Detection of Bartonella henselae DNA in the blood of patients with livedoid vasculopathy

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.

Medium-pressure hyperbaric oxygen therapy for livedoid vasculopathy / Terapia de oxigenación hiperbárica de media presión en vasculopatía livedoide

Abstract Livedoid vasculopathy (LV) is a cutaneous manifestation of several diseases that lead to non-inflammatory thrombosis of dermal vessels. We report the case of a 26-year-old female with a 4 years and 8 months-old history of diagnosis of LV and a non-healing ulcer of more than a year of evolution. Because of refractory response to standard care, low-pressure hyperbaric oxygen (LPHBOT) was added to the therapeutic scheme (azathiopine 2.5 mg/kg, folic acid and acetylsalicylic acid). After 12 sessions of LHBOT (60 min, 1.45 ATA ≈100% O2), ulcers achieved complete healing with significant pain relief and no recurrence was present over 6 months. More studies are necessary to determine the effectiveness of HBOT for LV treatment.

Resumen La vasculopatía livedoide (VL) es una manifestación cutánea de varias enfermedades que conducen a una trom bosis no inflamatoria de los vasos dérmicos. Se presenta el caso de una mujer de 26 años con antecedente de diagnóstico de vasculopatía livedoide de 4 años y 8 meses, además de una úlcera no cicatrizante de más de un año de evolución. Debido a la respuesta refractaria a la atención estándar, se añadió oxígeno hiperbárico a baja presión (LPHBOT) al esquema terapéutico (azatriopina 2.5 mg/kg, ácido fólico y ácido acetilsalicílico). Después de 12 sesiones de LHBOT (60 min, 1,45 ATA ≈100% O2), las úlceras tuvieron una curación completa con un alivio significativo del dolor y no hubo recurrencia durante 16 meses. Se necesitan más estudios para determinar la eficacia de TOHB para el tratamiento del VL.

Analysis of clinical characteristics of 64 cases of livedoid vasculopathy / 中华皮肤科杂志

Objective:To analyze clinical features and distribution patterns of livedoid vasculopathy lesions, especially obvious livedo reticularis and purpuric lesions.Methods:Clinical data were collected from 64 patients with confirmed livedoid vasculopathy in Hospital of Dermatology, Chinese Academy of Medical Sciences from July 2017 to October 2020, and analyzed retrospectively.Results:Among the 64 patients, 23 were males and 41 were females, aged 13 - 54 years; their age at onset ranged from 7 to 51 years, and 48 developed livedoid vasculopathy before the age of 25 years; the course of disease ranged from 6 months to 10 years. Livedoid vasculopathy occurred or worsened in summer in 49 patients, and skin lesions mainly manifested as necrotic irregular purpura, purpuric dermatosis-like erythema, irregular ulcers, livedo reticularis, telangiectasia, irregular white atrophic scars and pigmentation. Among the 64 patients, ulcers and necrotic purpura were mostly irregular, and occurred on the dorsum of the foot and around the ankle. A total of 40 patients presented with purpuric dermatosis-like lesions, including 32 with pigmented purpura and 4 with telangiectatic purpura. Besides, numbness, tingling and other symptoms of nerve terminal damage occurred in 4 patients.Conclusion:Clinical manifestations of livedoid vasculopathy are diverse, and differential diagnosis is important for patients with generalized livedo reticularis, purpuric dermatosis-like lesions and symptoms such as numbness.

Successful treatment of three cases of livedoid vasculopathy with rivaroxaban alone or in combination / 中华皮肤科杂志

Livedoid vasculopathy is a rare chronic ischemic necrotic skin disease caused by microvascular thrombosis in superficial vascular plexuses.Effective treatments are lacking.Efficacy of anticoagulants has been clinically proven in the treatment of this disease,but the administration route and adverse reactions limit their extensive application.The new oral factor Xa inhibitor rivaroxaban,which has many advantages,such as no need for coagulation monitoring,few adverse reactions and convenient oral administration,can be used in the prevention and treatment of thrombosis and post-thrombotic ulcers.In this article,3 patients with livedoid vasculopathy were treated with rivaroxaban alone or in combination,and satisfactory efficacy was obtained.

Livedoid vasculopathy: A review of pathogenesis and principles of management.

Livedoid vasculopathy is a rare cutaneous disease manifesting as recurrent ulcers on the lower extremities. The ulceration results in atrophic, porcelain white scars termed as atrophie blanche. The pathogenesis is yet to be understood with the main mechanism being hypercoagulability and infl ammation playing a secondary role. The important procoagulant factors include protein C and S defi ciency, factor V Leiden mutation, antithrombin III defi ciency, prothrombin gene mutation and hyperhomocysteinemia. Histopathology of livedoid vasculopathy is characterized by intraluminal thrombosis, proliferation of the endothelium and segmental hyalinization of dermal vessels. The treatment is multipronged with anti-thrombotic measures such as anti-platelet drugs, systemic anticoagulants and fi brinolytic therapy taking precedence over anti-infl ammatory agents. Colchicine, hydroxychloroquine, vasodilators, intravenous immunoglobulin, folic acid, immunosuppressive therapy and supportive measures are also of some benefi t. A multidisciplinary approach would go a long way in the management of these patients resulting in relief from pain and physical as well as psychological scarring.

Refractory Livedoid Vasculopathy Treated by Low-Molecular-Weight-Heparin / 대한피부과학회지

No abstract available.

Livedoid Vasculopathy with Hyperhomocysteinemia due to MTHFR Mutation / 대한피부과학회지

Livedoid vasculopathy is a hyalinizing vascular disease characterized by thrombosis and ulceration of the lower extremities. It can be caused by an alteration in control of coagulation with the formation of thrombi within dermal blood vessels. We report a case of livedoid vasculopathy with hyperhomocysteinemia due to MTHFR mutation, which is treated by folic acid and which also showed very unusual clinical manifestations. A 38-year-old male visited the department of dermatology with a 1 year history of purplish-brown purpura with punched-out ulcers on both lower legs. He had a history of homocysteinemia due to methylene tetrahydrofolate reductase (MTHFR) mutation. The histopathologic findings of the lesional skin revealed dense superficial and deep perivascular and perifollicular infiltrates of lymphocytes and fibrin deposition within the vessels in the dermis. On the basis of clinical and pathological findings, livedoid vasculopathy with hyperhomocysteinemia due to MTHFR mutation was diagnosed and improved by the treatment of 1 mg of folic acid daily.

Acute Central Retinal Artery Occlusion Associated with Livedoid Vasculopathy: A Variant of Sneddon's Syndrome

Livedoid vasculopathy (LV) is characterized by a long history of ulceration of the feet and legs and histopathology indicating a thrombotic process. We report a case of acute central retinal artery occlusion in a 32-year-old woman who had LV. She showed no discernible laboratory abnormalities such as antiphospholipid antibodies and no history of cerebrovascular accidents. Attempted intra-arterial thrombolysis showed no effect in restoring retinal arterial perfusion or vision. The central retinal artery occlusion accompanied by LV in this case could be regarded as a variant form of Sneddon's syndrome, which is characterized by livedo reticularis and cerebrovascular accidents.

Treatment of Livedoid Vasculopathy with Pulsed Intravenous Immunoglobulin / 대한피부과학회지

No abstract available.

Ischemic Neuropathy Associated with Livedoid Vasculitis

BACKGROUND: Livedoid vasculitis is a chronic dermatological problem with an unclear etiology. Clinical findings are petechiae with painful ulcers in both lower extremities, which heal to become hyperpigmented and porcelain-white satellite lesions. There are only a few reported cases of livedoid vasculitis presenting in combination with peripheral neuropathy. CASE REPORT: We report the first case of a Korean patient presenting with mononeuritis multiplex combined with livedoid vasculitis, which was confirmed by electrophysiological and pathological studies. CONCLUSIONS: Our report supports the possible vaso-occlusive etiology of livedoid vasculitis in multifocal ischemic neuropathy.

Vasculopatía livedoide: reporte de dos casos / Livedoid vasculopathy: a two case report

Vasculopatía livedoide es una enfermedad poco frecuente que se presenta principalmente en mujeres de edad media de la vida, antes de los cuarenta años. Es llamado también atrofia blanca, livedo reticular con ulceración de verano, vasculitis hialinizante segmentaria, o PURPLE (úlceras purpúricas dolorosas con patrón reticular de extremidades inferiores). Generalmente afecta los tobillos y se exacerba en primavera y verano. Su etiología es aún desconocida, pero se puede clasificar en formas primarias (sin ninguna patología asociada) y secundarias, ambos con un componente trombótico. A continuación se describen dos casos de vasculopatía livedoide: una mujer de 22 años con úlcera en pierna izquierda y púrpura retiforme en ambas extremidades. El estudio demostró que el cuadro estaba asociado o síndrome antifosfolípidos. El segundo caso es de una mujer de 47 años con uno vasculopatía livedoide secundaria o lupus eritematoso sistémico.

Livedoid vasculitis is a rare disease that occurs mainly in women under 40 years of age. It has also be ennamed atrofia blanche, hyaline segmentary vasculitis or PURPLE (purpuric ulcers with reticular pattem in low legs). It usually affects ankles and has an exacerbation during Spring and Summer seasons. It has an unknown etiology, and it has been classified as primary (or idiopathic) or secondary (associated to another disease). We describe two cases of livedoid vasculopathy: the first case is o 22-year-old women with ulcers and retiform purpura associated to antiphospholipid syndrome. The second case is o 47-year-old women with livedoid vasculopathy associated to systemic lupus erythematosus.

Livedoid Vasculopathy Associated with Hepatitis C / 대한피부과학회지

Livedoid vasculopathy is a chronic disease which appears with recurrent painful livedo reticularis patches and ulcers. The exact cause is unknown but it is known to be associated with some connective tissue or infectious diseases. Vasculopathy has been frequently reported as a cutaneous manifestation in chronic hepatitis C patients. As research into the pathology of hepatitis C progresses, new relationships between it and skin symptoms are emerging. We report a 44-year-old woman with livedoid vasculopathy who has no other known associated disease but chronic hepatitis C.

Drug-associated livedoid vasculopathy

Livedoid vasculopathy is reported in a 38-year-old white female, who first presented with spontaneous skin lesions in the left ankle, at 17 years old. For the last fifteen years she used low-dose oral contraceptive (gestodene and ethinylestradiol) and persisted asymptomatic for a long period. Nevertheless, painful red papules and dark spots reappeared in the same area and progressed to an intensely tender and irregular shallow ulcer, during summer. Skin biopsy samples showed dermal vessels with subintimal fibrinoid necrosis and intraluminal thrombosis, without clear inflammation. Cutaneous lesions have improved faster after topical betamethasone was added to treatment. Data from the present case suggests a drug-associated livedoid vasculopathy.

Relata-se caso de vasculopatia livedóide em uma mulher branca de 38 anos, que inicialmente apresentou lesões cutâneas espontâneas no tornozelo esquerdo aos 17 anos de idade. Durante os últimos quinze anos, fez uso de contraceptivos orais (gestodene e etinilestradiol). Permaneceu assintomática por longo período; entretanto, no último verão, pápulas avermelhadas dolorosas e manchas escuras reapareceram na mesma área e formaram uma úlcera rasa irregular intensamente dolorosa. O exame das amostras da biópsia de pele mostrou vasos dérmicos com necrose fibrinóide e trombose intraluminar, sem reação inflamatória. As lesões cutâneas apresentaram cicatrização mais rápida após acréscimo de betametasona tópica ao esquema terapêutico. Os dados do presente caso sugerem vasculopatia livedóide associada ao uso de droga.