases of lymphocytic vasculitis. Among them, 48 cases involved medium‐sized vessels, including arterioles and venules, whereas 89 cases involved only small vessels. In addition, 12 cases displayed a segmental distribution of vasculitis. The infiltrating lymphocytes were mainly T cells, with dominant cells stained positive for CD4. Conclusions: Lymphocytic vasculitis forms part of the histological spectrum of LV, affecting both medium‐sized and small vessels. It is possible that the occlusion of small vessels may represent a phenomenon secondary to lymphocytic vasculitis. [ABSTRACT FROM AUTHOR]

Abstract Background Livedoid vasculopathy (LV) and cutaneous arteritis (CA) are rare cutaneous diseases. A consideration of these two conditions as different entities is debatable, because thrombosis and inflammation are closely related in their pathogenesis mechanisms. Varying reports on the effectiveness of anticoagulant and immunosuppressant therapies in LV and CA also point to diagnostic challenge and overlap in these conditions. Objectives We sought to assess the therapeutic effect of anticoagulation therapy in adult patients with CA, who were recalcitrant to immunosuppressive modalities. Methods A monocentric retrospective analysis of adult patients who were diagnosed with CA between 2010 and 2018, was carried out. Results We describe 8 cases of long‐term recalcitrant CA, in whom anticoagulation therapy significantly reduced the pain and cutaneous symptoms. All patients had cutaneous symptoms compatible with CA along with clinical features also characteristic of LV, including underlying procoagulative disorders in the majority (87.5%), as confirmed by screening for thrombophilia. We also review current concepts regarding LV and CA, and suggest vascular occlusion is a common link between these two entities. Conclusions Although current knowledge advocates segregating vasculopathy from vasculitis by means of histopathological examination in LV and CA, clinical experience suggests some overlap and common complex underlying pathomechanisms.

Abstract Background: Livedoid vasculopathy (LV) manifests as ulcers and atrophic white scars on the lower extremities. The main known etiopathogenesis is hypercoagulability with thrombus formation, followed by inflammation. Thrombophilia, collagen and myeloproliferative diseases may induce LV, but the idiopathic (primary) form predominates. Bartonella spp. may cause intra-endothelial infection and skin manifestations caused by these bacteria may be diverse, including leukocytoclastic vasculitis and ulcers. Objective: The aim of this study was to investigate the presence of bacteremia by Bartonella spp. in patients with difficult-to-control chronic ulcers diagnosed as primary LV. Methods: Questionnaires and molecular tests (conventional PCR, nested PCR and real-time PCR) were applied and liquid and solid cultures were performed in the blood samples and blood clot of 16 LV patients and 32 healthy volunteers. Results: Bartonella henselae DNA was detected in 25% of LV patients and in 12.5% of control subjects but failed to reach statistically significant differences (p = 0.413). Study limitations: Due to the rarity of primary LV, the number of patients studied was small and there was greater exposure of the control group to risk factors for Bartonella spp. infection. Conclusion: Although there was no statistically significant difference between the groups, the DNA of B. henselae was detected in one of every four patients, which reinforces the need to investigate Bartonella spp. in patients with primary LV.

Lymphocytic thrombophilic arteritis and livedoid vasculopathy may both present with livedo racemosa and ulceration. We present 6 cases with features of both conditions, raising the possibility that they are either closely linked or are part of a spectrum of the same condition. [ABSTRACT FROM AUTHOR]

A 40-year-old woman presented with painful ulcerations on the bilateral lower extremities. A biopsy confirmed the diagnosis of livedoid vasculopathy (LV). She was treated initially with aspirin and pentoxifylline, and with the addition of dipyridamole she has had no recurrence of her ulcerations to date. Despite this positive response to treatment she reported numbness and paresthesias in her legs. Nerve conduction studies confirmed a diagnosis of mononeuritis multiplex. This case highlights mononeuritis multiplex as a rarely described complication of LV, and suggests that early recognition of symptoms and a multidisciplinary approach are necessary for optimal management of this condition.

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. [ABSTRACT FROM AUTHOR]

Background: Clinical data regarding cutaneous manifestations in Chinese patients with livedoid vasculopathy (LV) are limited. Objectives: To assess clinical features of skin lesions in LV, especially the characteristics of extensive livedo reticularis and pigmented purpuric dermatosis‐like lesions in these patients. Methods: This was a single‐center retrospective study of 46 Chinese patients with LV between March 2021 and July 2021. The characteristics of skin lesions in LV were described in detail. Results: A total of 29 females and 17 males were included in this study, with a mean age of 27.7 years (ranging from 13 to 51 years). Twenty (43.5%) patients developed their first skin lesions before age 18. Among 46 patients, 33 presented livedo reticularis with 78.8% (n = 26) of these patients whose livedo reticularis was extensive. Seven patients had lesions simulating pigmented purpuric dermatosis, including four cases of pigmented purpura and three cases of telangiectatic purpura. Numbness was found in 16 patients, mainly in the lower limbs (62.5%), ankles (31.3%), and dorsum of the feet (18.8%). Conclusions: For patients with symptoms of extensive livedo reticularis, retiform purpura, or numbness, it is necessary to make a differential diagnosis. [ABSTRACT FROM AUTHOR]

Livedoid vasculopathy (LV) is a rare thrombotic vasculopathy of the dermis characterized by painful, relapsing ulcers over the lower extremities. Diagnosis is challenging due to the overlap in clinical appearance and nomenclature with other skin disorders. Treatment selection is complicated by poor understanding of the pathogenesis of LV and lack of robust clinical trials evaluating therapy efficacy. The terminology and pathophysiology of LV are reviewed here, along with its epidemiology, clinical and histologic features, and treatment options. A diagnostic pathway is suggested to guide providers in evaluating for comorbidities, referring to appropriate specialists, and choosing from the available classes of therapy. [ABSTRACT FROM AUTHOR]

Keywords: livedoid vasculopathy; skin; thrombotic vasculopathy; wound/ulcer EN livedoid vasculopathy skin thrombotic vasculopathy wound/ulcer 609 612 4 12/12/22 20221201 NES 221201 What is livedoid vasculopathy (LV)? Summary Livedoid vasculopathy is a rare condition characterized by small ulcers, reddish-purple skin discoloration, and scars (atrophie blanche) on the lower legs that come and go without a clear trigger. Treatment options include diligent wound care, antibiotics if the ulcers are infected, blood thinners (anticoagulation and/or antiplatelet agents), and immune-modulating medications. Blood clots in LV cut off the flow of oxygen to the skin, causing the skin tissue to die and form ulcers. [Extracted from the article]

Abstract Livedoid vasculopathy (LV) is a rare thrombotic occlusive vasculopathy presenting with chronic relapsing‐remitting bilateral painful skin ulcerations predominating on the ankles and dorsal surface of the feet. LV can be idiopathic or secondary to endothelial dysfunction, rheological disorder, haemostatic or fibrinolytic pathology. We describe the case of a 49‐year‐old patient with LV and summarise the current knowledge on epidemiology, risk factors, and treatment of this rare condition. It is important to differentiate LV from vasculitic disorders, the management of the latter being based on immunosuppression, whereas the treatment of LV is based on anticoagulant or antiplatelet therapy.

Livedoid vasculopathy (LV) is a rare clinical condition presenting as painful lesions mostly on the lower extremities. We present a case of LV with peripheral neuropathy in a young man initially misdiagnosed and treated for cellulitis. He was started on aspirin, pentoxifylline and apixaban immediately after the diagnosis of LV. However, pain management was a real challenge for the clinicians. Hence, he was later treated with epoprostenol and amlodipine for vasodilation, steroids for any possible inflammation, and antibiotics to treat superimposed infection. Irrespective of all the above, his pain was uncontrollable, and he finally received ketamine infusions along with narcotics, achieving better pain control. Various studies support the use of intravenous immunoglobulin and anti-TNF agents for pain relief in idiopathic and secondary LV. Intermittent low-dose dabigatran has also been found to be effective in the maintenance of remission in LV. However, no large studies have yet been conducted to confirm the efficacy of these medications.

Objective To analyze the clinical features, diagnosis and treatment of 11 patients with livedoid vasculopathy. Methods The general data, clinical manifestations, dermoscopic findings, histopathological features and prognosis of diagnosis and treatment of 11 patients with livedoid vasculopathy were retrospectively analyzed. Results Among the 11 patients, there were 1 male and 10 females. The calf, ankle and dorsum were involved with erythema, purpura and blisters, followed by ulceration, ivory-white atrophy, scar and pigmentation after recovery. Five cases were severe in summer and mild in winter, and 6 cases were misdiagnosed as vasculitis and other diseases. Histopathological examination revealed fibrinoid necrosis and/or vascular embolism in dermal vessels. Five patients had common features of livedoid vasculopathy by dermoscopy. There was no recurrence in 6 patients who received combined treatment with oral glucocorticoid, rivaroxaban, dipyridamole, and aspirin, but 3 of 5 patients relapsed after single-drug treatment. Conclusions Livedoid vasculopathy is easy to be misdiagnosed clinically. When the ankle and dorsum of the foot are involved, the disease should be highly vigilant. Diagnosis is mainly based on histopathological changes, and dermoscopy can improve the diagnostic accuracy. Single-drug treatment is prone to recurrence, and combined drug treatment is effective. [ABSTRACT FROM AUTHOR]

Livedoid vasculopathy is a rare disorder clinically presenting with triad of livedo reticularis, leg ulcerations, and atrophie blanche. We present a case series of 17 patients with clinical and/or histopathologically confirmed livedoid vasculopathy from a single tertiary centre in India with female-to-male ratio of 1.5:1 and mean age of 36.12 ± 12.02 years. Presentation with burning pain around ankles was seen in 83.33% of patients, while 100% had atrophie blanche/scarring and 76.47% had retiform ulcers. Hypercholesterolemia was seen in four patients, while systemic lupus erythematosus (SLE), anti-phospholipid antibody with SLE, dermatomyositis and hyper-homocysteinemia were seen in one patient each. The most common histopathology finding was hyaline thrombi within dermal vessels in 94.11%. On treatment with dual anti-platelet therapy, 70.58% of patients could achieve significant improvement in their Visual Analog Scale, Dermatology Life Quality Index and reduction in ulcer scores without serious adverse events. Out of 17 patients, 11 experienced flare in their disease course over one year period of follow-up. This cohort aims to contribute to Indian literature of this underreported entity. [ABSTRACT FROM AUTHOR]

Background: Livedoid vasculopathy is a rare, chronic, and recurrent disease with limited effective treatments. Its etiopathogenesis remains incompletely understood. Baricitinib, a selective Janus kinase 1 and 2 inhibitor, has been used to treat rheumatoid arthritis and could reduce the disease severity in patients with livedoid vasculopathy. Methods: We retrospectively observed eight patients who received 2 mg/day of baricitinib for the treatment of refractory livedoid vasculopathy. We evaluated their clinical scores before and after treatment to determine its effectiveness and safety. Results: Improvement in livedoid vasculopathy was observed with significant regression in the clinical scores after baricitinib treatment. The mean clinical scores were 7.0 ± 1.6 and 1.4 ± 1.2 before and after baricitinib treatment, respectively (P <0.01). Furthermore, six out of the eight patients achieved a clinical score of 0 or 2 after treatment. These scores indicated remission. Clinical findings, including erythema, ulceration, and pain, improved in all the patients. The remission times ranged from 3 to 13 weeks, with a mean remission time of 7.75 ± 3.45 weeks. There were no reports of adverse events in any patient. Conclusions: Our study showed that baricitinib treatment was safe and could significantly relieve the signs and symptoms of livedoid vasculopathy. However, randomized controlled studies should be conducted to confirm these results. [ABSTRACT FROM AUTHOR]

Background: The pathogenesis of livedoid vasculopathy (LV) remains unknown. Although platelet activation occurs in LV, little research has been conducted on LV platelet morphology parameters. The purpose of this study was to investigate whether platelet morphology changes in LV and its clinical significance. Methods: Twenty‐seven LV patients and 21 cutaneous small vessel vasculitis (CSVV) patients, all at the active stage, were included. Platelet parameters in active‐ and stable‐stage LV and CSVV patients were compared. Correlations between these platelet parameters and LV composite clinical scores were analysed. Results: LV patients' mean age was 25.48 years (range: 9–62 years), and 81.48% (22/27) were women and 18.52% (5/27) were men. The platelet counts and plateletcrit (PCT) levels were significantly elevated in LV patients compared with CSVV patients and in active‐stage LV patients compared with stable‐stage LV patients after treatment. LV patient composite clinical scores that reflected disease severity and activity were positively correlated with the platelet count and PCT levels. Conclusion: Altered platelet morphology was detected in LV patients. Platelet count and PCT might be haematological biomarkers for early prediction of LV activity and relapses and for differential identification between LV and CSVV. [ABSTRACT FROM AUTHOR]

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Primary systemic vasculitis can present with a wide spectrum of manifestations ranging from systemic non-specific features such as fever, malaise, arthralgia and myalgia to specific organ damage. We describe two cases of cholesterol embolisation syndrome and Kaposi sarcoma mimicking primary systemic vasculitis, both of which were characterised by features such as livedo reticularis, blue toe syndrome, a brown purpuric skin rash and positive perinuclear anti-neutrophil cytoplasmic antibodies associated with Kaposi sarcoma. Establishing the right diagnosis was challenging and thus this report aimed to highlight the possible ways to distinguish them from primary systemic vasculitis. [ABSTRACT FROM AUTHOR]

Livedoid vasculopathy is a chronic, recurrent skin disorder. It seriously affects the quality of patients' life. However, the pathogenesis has not been fully identified yet. Here, this retrospective study describes the successful use of anti-TNF-α agent adalimumab in three cases of refractory livedoid vasculopathy, which has not been reported previously. In addition, we provide some clinical evidence that adalimumab therapy is efficient in improving skin lesions and relieving the pain of livedoid vasculopathy. [ABSTRACT FROM AUTHOR]

Resumen: La patología vascular oclusiva es causante de diversas y variadas manifestaciones clínicas, algunas de ellas con catastróficas consecuencias para el paciente. Dado que las causas de tal oclusión son muy variadas, hemos abordado en un artículo previo reciente en esta misma revista las causas trombóticas. En el presente artículo recopilamos diversas causas adicionales de oclusión intravascular. Abstract: Vascular occlusion has multiple, diverse clinical manifestations, some of which can have grave consequences for patients. It also has a wide variety of causes, including thrombi, which we recently addressed in part I of this review. In this second part, we look at additional causes of vascular occlusion.

Livedoid vasculopathies are a group of heterogeneous disorders featuring cutaneous ischemia. Common pathological features include perivascular fibrinoid deposition with absence of evident features of vasculitis. Livedo reticularis is a pattern of vascular reticular pigmentary changes that associate in some conditions and disorders, reflecting the underlying mechanism of predominantly arterial ischemic event. In this case report, we present a 43-year-old female patient who developed a bilateral multiple hyperpigmented nonpruritic lesions on both arms and thighs with spontaneous skin ulcerations of 3-month duration. The biopsy results revealed characteristics consistent with vasculopathy. The patient has showed a remarkable improvement on combination of cyclosporin and dapsone.

Livedoid vasculopathy (LV) is a dermatopathy characterized by skin ulceration consequential to chronic thombo-occulusive process. It is sometimes idiopathic or occurs consociated with chronic venous disease, thrombophelibitis, hyper coagulable state, protein C deficiency, factor VII deficiency, antiphospholipid antibody syndrome and hyperhomocystinemia. LV presents as grouped, painful, voilaceous macules, papules and patches with a proclivity to ulcerate over a long span of time. Dependent sites of the legs are affected, often bilaterally. Discrete ulcers are painful, small, angulated and coalesce at places to form bigger ulcers. On healing they leave behind atrophic, porcelain scars, surrounded by telangiectasia termed "atrophie blanche". We present a case of LV in a 22 year old female who presented with painful ulcers around the ankles for 8 months. Ulcers were covered with thick crust, and were painful on palpation. Skin biopsy revealed dermal vessel walls thickening and hyaline changes and their lumen had fibrin deposition, intra-luminal thrombosis, thus confirming the diagnosis of livedoid vasculopathy. Ulcers healed on treatment with pentoxifylline and colchicine. [J Med Allied Sci 2020; 10(2.000): 129-132]

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. This pilot study aimed to determine the efficacy of an anti-TNF-alpha agent in patients with refractory livedoid vasculopathy. 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. 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. 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Livedoid vasculopathy (LV) is a rare disease characterized by livedo racemosa, atrophie blanche, ulcerations, and severe pain. Low molecular weight heparins and rivaroxaban can be used in LV-patients. In addition, intravenous immunoglobulins (IVIG) have been described as treatment-option. Objectives: Objective was to investigate the therapeutic effect of IVIG on ulcer, pain and restrictions in daily life. Methods: Thirty-two LV-patients who received IVIG at the Department of Dermatology Tübingen between 01/2014 and 06/2019 were identified. Twenty-five of these patients were available for further follow up and were included in the study. Patients were interviewed using a questionnaire focusing on the course of the disease, symptoms, and subjective response to IVIG-treatment. Results: Twenty-five patients were included in the study (mean follow up: 28.9 months). Patients received an average of 6.8 cycles (range 1-45) of IVIG during the observed period. Significant improvements were seen regarding skin findings, pain, and limitation of daily activities. Complete remission of symptoms was observed in 68% of patients. Good tolerability of IVIG was shown in 92%. Conclusions: A good therapy response regarding ulceration, pain, and daily life restrictions with good tolerability was demonstrated for IVIG (2 g/kg bodyweight over 5 days). [ABSTRACT FROM AUTHOR]

T‐cell prolymphocytic leukemia (T‐PLL) is a rare, aggressive neoplasm derived from post‐thymic T‐cells. Patients are typically middle aged with a slight male predominance who present with a high white blood cell count, hepatosplenomegaly, lymphadenopathy, and other symptoms typically associated with leukemia. Although cutaneous involvement has been reported in up to 30% of cases of T‐PLL, to our knowledge, none have presented with a presentation resembling livedoid vasculopathy. In the correct clinical context, an underlying hematolymphoid neoplasm should be included in the differential diagnosis of a patient presenting with livedoid vasculopathy. [ABSTRACT FROM AUTHOR]

Livedoid vasculopathy is a chronic, recurrent skin disorder. It seriously affects the quality of patients’ life. However, the pathogenesis has not been fully identified yet. Here, this retrospective study describes the successful use of anti-TNF-α agent adalimumab in three cases of refractory livedoid vasculopathy, which has not been reported previously. In addition, we provide some clinical evidence that adalimumab therapy is efficient in improving skin lesions and relieving the pain of livedoid vasculopathy.

Livedoid vasculopathy (LV) is an uncommon, chronic, and recurrent thrombo‐occlusive vascular disorder. Data specific to LV in Thai population remains scarce. This study aimed to evaluate the clinical course and treatment outcomes of LV in Thai patients, and to perform a literature review for studies that reported on anticoagulant treatment in LV. Seventy‐four patients with a mean age of 37.6 ± 14.7 years were included. The female to male ratio was 5.2:1, and the median follow‐up was 10.5 months. Most patients had primary LV disorder. Forty‐eight patients were improved with treatments, with a median duration of 11.4 months. Combination treatments were commonly used, including anti‐inflammatories, antiplatelets, and immunosuppressants. Add‐on therapy with anticoagulant or psoralen plus ultraviolet‐A (PUVA) led to disease improvement in a majority of the patients treated. Kaplan‐Meier analysis demonstrated that 38.5%, 53.7%, and 57.9% would have disease improvement at 1, 2, and 3 years, respectively. Of 39 studies (n = 219) that reported on anticoagulant treatment in LV, anticoagulant drug was used as monotherapy in 104 patients. The mean duration of anticoagulant treatment was 7.2 ± 3.8 months, which led to disease improvement in 97 patients (93.3%). Bleeding side effect was found in 9 patients (8.7%). The highest incidence of LV was found among females aged 30 to 40 years. Combination therapy with anti‐inflammatory drugs, antiplatelet drugs, and immunosuppressants led to disease improvement. The observed efficacy of add‐on PUVA or anticoagulant is promising and should be further investigated. Further studies are needed to guide the development of an LV management guideline. [ABSTRACT FROM AUTHOR]

Sneddon's syndrome (SS) manifests through multiple strokes and livedo reticularis. Livedoid vasculopathy (VL) is characterized by a long history of foot and leg ulceration and histopathology indicating a thrombotic process. Arterial retinal branch occlusion is described in a 52-year-old male with VL. He did not present noticeable laboratory abnormalities, such as antiphospholipid antibodies, or a history of strokes. Retinal artery occlusion accompanied by VL could be a variant of Sneddon's syndrome. Optical coherence tomography angiography revealed a reduction in the macula's vascular layers in the asymptomatic eye, indicating localized microvascular changes as an evolving marker in the pathogenesis of SS., (Copyright © 2024 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

In this CME, we review two specific categories of ulcers: inflammatory (where inflammation is the primary pathologic process leading to ulceration) and vaso-occlusive (where occlusion is the primary process). Inflammatory ulcers include pyoderma gangrenosum and vasculitides, whereas livedoid vasculopathy, calciphylaxis and Martorell ulcers are vaso-occlusive ulcers. Determining the causes of ulcers in these conditions may require laboratory evaluation, biopsy and imaging., (Copyright © 2024. Published by Elsevier Inc.)

In the second part of this CME, we present an approach for the management of inflammatory and vaso-occlusive ulcers and highlight the need for further research in this field. The three overarching principles for management are etiology-specific treatment, ulcer care, and consideration of patient comorbidities and risk factors for poor healing. Both etiology-specific treatment and management of patient comorbidities and risk factors often require collaboration with providers from other specialties. Ulcer care is governed by TIME, or tissue debridement, infection control, management of moisture imbalance and epithelial edge advancement. As wound healing is a dynamic process, management should be adapted to changes in the status of the ulcer., (Copyright © 2024. Published by Elsevier Inc.)

Abstract is missing (Short communication)